Benign and Malignant Neoplasms Flashcards

Question

26- A 56-year-old marathon runner presents with a superficial BCC On the left lower leg. He prefers to avoid excision or dessication and curettage and presents 4 weeks later with the findings seen here. Which of the following is the best statement regarding counseling for the patient? A- Treatment with topical creams for superficial cancers has a lower cure rate than surgery but has an excellent cosmetic outcome. The findings here are consistent with a robust response and suggests a better clearance of the tumor at completion of therapy B- The tumor here has grown significantly and the patient must now undergo Mohs surgery for therapy given the size and location of the lesion C- Desiccation and curettage has a similar cosmetic outcome to topical therapies (5FU or imiquimod) as well as similar cure rate to excision D- Mohs surgery is never appropriate for superficial basal cell or squamous cell carcinoma in situ malignancies as they are minimally invasive E- It is best to monitor the superficial basal cell cancer annually because it is not an aggressive malignancy

Answer 1

Correct choice: A. Treatment with topical creams for superficial cancers has a lower cure rate than surgery but has an excellent cosmetic outcome. The findings here are consistent with a robust response and suggests a better clearance of the tumor at completion of therapy. Explanation: Imiquimod can be a reasonable treatment for superficial basal cell cancers. It has an excellent cosmetic outcome but can be very inflammatory, as seen in the image. The tumor has not grown significantly, this is a reaction to the imiquimod. Dessication and curettage often has a worse cosmetic outcome than topical chemotherapeutics and has a lower cure rate than excision. Based on location and size, Mohs surgery is sometimes an appropriate option for superficial keratinocyte cancers. It is not unreasonable to monitor a superficial BCC as they are slow growing tumors, however it is not the best answer and annual follow-up may not be enough treatment.

Answer 2

Correct choice: A. Scalp Explanation: This is an angiosarcoma as evident by the numerous atypical vessels, hemorrhage and atypical endothelial cells within the atypical vessels. These tumors most commonly occur on the scalp as violaceous nodules in elderly men.

Answer 3

Correct choice: E. fluorouracil 5% Explanation: Fluorouracil 5%. A standard course of fluorouracil treatment (application twice daily for 2 to 4 weeks) to the face and ears were shown to decrease the risk of SCC by 75% in the first year after treatment, although it was not shown to decrease the risk of keratinocyte carcinomas over a 4-year period. The remaining medications are FDA-approved for the treatment of actinic keratosis, but have not yet been studied for chemoprevention of basal or squamous cell carcinomas.

Answer 4

Correct choice: C. Upper cutaneous lip at the base of the nasal ala Explanation: This is a microcystic adnexal carcinoma. These typically occur on the upper cutaneous lip at the base of the nasal ala in young adult females. It is imperative the base of the tumor be seen to ensure it is completely removed as the tumor is aggressive.

Answer 5

Correct choice: A. Arborizing vessels Explanation: This is a basal cell carcinoma which will show arborizing vessels. A hemangioma will show red lagoons on dermoscopy. A spitz nevus has a starburst pattern. A blue-grey veil is associated with melanoma. Brown globules is indicative of pigmented nests in the papillary dermis.

Answer 6

Correct choice: E. it is a mixture of polyclonal antibodies to tyrosinase Explanation: The ‘MCW Melanoma Cocktail’ is an immunostain made up of monoclonal antibodies to MART-1, Melan-A and tryrosine. It is used intraoperatively during sentinel lymph node biopsy and allows for rapid and accurate determination of micrometastases. The mixture of stains are monoclonal antibodies; not polyclonal.

Answer 7

Correct choice: A. Histone deacetylase inhibitor Explanation: Vorinostat and romidepsin are histone deacetylase (HDAC) inhibitors. The most common side effects are fatigue, gastrointestinal symptoms and reversible thrombocytopenia. Denileukin difitox is an IL-2 receptor inhibitor, and is associated with capillary leak syndrome. Ipilimumab is an antibody to CTLA-4. Tofacitinib is a JAK 1/3 inhibitor.

Answer 8

Correct choice: E. Posterior shoulder Explanation: Spindle cell lipoma is a solitary benign tumor seen in adult male patients, and is most often located on the posterior shoulder or neck regions. The tumor histologically consists of mature collagen, adipose tissue, spindle cells, and mast cells. Treatment is with local excision. None of the remaining answer choices represent the most common location for spindle cell lipoma.

Answer 9

Correct choice: E. Tumors arising within scars/chronic ulcers have the highest risk of metastasis Explanation: This is SCC of the ear. SCCs arising within scars/chronic ulcers metastasize in ~30-40% of cases, whereas those arising on the lip or ear have a 10-20% risk of metastasis. Recurrent SCCs have a metastasis risk of up to 30%. SCC is the 2nd most common skin cancer in Caucasians (BCC is most common). Oral SCC most commonly presents on the lateral tongue. SCC is typically more aggressive in patient with CLL. Organ transplant patients have a 65-fold increased risk for developing cutaneous SCC.

Answer 10

Correct choice: B. Locally advanced or metastatic variants may respond to sonic hedgehog inhibitors Explanation: The dermoscopic picture is of a pigmented BCC. Answer choice 1 refers to melanomas; choice 3 refers to keratoacanthoma; choice 4 refers to merkel cell carcinoma; choice 5 refers to squamous cell carcinoma.

Answer 11

Correct choice: D. Myeloperoxidase Explanation: Chloromas are greenish tumor grossly secondary to involvement of the skin in acute granulocytic leukemia. The green color is secondary to myeloperoxidase. The other listed answers do not contribute to the green color of chloroma.

Answer 12

Correct choice: D. These lesions often have a better prognosis than their pigmented variants. Explanation: The correct answer is D (D is false). Amelanotic melanomas are often diagnosed later (i.e. thicker Breshlow depth) than their pigmented counterparts, which is the reason for their poorer prognosis. Amelanotic melanomas are often mistaken for basal cell carcinomas as both can appear clinically similar. The recently published AJCC 8th Edition Melanoma Staging System revised the definitions of T1a and T1b so that T1a melanomas include those <0.8 mm without ulceration while T1b melanomas include those 0.8-1 mm with or without ulceration and those <0.8 mm with ulceration. Mitotic rate is no longer a T1 category criterion but should be documented for all invasive primary melanomas. Thus, in the absence of ulceration, sentinel lymph node biopsy should be considered if tumor thickness is 0.8mm or greater. Amelanotic melanomas are treated identically to their pigmented counterparts, and increased tumor thickness is associated with a worse prognosis (irrespective of the clinical subtype of melanoma).

Answer 13

Correct choice: D. Brooke-Spiegler syndrome Explanation: This is a biopsy of a cylindroma. Multiple cylindromas seen with CYLD mutation either in cylindromatosis or in conjunction with other adnexal neoplasms in Brooke-Spiegler syndrome.

Answer 14

Correct choice: A. Imatinib Explanation: Imatinib has been used in the treatment of primary or locally recurrent dermatofibrosarcoma protuberans. Even with wide excision, the 5 year recurrence rate for this tumor can be 20-25%. This neoplasm commonly possesses a reciprocal translocation t(17;22) resulting in fusion of collagen 1alpha1 and platelet derived growth factor B (a fusion oncogene). Activation of the platelet derived growth factor receptor associated with overexpression of platelet derived growth factor is central to the development of DFSP. Imatinib is a protein tyrosine kinase inhibitor used primarily to treat chronic myelogenous leukemia with the Philadelphia chromosome defect. Imatinib also inhibits the tyrosine kinases associated with platelet derived growth factor and stem cell factor. Therefore, it directly inhibits the platelet derived growth factor receptor signaling cascade, which plays a critical role in the pathogenesis and growth of DFSPs. While Imatinib has been successful in clinical trials, it is not yet FDA-approved for the treatment of DFSP. DFSP stains positive for CD34 and negative for Factor XIIIa, which differentiates it from a dermatofibroma (stains positive for Factor XIIIa and negative for CD34). Vismodegib is a smoothened-inhibitor that is FDA-approved for the treatment of basal cell carcinoma. Avelumab is an anti-PD-L1 monoclonal antibody that was recently FDA-approved for the treatment of merkel cell carcinoma. Ipilimumab is an anti-CTLA-4 monoclonal antibody that is FDA-approved for the treatment of melanoma. Nivolumab is an anti-PD-1 monoclonal antibody that is FDA-approved for the treatment of melanoma.

Answer 15

Correct choice: B. Reticulated pigment network Explanation: Typical dermoscopic features of seborrheic keratoses include: milia-like cysts, irregular crypts, fissures/ridges, blue-gray lobules, light brown fingerprint-like parallel structures, fat fingers (the gyri of a cerebriform surface). A reticulated pigment network is not a common feature of seborrheic keratoses.

Answer 16

Correct choice: B. Milky red globules Explanation: This is an image of melanoma, which is associated with milky red globules on dermoscopy.

Answer 17

Correct choice: D. It is the most common type seen in darker-skinned patients Explanation: The associated image depicts an acral lentiginous melanoma (ALM), which is the most common type of melanoma in darker-skinned individuals. Notably, and very sadly, the famous musician Bob Marley was diagnosed with ALM in 1977 and succumbed to this malignancy in 1981 as it had metastasized to his lungs and brain. Breslow depth, not ulceration, is the most important prognostic factor for melanoma. Immunosuppression increases one's risk of melanoma by a factor of 3-4. Male gender is a poor prognostic factor. Anti-PD-1 antibodies (e.g. nivolumab, pembrolizumab) are proven to prolong survival in patients with metastatic melanoma.

Answer 18

Correct choice: D. BRAF Explanation: The pictured lesion is a melanoma. The most common mutation in a melanoma is in the BRAF gene (~50% of advanced or unresectable melanomas). NRAS mutations are the next most commonly found mutation (~30% of melanomas). BAP-1 (BAP-1 tumor predisposition syndrome), CDKN2A (melanoma pancreatic cancer syndrome), and C-kit may also be mutated in cases of melanoma.

Answer 19

Correct choice: A. Ferguson-Smith Explanation: Patients that have a sudden appearance during childhood of multiple keratoacanthomas have Ferguson-Smith type of KA. This is autosomal dominant and are the KAs are typically self-healing. Grzybowski is typically diagnosed in adulthood with the sudden appearance of hundreds to thousands of lesions in a disseminated fashion. Gorlin syndrome is characterized by the appearance of multiple BCCs (not KAs) during childhood. It is also characterized by odontogenic keratocysts of the jaw and skeletal defects (e.g., macrocephaly, hypertelorism, frontoparietal bossing, spina bifida, or rib abnormality, among others). Tumors associated with this disease include medulloblastoma, meningioma, ovarian fibromas (bilateral), and cardiac fibromas. It is inherited in an autosomal dominant pattern, and due to a mutation in PTCH gene. KA centrifugum is a subtype of solitary KA that may reach a size of up to 20 cm in diameter. Buschke-Lowenstein tumor is a verrucous carcinoma caused by HPV 6 and 11 that is locally invasive and destructive but rarely metastatic.

Answer 20

Correct choice: D. Multicentric Castleman disease Explanation: The clinico-pathologic correlation is best for Kaposi sarcoma, which is caused by HHV-8 (KSHV). Multicentric Castleman disease and primary effusion lymphoma are also associated with HHV-8. The other remaining answer choices are not associated with HHV-8.

Answer 21

Correct choice: E. p53 mutation Explanation: This is a SCC of the tongue. SCCs most commonly have mutations in p53. PTCH mutations are in basal cell carcinomas. CDKN2A mutations are in familial melanomas. A lichenoid infiltrate would be seen with lichen planus which is usually on the buccal mucosa. Heck’s disease is due to HPV 13 and 32.

Answer 22

Correct choice: D. Mesothelioma Explanation: This clinico-pathologic picture is most consistent with the BRCA1 associated protein-1 (BAP1) cancer syndrome. BAP1 is a nuclear deubiquitinating enzyme that functions as a tumor suppressor via its role in DNA damage repair. The BAP1 cancer syndrome is a rare autosomal dominant genetic syndrome typified by the development of mesotheliomas and uveal melanomas. Patients with BAP1 cancer syndrome develop several, distinct, melanocytic neoplasms. These are raised, pink or tan, dome-shaped, benign lesions that have been referred to in the literature as “melanocytic BAP1-mutated atypical intradermal tumors/MBAITs” or “BAPomas.” Less commonly, cutaneous melanomas, various types of carcinomas (mostly from the kidney and gallbladder), sarcomas, and brain tumors can arise in the BAP1 cancer syndrome. Thus, this patient is less likely to develop one of the other listed answer choices compared to mesothelioma.

Answer 23

Correct choice: D. Diabetes mellitus Explanation: This is a syringoma. The clear-cell variant (a rare histological variant of syringoma that is clinically indistinguishable from an ordinary syringoma) has been associated with diabetes mellitus.

Answer 24

Correct choice: E. PTCH Explanation: This is a basal cell carcinoma. The most common gene mutation is PTCH. p53 is mutated in SCCs, PMS2 and MSH6 are lost in sebaceous neoplasms of Muir-Torre. c-KIT is mutated in acral melanoma, mucosal melanoma, and mast cell disorders.

Answer 25

Correct choice: D. Mohs micrographic surgery Explanation: Standard treatment of dermatofibrosarcoma protuberans (DFSP) consists of complete surgical extirpation. The rate of recurrence is lower following Mohs micrographic surgery compared to wide local excision. This question asks the examinee to identify DFSP presenting as a characteristic firm red-brown tumor on the shoulder, and know the treatment that will impart lowest recurrence risk. Initial treatment of this tumor is complete surgical removal, with studies demonstrating lower risk of recurrence with Mohs micrographic surgery (choice 4) versus wide local excision (choice 5). The programmed cell death-1 inhibitor pembrolizumab (choice 1) has not been used to treat DFSP. Imatinib mesylate (choice 2), which interferes with the activity of the COL1A1-PDGFB fusion protein found in DFSP, is FDA-approved for unresectable, recurrent, and metastatic cases. Radiation (choice 3) has been utilized for unresectable or recurrent DFSP, and would not be an appropriate choice for initial therapy.

Answer 26

Correct choice: D. 1-2cm to fascia Explanation: Merkel cell carcinoma is an aggressive rare tumor of the skin accounting for less than 1% of cutaneous malignancies. Also known as neuroendocrine cancer of the skin, this tumor presents as a painless red to violaceous, firm, solitary, nodule that usually presents on sun exposed areas such as the head, neck and upper extremities. These tumors present usually during the 6th and 7th decades, and have a 2 year survival rate of 50-70%. Because of this tumor's high potential for regional and distal metastasis, this tumor should be excised with wide local excision with 1-2cm surgical margins to the depth of fascia or pericranium, or treated with Mohs Surgery.

Answer 27

Correct choice: C. Maple leaf structures Explanation: Maple leaf structures on dermoscopy support the diagnosis of basal cell carcinoma. This question asks the examinee to identify a pigmented BCC and select the most closely associated dermoscopic finding. In the case of this dark blue-black irregular papule at the alar crease, initial concern for melanoma is quite reasonable. However, on close inspection, there is a subtly rolled border, suggesting the correct diagnosis, pigmented BCC. Dermoscopy can assist in distinguishing between pigmented BCCs and other pigmented lesions. Among the answer choices, maple leaf structures (choice 3) are most closely associated with BCC. The starburst pattern (choice 1) is classically seen in pigmented spindle cell nevus of Reed. Blue-whitish veil (choice 2), regression structures (choice 4), and irregular streaks (choice 5) are features of melanoma, not routinely observed in pigmented BCC.

Answer 28

►C The Ferguson-Smith is a keratoacanthoma variant characterized by the sudden appearance during childhood or adolescence of multiple KAs that may resolve and later-on reappear. This condition is inherited in an autosomal dominant pattern

Answer 29

►E The treatment of choice for Dermaotfibrosarcoma protuberans is Mohs surgery. Radiation therapy has been used, however has limited value as solitary therapy for thsi tumor. Radiation therapy can be used as an adjunct to wide surgical excision. Classically, these tumors should be excised with 3cm margins. The recurrence rate associated with these tumors can be 10 -20 percent with wide excision with 3 cm margins. With Mohs surgery, the recurrence rate ranges from 0% to 6%. 5FU and Imiquimod are not effective modalitites in treating DFSPs, as it infiltrates deep into the subcutaneous tissue.

Answer 30

►B Squamous cell carcinoma is the second most common cancer of the skin. Mutations in the tumor suppressor p16 and p53 are commonly found in SCC's. Normally, UV damage upregulates p53 thereby delaying cell cycle progression. DNA damage can then be repaired or the cell could undergo apoptosis. In squamous cell carcinoma, p53 exhibits loss of heterozygosity due to C to T or CC to TT mutations.

Answer 31

►E This patient has the Grzybowski type of keratoacanthomas. Typically diagnosed in adulthood, these patients have the sudden appearance of hundreds of small lesions in a disseminated fashion. The lesions can be found anywhere on the body including palms, soles, larynx, and oral mucosa.

Answer 32

►C The patient has actinic cheilitis. Notice the red, scaly lower lip, with erosions and fissures. The CO2 laser is currently a common treatment alternative for this condition.

Answer 33

►C Characterized by the presence of multiple, small, hyperpigmented, sessile SKs on the face, DPN is typically diagnosed on the African-American and Hispanic population.

Answer 34

►D It has been reported that the risk for the development of atypical nevi and melanoma is higher than twofold with a history of five or more episodes of painful sunburn during adolescence.

Answer 35

►E Keratoacanthomas present as a solitary, firm, dome-shaped papule with a cratiform center.

Answer 36

►C TX Primary tumor cannot be assessed T0 No evidence of primary tumor Tis Carcinoma in s itu T1 Tumor ≤2 cm in greatest dimension with <2 high-risk features∗ T2 Tumor >2 cm in greatest dimension with or without one additional high-risk feature, or tumor any size with ≥2 high-risk features∗ T3 Tumor with invasion of maxilla, mandible, orbit, or temporal bone T4 Tumor with invasion of skeleton (axial or appendicular) or perineural invasion of skull base. *High risk features: Depth/invasion >2 mm thickness or Clark level ≥IV,Perineural invasion, Primary site ear, Primary site hair-bearing lip, Poorly differentiated or undifferentiated.

Answer 37

►A Hemangioma have a characteristic appearance under dermoscopy. Typically, they have a maroon lagoon or red sacculae appearance.

Answer 38

►B The CDKN2A gene located on chromosome 9p21 is the most important mutated gene associated with MM of the listed choices. BRAF is another important mutation.

Answer 39

►A Patients that have SCC have a 0.3-16% of chance of metastasis. The location, size and type of SCC can determine the possibility of metastasis.

Answer 40

►B The metastatic potential of BCC is very low with rates ranging from 0.0028 to 0.1%. The head and neck region is the most frequent location of the primary tumor with regional lymph nodes being the most common site of metastasis. The lungs, bone, liver, and pleural are also potential sites of metastasis.

Answer 41

►D Chloromas, also termed granulocytic sarcomas, are a localized tumor composed of immature granulocytic cells. They frequently have a greenish coloration due to the presence of myeloperoxidase and most commonly affect the bone. The condition most often occurs in patients with acute leukemia of the myeloid type.

Answer 42

►A p53 is a tumor suppressor gene which arrests cell cycle in G1 as it controls the transition from G1 to S. It also downregulates BCL-2. Mutations in p53 are associated with Li-Fraumeni syndrome as well as the development of squamous cell carcinomas.

Answer 43

►B Compliance is a key feature in treatment with 5-FU. Erythema, inflammation and erosion must develop and is considered a sign of successful treatment.

Answer 44

►E Dermoscopy is a useful tool in differentiating a pigmented basal cell carcinoma from melanoma. Basal cell carcinomas may have arborizing blood vessels, maple leaf pattern, blue-grey ovoid nests, and orange crust or ulcer. Milky red globules are sometimes seen in melanoma.

Answer 45

►A Acral lentiginous melanoma is the predominant type of melanoma in dark-skinned individuals. It is usually located on the soles, palms, and subungeal region of patients in their fifth to sixth decade of life.

Answer 46

►D Neuron specific enolase stains merkel cells. Vimentin stains melanocytic lesions, sarcomas and lymphomas. S-100 and HMB-45 stains melanocytic lesions, such as melanoma.

Answer 47

►A Verrucous carcinomas are low-grade carcinomas which are slow-growing and metastasize very late in the course. The presence of HPV has been demonstrated in cases both by electron microscopy and DNA hybridization.

Answer 48

►E A chromosomal reciprocal translocation t(17;22)(q22;q13), and supernumerary ring chromosome have been reported as cytogenetic characteristics of DFSP. Typically DFSP is CD34 positive and factor XIIIa negative, allowing its differentiation from dermatofibroma.

Answer 49

►A The L-Myc gene has been found to be amplified in Merkel cell carcinoma but not in normal skin. C-Myc has been found to be amplified in neuroblastoma. GLI1 is a transcription factor involved in hedgehog signaling and a potential target in basal cell carcinomas. CDKN2A is implicated in familial forms of melanoma that are associated with pancreatic cancer. PTEN is a tumor suppressor gene that is mutated in Cowden Disease, Bannayan-Riley-Ruvalcaba Syndrome, and Proteus Syndrome.

Answer 50

►A The most common location for a BCC is the lower eyelid. It is the most common epithelial tumor of the eyelid.

Answer 51

►E Cutaneous horn can overlie an AK or SCC or a benign lesion and it presents as a conical protuberance arising from an erythematous base. Actinic cheilitis results from the confluence of multiple AKs on the lips. Leukoplakia is a clinical diagnosis and is defined as a white patch in the oral cavity. It is the most common premalignant condition of the oral cavity. Bowenoid papulosis manifests clinially as multiple red-brown warty papules that histologically represent high grade squamous intraepithelial lesions.

Answer 52

►C The current surgical margins are 0.5 cms for melanoma in situ, 1 cm for melanomas that measure less than 2mm in thickness and 2cm for melanomas >2mm.

Answer 53

►E Denileukin diftitox is as diphtheria fusion toxin that targets the IL-2 receptor. It is a systemic treatment alternative for recalcitrant or advance CTCL.

Answer 54

►B Maple leaf-like structures seen on dermoscopy are an important diagnostic criterion for pigmented basal cell carcinomas. They correspond to the heavily pigmented cells in the bas aloid cells.

Answer 55

►D Cyclooxygenase-1 and -2 and enzymes that catalyze the conversion of arachidonic acid to prostaglandins. It is believed the prostaglandin E2 (PGE2), whose levels are increased by ultraviolet irradiation, is pro-inflammatory and may contribute to skin carcinogenesis. In a study by Orengo et.al., hairless mice who were given celecoxib were found to have a significantly longer latency period between exposure to ultraviolet light and the development of skin carcinomas.

Answer 56

►A The p53 and PTCH genes are the major targets of UVB for the development of BCC. Other genes involved include the Smoothened-activating mutations, and PTCH2 mutations.

Answer 57

►C Infections associated with Human Papilloma Virus can produce growths on the epithelial or mucosal surfaces. There are over 100 strains of these viruses, and some of these strains can predispose to intraepithelial carcinomas, particularly when involving the anal or g enital mucosa. In general, HPV strains 16 and 18 are classified as his risk virus types and can be associated with cervical cancer , oral cancer, anal cancer and periungual cancers. HPV 6 and 11 are associated with condyloma acuminata; HPV types 2, 4 are associated with common warts; HPV type 13 has been associated with Heck's disease and HPV type 8 has been associated with epidermal dysplasia verruciformis.

Answer 58

►E Regression in malignant melanoma appears histologically as a focal area of fibrosis with lymphocytes and melanophages in the papillary dermis. The presence of regression precludes accurate measurement of true thickness of the melanoma. Extensive regression in thin melanomas has a significant association for the risk of metastases.

Answer 59

►A Dermatofibrosarcoma protuberans is a rare, low-grade dermal sarcoma. Typically, the lesion occurs as a painless subcutaneous mass that grows slowly. This malignancy typically has lateral spread but invade deep. Metastasis is rare but has been reported to the lung.

Answer 60

►C p53 is the most commonly mutated tumor suppressor gene involved in human cancer and is often mutated in SCC and BCC. p53 acts via two main pathways, 1) activation of p21 (Cdk inhibitor) which leads to cell cycle arrest, and 2) activation of Puma which inhibits Bcl-2 (apoptosis inhibitor) thereby leading to cell death. CKDN2A, a gene that when mutated leads to a risk of melanoma, acts via 1) activation of p16 (another Cdk inhibitor) and 2) activation of p14ARF which inhibits Mdm2 (which normally degrades p53). Akt is involved in the PI3K-Akt signaling pathway and inhibits cell cycle arrest and apoptosis.

Answer 61

►B Mucinous carcinoma presents as a slowly growing, asymptomatic, round, erythematous nodule on the head and neck. Forty percent of cases occur on the eyelid. Histologically it is characterized by the presence of large areas of mucin (―sea of mucousǁ) with small islands of basophilic epithelial cells. Primary mucinous carcinoma of the skin has an indolent course. Local recurrence occurs in 1/3 of patients following excision. The rate of metastasis is low (9.6%).

Answer 62

►A The image shows erythrodermic patient with generalized desquamation of skin. Skin biopsy and blood tests the diagnosis of Sezary syndrome. Sezary syndrome is a leukemic variant of mycosis fungoides, is characterized by the triad of pruritic erythroderma, g eneralized lymphadenopathy, and the presence of Sezary cells more than 1,000 cell/mm3 (abnormal, large hyperconvoluted lymphocytes) in peripheral blood . Other manifestations include scaling and fissuring of palms and soles, alopecia, pruritus, peripheral edema, and nail dystrophy. Exfoliative dermatits can be manifested in many conditions other than Sezary syndrome inculude pemphigus foliaceus, psoriasis and drug reaction.

Answer 63

►E Bowenoid papulosis manifests clinically as multiple red-brown warty papules or confluent planques on the external genitalia. These lesions may resemble genital warts but histologically represent high-grade squamous intraepithelial lesions. Bowenoid papulosis is caused by infection by HPV and linked to HPV 16, 18, 31, 35, and 39.

Answer 64

►C An ulcerated tumor of any size with micrometastasis in 1 node is T1-4b N1a M0. This corresponds to stage IIIB.

Answer 65

►A The #1 site of metastasis for DFSP are lungs. It has a low grade of metastasis <5% and treatment is wide local excision with 3 cm margins.

Answer 66

►D Typically DFSP is CD34 positive and factor XIIIa negative, allowing its differentiation from dermatofibroma.

Answer 67

►C Angiosarcomas are very rare, aggressive vascular tumors. They occur most commonly in the head and neck region of white, elderly individuals. Men are more commonly diagnosed with this neoplasm. The lesion initially arises as a painless, purple macule or plaque on the scalp or face. Later on it becomes an elevated bluish or purple nodule that may ulcerate. Common symptoms include bleeding, edema, and ultimately pain. Cervical lymph node and hematogenous metastases commonly occur. Wide surgical excision is the treatment of choice, with radiation therapy usually employed after surgical excision.

Answer 68

►E Characterized by the sudden appearance of multiple SKs, the sign of Leser-Trelat may have lesions located anywhere in the body. However, the eruptionǁ is classically described to appear in a Christmas treeǁ pattern commonly located on the chest and back. It may be associated with internal malignancies such as adenocarcinomas.

Answer 69

►E Tumor size (greater than 2 cms), location (lips, ears, eyelids), depth of invasion, degree of differentiation, perineural invasion, immunosupression, as well as recurrent tumors and those that arise in areas of chronic inflammation are all factors that increase the risk of metastasis.

Answer 70

►A The most common locations for BD include the head and neck regions and the extremities. BD arising on the lower limbs is frequently found in women, whereas lesions located on the ears and scalp are more common in men. The basement membrane remains intact on histopathology. 5% of patients develop invasive SCC. Risk factors include chronic sun exposure, immunosuppression, HPV, arsenic exposure, and ionizing radiation. Mucosal BD may appear as a verrucous or polypoid plaque, as an erythroplakic patch, or as a velvety red plaque.

Answer 71

►E The risk of skin cancer in organ transplant patients is dramatically increase and may be more aggressive. In one study, SCCǁs had a 65x increased incidence, BCC 10x, and melanoma 3.4x.

Answer 72

►C Pagetoid reticulosis is an indolent cutaneous T-cell lymphoma. Pagetoid reticulosis favors an acral distribution and typically presents as scaly oval plaques. Epidermotropism is present, with tumor cells being CD4 positive or CD8 positive. Another interesting immunohistochemical finding is the absence of CD45 expression.

Answer 73

►C Reed's syndrome is an autosomal dominant disease with incomplete penetrance characterized by uterine and cutaneous leiomyomas. Clinically, leiomyomas appear as flesh colored or pinkish brown dermal papules or nodules that range from 0.2 to 2.0 cm in diameter. Their presentation may otherwise be quite variable. They may be isolated or many in number, may be variably distributed or dermatomal, and may be asymptomatic or painful in response to pressure or cold. The predisposition gene for Reedǁs syndrome has been localized to chromosome 1q42.3-43 and the gene encoding fumarate hydratase. Currently, the United States National cancer Institute is recommending screening for all patients with leiomyomatosis to evaluate for an occult renal malignancy.

Answer 74

►D IFN-alpha, IL-2, TNF-alpha, have all been demonstrated to have some therapeutic benefit in t he treatment of melanoma. GM-CSF is the immune stimulator in the vaccine Talimogene laherparepvec. IL-10 has been used to treat inflammatory disorders such as atopic dermatitis and psoriasis.

Answer 75

►C Bowenǁs disease arising on the lower limbs is frequently found in women; whereas lesions located on the ears and scalp are more common in men. The epidermal dysplasia does not interrupt the basement membrane confirming the diagnosis of SCC in situ.

Answer 76

►B Five clinical variants of porokeratosis are recognized. They are the classic porokeratosis of Mibelli, disseminated superficial actinic porokeratosis, prokeratosis palmaris et plantaris disseminata, linear porokeratosis, and punctate porokeratosis. On histopathology they have varying degrees of a cornoid lamella. This appears as a column of parakeratosis.

Answer 77

►D Topical 5-FU blocks the methylation reaction of deoxyuridylic acid to thymidylic acid, thus interfering with the synthesis of DNA and RNA. Normal side effects during treatment include pruritus and burning at the site of application. It is a treatment option for patients with multiple AKs within an area.

Answer 78

►D Providing the highest cure rates, Mohs micrographic surgery is the treatment of choice for large BCCs (1cm on the face or 2 cms on the trunk), located on high-risk anatomic areas (ear, eyelids, lips, genitals, nose, temples). , Morpheaform BCCs, tumors with positive margins after conventional excision, and recurrent BCCs, are also an indication for Mohs surgery.

Answer 79

►C UVB radiation triggers the formation of thymidine dimers both in DNA and RNA, resulting in mutated keratinocytes. The mutations occur on the tumor suppressor gene p53 within the keratinocytes resulting in impairment of the mechanism of apoptosis. Therefore, clonal expansion of mutated keratinocytes may occur leading to the formation of AKs.

Answer 80

►E Features of atypical spitz include location in the back over one cm and can have irregular color and border. The risk factors for metastatic Spitz is ulceration, increased Breslow thickness, atypical mitotic features and more H-RAS mutations as opposed to BRAF and NRAS.

Answer 81

►D Granular cell tumors are benign growths which typically occur on the tongue. They are typically well-circumscribed, raised, firm nodules. Histologically, the cells have uniform nuclear characteristics and granular cytoplasm due to presence of lysozyme. The lesions are PAS positive and S-100 positive.

Answer 82

►C Multiple case series have shown that the hard palate (and specifically the anterior hard palate/ alveolar arch) is the highest risk location for intra-oral melanoma. Prognosis is generally worse for oral melanoma, which is usually due to delay in diagnosis and presentation with more invasive disease.

Answer 83

►B Syringotropic and folliculotropic mycosis fungoides (MF) are the variants of MF that should be diagnosed by punch biopsy. Both variants of pagetoid reticulosis, Woringer-Kolopp disease and the disseminated Ketron-Goodman disease, are best diagnosed with a broad shave biopsy. Classic and poikilodermatous MF should also be diagnosed with a shave biopsy. To rule out Sezary syndrome, flow cytometry should be performed.

Answer 84

►E Sentinel lymph node biopsy has gained acceptance as part of the work up for melanomas 1-4 mm in thickness. Even though it has been widely accepted due to its low morbidity and high feasibility, the role of SLN biopsy in survival has not been established. It is mainly being used for a more accurate staging, prognosis and to determine if further adjuvant therapy is necessary.

Answer 85

►B Angiosarcomas are CD31, CD34, factor VIII, and cytokeratin positive. The carcinoembryonic antigen (CEA) stains positive in Paget‘s disease, metastatic adenocarcinoma, and tumors with eccrine differentiation.

Answer 86

►A Increasing age and male gender have a negative effect on survival. Primary lesions located on the extremities have a better prognosis than those located on the trunk, head or neck region. Ulceration is also considered a poor prognostic factor. Furthermore, there is a significantly lower survival for those patients with palpable metastatic nodes (macrometastasis) when compared to those with micrometastatic nodes (nonpalpable).

Answer 87

►D Size of a BCC is related to its risk for metastasis. 3cm BCC has a 1.9% risk of metastasis. If a BCC is >10cm it has approximately a 50% risk of metastasis. If metastasis does occur it most commonly metastasizes to lymph nodes>lung>bone>skin>liver.

Answer 88

►D The patient has Bowen's Disease, or squamous cell carcinoma in situ. Multiple treatment options are available, including conventional excision, Mohs micrographic surgery, imiquimod, cryosurgery, and 5-FU, among others. Conventional excision is the most commonly used method. With this method, not only is the lesion removed, but it also provides the specimen for histologic verification to rule out invasive SCC. Recurrence rates of 5% have been reported with conventional excision. Mohs micrographic surgery is an option when the lesion is located in areas where there is an increased risk of sub-clinical spreading, or when tissue sparing is a priority. Imiquimod is not approved to treat SCCIS.

Answer 89

►A Organ transplant recipients are at increased risk of having both systemic and cutaneous develop. The estimated increased risk is 3-4 times that of the general population. However, the risk of skin cancer alone is much higher (e.g. SCC ― 65x increase, BCC 10-fold increase, Melanoma 3.4x increase). This increased risk is thought to be partially due to the immunosuppressant agents used to prevent graft rejection.

Answer 90

►E When considering a keratoacanthoma, it is important to obtain a biopsy of the specimen down to the subcutaneous fat. This can be achieved either by complete excisional biopsy, full-thickness shave biopsy, or fusiform incision through the entire KA including its center and sides.

Answer 91

►D Merkel cell carcinoma is an aggressive rare tumor of the skin accounting for less than 1% of cutaneous malignancies. Also known as neuroendocrine cancer of the skin, this tumor presents as a painless red to violaceous, firm, solitary, nodule that usually presents on sun exposed areas such as the head, neck and upper extremities. These tumors present usually during the 6th and 7th decades, and have a 2 year survival rate of 50-70%. Because of this tumor's high potential for regional and distal metastasis, this tumor should be excised with wide local excision with 3cm surgical margins.

Answer 92

►E Keratoacanthomas have three, consecutive, clinical stages: proliferative, mature and resolving. The proliferative stage is characterized by the appearance of a rapid growing papule. This phase is followed by the mature stage when the lesion acquires its ch aracterisitic dome-shaped appearance with a central, keratinous core. Tumor resorption occurs during the involution stage resulting in a slightly depressed, hypopigmented scar.

Answer 93

►E The risk of metastasis from a primary SCC increases with tumor size (>2cm), location (lips, ear, and eyelid, among others), depth of invasion, immunosuppression, degree of differentiation, and perineural invasion. Recurrent tumors and SCCs arising in areas of chronic inflammation (such as osteomyelitis, burn scars, or ulcers) are also considered high risk for metastases.

Answer 94

►C Typical AKs appear as flat, erythematous, rough papules that are better felt than seen.

Answer 95

►E Sister Mary Joseph nodule is a cutaneous metastasis that is most commonly associat ed with stomach, large bowel, ovary, and pancreatic cancer. Tripe palms are most commonly associated with lung, Leser-Trelat with adenocarcinomas of the stomach, colon and breast, Trousseau Sign with carcinoma of the pancreas and pityriasis rotunda with hepatocellular carcinoma and gastric cancer.

Answer 96

►E Extramammary Paget's cutaneous adenocarcinoma of glandular differentiation. Approximately 25% of these tumors are associated underlying neoplasms. Histologically, the Paget cells stain positively with PAS, mucicarmine, CEA, EMA, LMW keratin. They are HMB45 negative which is a melanocytic marker.

Answer 97

►E Cryotherapy is the most common treatment for AKs, with a cure rate of 98.8%.

Answer 98

►C While there is an overrepresentation of CD8+ cases compared to classic mycosis fungoides, more cases are still predominantly CD4+. Patients typically present at a younger age (median age 44 years), with a slight male predominance. There is an excellent response to photo therapy with clinical improvement in ~90% of patients treated. There is an increased association with LyP compared to other types of mycosis fungoides. (Abbott et al, JAAD 2011; in press)

Answer 99

►C Microcystic adnexal carcinomas are more commonly located in the perioral, nasolabial, or periorbital areas.

Answer 100

►D Merkel cell carcinoma can be summarized with "AEIOU" that stands for expanding rapidly, immune suppression, older than 50 years, ultraviolet-exposed area in person with fair skin.

Answer 101

►A SCC is the most common type of skin cancer in Asian Indians and Blacks. BCC is the most common type of skin cancer in Caucasians, Japanese, Chinese, and Hispanic people.

Answer 102

►B Hypomelanosis associated with melanoma most commonly may be seen in three ways. The first type is analogous to a halo nevus. The second is a remote leukoderma distant from the primary lesion. The third is a vitiligo like leukoderma. It may portend a better prognosis in compa rison to others with the same stage of disease.

Answer 103

►D The main dermoscopic features of seborrheic keratoses include comedolike openings, fissures, milialike cysts, fingerprinting and lack of true pigment network. In fact, it is the lack of true pigment network, branched streaks, and pigment globules that differentiate SKs from melanocytic lesions.

Answer 104

►C Basal cell carcinoma. It has a male to female ratio 2:1. UVB damages DNA and produces a C -T transition mutation (65% of BCC). P53 and PTCH genes are commonly affected. It has a slow, indolent growth potential: metastatic potential 0.0028% to 0.1%.; known to metastasize to lymph nodes and lungs.

Answer 105

►D This patient has a Merkel cell carcinoma. Vimentin, desmin, and S-100 are consistently absent in MCC. Mohs micrographic surgery has been used successfully for the treatment of MCC, with or without adjuvant therapy. This neoplasm may sometimes contain several neuropeptides including vasoactive intestinal peptide, calcitonin, ACTH, gastrin, and somatostatin.

Answer 106

►A The translocation for DFSP is Chr 17 and 22. These chromosomes involve the fusion of PDGF beta chain and collagen type I alpha I genes. DFSP are generally CD34+ and factor XIIIa negative.

Answer 107

►C Kaposi's sarcoma is an AIDS defining illness. It if found at a much higher incidence in homosexual men. Human herpes virus 8 is thought to be pathogenic in this tumor.

Answer 108

►A The condition shown is an acquired digital fibrokeratoma. It is a pedunculated, acral lesion with collarette which is defined by the lack of nerve twigs and bone on pathologic exam.

Answer 109

►D Amelanotic melanoma are difficult to diagnosis with the naked eye and even with the dermoscope. Pigmented basal cell carcinomas may have a characteristic maple leaf pattern, arborizing blood vessels, and /or blue-grey ovoid nests. Seborrheic keratoses often have comedolike openings and milia cysts. Hemangiomas typically have red lagoons.

Answer 110

►E Merkel cell carcinoma usually presents as a violaceous, solitary, dome-shaped nodule, most commonly located on the head and neck region.

Answer 111

►A One of the most common locations for MAC includes the perioral area. Histologically it presents with poorly demarcated tumor cells invading the dermal and subcutaneous tissue. Islands of basaloid keratinocytes, horn cysts and duct structures are also seen within a desmoplastic stroma.

Answer 112

►E The incidence of SCC increases with age, and it is 35 times higher in individuals older than 75 years of age when compared to ages 50-55. The incidence doubles for each 8 to 10 degree decline in latitude, therefore, individuals living closer to the equator have a greater risk.

Answer 113

►D Increasing age and male gender have a negative effect on survival in melanoma. Patients with primary lesions located on the extremities (except acral lesions) have a better prognosis than those with tumors located on the head, neck, or trunk. There is a significantly lower survival for patients with palpable metastatic nodes (macrometastasis) when compared to those with micrometastatic nodes. For stage IV disease, patients with non-visceral metastases (eg skin, subcutis, distant lymph nodes) have a better prognosis compared with those with visceral metastases.

Answer 114

►B Giant congenital nevi overlying the spinal columna nd skull can be associated with neurocutaneous melanosis. Presenting symptoms include increased cranial pressure, spinal cord compression or leptomeningeal melanoma. A MRI should be performed in these children to ruleout CNS involvement. The other tests would not be as useful in this situation.

Answer 115

►A Merkel cell carcinoma a cutaneous neuroendocrine carcinoma that usually occurs on the head and neck. It is a high grade malignant tumor with a 5-year mortality rate of 30-64%. On histopathology, there are trabecular strands of basophilic cells that stain with a characteristic paranuclear dot pattern with cytokeratin 20.

Answer 116

►B HPV 6 and 11 are most often associated with anogenital warts or condyloma acuminata. They can present as sessile lesions on the skin or peducnulated cauliflower lesions. HPV type 5 is associated with epidermodysplasia verruciformis, HPV 4 is assoicatd with common warts, HPV type 7 is associated with Butcher warts, and HPV type 1 is associated with common warts.

Answer 117

►C DFSP intially arises as an asymptomatic, reddish or skin colored indurated plaque, which may later enlarge, and become raised, firm and multinodular. DFSP is histologically characterized by the presence of monomorphic spindle cells arranged in a "storiform" or "cartwheel" pattern.

Answer 118

►E Asymmetry, multicomponent pattern, blue-whitish veil, parallel-ridge pattern, atypical pigment network, uneven radial streaming, localized irregular and diffuse pigmentation, irregularly distributed globules, and regression structures are all dermoscopic features suggestive of malignant melanoma.

Answer 119

►B When staging lymphoma, it is critical to determine whether the lymphoma is primary cutaneous arising in the skin or secondary cutaneous arising in association with nodal or extranodal tumor. The prognosis is worse in secondary when compared to primary lymphomas, irrespective of histologic diagnosis.

Answer 120

►D A recent study in the New England Journal of Medicine reported an increase in survival of patients with stage III and stage IV melanoma with a new immunologic therapy agent named ipilimumab. The survival was increased from 6 months to 10 months (P < 0.001). All the other medicines are not used to treat melanoma; they are used to treat psoriasis, among other disease.

Answer 121

►B Large/giant congenital nevi are greater than 20 cm or greater than 10% of the body surface area. The risk of melanoma in this type of lesion is approximately 6% by the age of 60 (Rhodes, 1981; Bett, 2005). 50% of these melanomas occur by 5 years of age.

Answer 122

►E High risk locations for recurrence and metastases include the ―H-zoneǁ of the face, skin overlying cartilage and bony structures (e.g. preauricular area, retroauricular sulcus, nasolabial fold, inner canthus, philtrum, temple, upper lip, columella, nose, lower eyelid). Lesions located on the trunk and extremities are usually considered low risk unless aggressive histologic pattern is present.

Answer 123

►A Patients with recessive dystrophic epidermolysis bullosa havea high risk of aggressive SCC in the areas of chronic erosion and must be screened thoroughly and frequently. Actinic keratosis is not skin cancer.

Answer 124

►A Digital mucous cysts are pseudocysts found on the dorsal digits between the distal interphalangeal joint and the nail fold. The fibroblasts in digital mucous cysts produce large quantities of hyaluronic acid.

Answer 125

►E Typically DFSP is CD34 positive and factor XIIIa negative, allowing its differentiation from dermatofibroma.

Answer 126

►C Clear cell acanthomas are slow growing, benign growths which typically occur on the leg. They have a striking histologic appearance in the epidermis with sharp demaracation and enlarged, pale cells. Excess glycogen in the cells accounts for their clear appearance and is due to a defect in phosphorylase.

Answer 127

►E Gorlins syndrome is characterized by the appearance of multiple BCCs during childhood, odontogenic keratocysts of the jaw, and skeletal defects. It is inherited in an autosomal dominant pattern.

Answer 128

►D The most likely diagnosis in this patient is recurrent BCC, therefore Mohs surgery is the treatment of choice. Furthermore, any NMSC measuring 3 cms located on any anatomical site is an indication for Mohs surgery.

Answer 129

►A Patients with Grzybowski keratoacanthoma have eruptive and disseminated KA during adulthood. KA centrifugum are large and solitary and Giant KA can invade underlying.

Answer 130

►B HPV infection has been associated with the development of cutaneous SCC. HPV 6 and 11 have been linked to genital lesions, whereas HPV 16 has been found in periungeal lesions.

Answer 131

►E The origin of KAs has not been established. Ultraviolet exposure, exposure to chemical carcinogens such as tar and pitch, as well as smoking, and a viral etiology, specifically the human papilloma virus, have all been proposed as etiologic factors.

Answer 132

►E Merkel cell carcinoma is a type of aggressive cancer with a high rate of recurrence. It is a neuroendocine carcinoma that is most commonly on sun exposed areas like the head and neck in the elderly. Treatment includes wide local excision of 2-3 cm, Mohs, radiation, and chemotherapy.

Answer 133

►E Sezary syndrome is characterized by the triad of pruritic erythroderma, generalized lymphadenopathy, and presence of Sezary cells in peripheral blood. Sezary cells are abnormal, large hyperconvoluted lymphocytes. The five year survival is estimated to be between 10-20%.

Answer 134

►B The incidence of skin cancer in transplant recipient (from greatest to least) is as follows: SCC>BCC>melanoma>Merkel cell.

Answer 135

►C Epidermotropism, defined as the presence of lymphocytes in the epidermis is characteristic of CTCL.

Answer 136

►A Imatinib or Gleevec is used in the treatment of primary or locally recurrent dermatofib rosarcoma protuberans. Even with wide excision, the 5 year recurrence rate for this tumor can be 20-25%. Activation of the Platelet derived growth factor receptor associated with overexpression of Platelet Derived growth factor is central to the development of DFSP Imatinib is a protein tyosine kinase inhibitor for Bcr-Abl kinase and is used primary in chronic myelogenous leukemia with the Philadelphia chromosome defect. Imatinib also inhibits the typosine kinases associated with platelet derived growth factor and stem cell factor. Therefore, it directly inhibits the Platelet Derived Growth factor receptor signaling cascade,that plays a critical role in the pathogenesis and growth of DFSPs. While Imatinib has been successful in clinical trials, it is not yet FDA approved for the treatment of DFSPs. The other listed agents are not adjunctive or primary therapies for DFSP.

Answer 137

►A The lip has an approximate rate of metastasis of 13%, which is slightly higher than the metastasis rate of 11% from the ear. Risks for metastasis include size greater than 2cm, perineural invasion, immunosuppression, treatment history, degree of tumor differentiation, or location on the ear or lip.

Answer 138

►C Curettage of giant congenital nevis is a highly controversial treatment option. De Raeve and Roseeuw reported on 16 neonates who underwent curettage for treatment of their giant congenital nevi. They noted that it was best performed within the first two weeks of life. They claim that it offers an adequate cosmetic alternative to surgical excision. The risk of melanoma developing within the giant congenital nevus is not decreased and long-term follow-up is essential.

Answer 139

►E Stewart-Treves syndrome refers to an angiosarcoma arising from chronic lymphedema. It typically occurs as a complication of long-lasting lymphedema of the arm after mastectomy and/or radiotherapy for breast cancer. It is a very late complication of disease, usually developing several years later. The mean time-frame for development of Stewart-Treves Syndrome in the literature is 23 years (4-40 years).

Answer 140

►A CTCL is a neoplasm of helper T cells (CD4) that originates in the skin.

Answer 141

►C The most commonly used markers for MCC are monoclonal antibodies to cytokeratins 8, 18, and 20; neuron-specific enolase (the most constant marker); chromogranin A/B, and synaptophysin. Leukocyte common antigen, vimentin, desmin, glial fibrillary acidic protein, and S-100 are consistently absent in MCC.

Answer 142

►C The most frequent sites of basal cell carcinomas in one study was the base of the nose (bordering the nasolabial fold and extending midway up the side of the nose), orbital region, apex of the nose, ear, forehead, temporal region, nasolabial area, and the buccal region (in order). The nose was by far the most frequent site of BCCǁs.

Answer 143

►D Desmoplastic trichoepithelioma is a variant of trichoepithelioma, an uncommon adnexal tumor with differentiation toward hair structures. It is three times more common in females and usually occurs in young adults. The tumor is a well-circumscribed nodule lesions located in the upper dermis. Strands or columns of basaloid cells are seen surrounded by fibrotic or desmoplastic stroma. Horn cysts may also be seen, as well as foci of sebaceous cells, calcification and ossification.

Answer 144

►C According to Riddel et al (JAAD 2011;64(6):1147-1153), Mohs micrographic surgery, although the treatment of choice, results in a 20% recurrence rate, which is significantly higher than cutaneous SCC. HPV16 is most often implicated. Men outnumber women 2:1. The rate of metastasis averages between 2-3%. Although common in transplant patients, HPV associated digital SCCs can occur secondary to trauma and in immunocompetent patients.

Answer 145

►B According to a study performed by Chamberlain, et.al., thick melanomas (> 3 mm) were predominantly nodular in type. They occurred in older men, mostly on the head and neck and were associated with fewer nevi.

Answer 146

►B Merkel cell carcinoma is frequently diagnosed in individuals older than 50 years of age. However, there have been reports of MCC in children and young adults.

Answer 147

►A There are three histologic patterns of MCC: trabecular, intermediate-cell type, and small-cell type. The trabecular variant consists of interconnecting trabeculae separated by strands of connective tissue. Pseudorosettes may be seen in this type.

Answer 148

►B This patient has the tumor stage of mycosis fungoides(MF). Patients with tumoral lesions are automatically classified as IIB. IA is the limited patch or plaque disease with less than 10% BSA involved. IB is the generalized patch/plaque stage with more than 10%BSA but less than 80% BSA involved. IIA implies lymph node involvement. IIIA is erythroderma without blood involvement. IIIB is erythroderma with low blood tumor burden. The stage IV of MF is divided in three substages: IVA1(high blood tumor burden-Sezary syndrome), IVA2(very abnormal nodes), IVB(visceral involvement).

Answer 149

►B Salicylic acid, tretinoin, and alpha-hydroxy acids have been reported to be useful in treatment of AKs. Low fat diets have been reported to be useful in decreasing the incidence of AKs. Cryotherapy has a reported cure rate of 98.8% for AKs, whereas topical 5-FU has a reported cure rate of 93%. UVB, not UVA, is most responsible for AK development. Male gender is a risk factor for the development of AKs.

Answer 150

►C Stewart-Treves syndrome is the development of angiosarcoma in the setting of chronic lymphedema. Originally, named after radical mastectomy for the treatment of breast cancer. The term applies to the development of angiosarcoma in any chronic lymphedematous condition.

Answer 151

►C Angiosarcomas present as painless, purple nodules in the head and neck region of elderly individuals. Men are more frequently diagnosed with this aggressive neoplasm.

Answer 152

►A In a patient with skin lesions and pathology suggestive of cutaneous lymphoma, gene rearrangement studies should be done to detect a clonal population of lymphocytes. Polymerase chain reaction (PCR) is approximately 1000 times more sensitive than Southern blot in the detection of a clonal population of cells. In addition, PCR may be used on formalin fixed tissue as well as fresh tissue, whereas Southern blot testing must be done of fresh tissue only.

Answer 153

►B Basal cell nevus syndrome (Gorlin Syndrome) is caused by a mutation in the PTCH gene, located on chromosome 9q22. This gene is found to be mutated in 30-40% of sporadic basal cell carcinomas. Gorlin Syndrome is inherited in an autosomal dominant pattern. Tumors associated with this sydrome include medulloblastoma and meningioma. It is characterized by the appearance of multiple BCCs during childhood, odontogenic keratocysts of the jaw, and skeletal defects (including frontoparietal bossing and hypertelorism, among others).

Answer 154

►E Cryosurgery destroys tumor if it is frozen to -40 to -70 degrees Celcius, for at least 2 cycles with a 60 second thaw. Benign lesions can be destroyed at temperatures around -25 degrees Celcius.

Answer 155

►C Tuberous sclerosis a rare genodermatosis characterized by adenoma sebaceum, seizures and mental retardation. Other cutaneous features include hypopigmented macules, periungual fibromas, fibrous plaque of the face, cafe-au-lait macules and connective tissue nevus.

Answer 156

►B Ferguson-Smith syndrome is a rare autosomal dominant condition that is characerized by multiple keratoacanthomas found in sun-exposed areas. Lesions typically regress over weeks to months.

Answer 157

►E Basal cell carcinomas are slow-growing, epithelial tumors. Highest area of recurrences after surgical excision is in the "H-zone" of the face which include nose, nasolabial folds, periocular, periauricular. The scalp is a M-zone.

Answer 158

►B Monoclonality and gene rearrangements can be detected with a Southern blot. Southern blots can be used to detect specific DNA fragments by gel-transfer hybridization.

Answer 159

►E Lentigo maligna is a type of melanoma in situ that usually presents as a poorly circumscribed, variably colored patch. It occurs on sun-exposed areas on the head and neck. Margins may be difficult to evaluate and recurrences are relatively common. There are rare reports of spread onto the oral mucosa as well as conjunctiva.

Answer 160

►D The rate of SCC metastasis from all skin sites ranges from 0.5% to 5.2%. Local recurrences and metastasis are related to: 1.treatment modality, 2. prior histological, 3. location, 4. size, 5. depth , 6. histological differentiation, 7. histological evidence of perineural involvement, 8. histological evidence of desmoplastic features, 9. precipitating factors other than UV light, and 10. host immunosuppression. In reference to metastatic disease, the highest rates occur from scars, the lip, and the external ear. Patients with perineural spread have a local recurrence rate of 47.2% and a metastatic rate of 34.8%. Demosplastic SCC's are 6 times more likely to metastasize than other histological patterns.

Answer 161

►A Breast cancer is the most common cancer to metastasize to the skin in women.

Answer 162

►E This patient has a desmoplastic trichoepithelioma. Desmoplastic trichoepithelioma is a variant of trichoepithelioma, an uncommon adnexal tumor with differentiation toward hair structures. It presents commonly in young women as a small, firm, umbilicated papule on the face. Local surgical excision is the treatment of choice.

Answer 163

►B The current melanoma staging is developed by the American Joint Comittee on Cancer. This system is based on four characterisitics: 1. Tumor thickness 2.Presence of ulceration within the primary tumor, 3. Involvement of lymph nodes and 4.presence of distal metastases. Stage 1A characterizes a melanoma that has less than 1mm in tumor thickness and no ulceration, no nodes, and no distant mets. Stage IB characterizes a melanoma with tumor thickness less than 1mm however with ulceration and no nodes or distal metastases. Stage IB also can characterize melanomas with tumor thickness between 1.01-2.00 mm without ulceration. Stage IIA classifies tumor with thickness between 1.01-2.00mm, with ulceration, and no nodes and no distal metastases. Stage IIA also characterizes those melanomas with thickness between 2.01-4.0mm without ulceration, nodes, or distal mets. Stage III and IV melanomas have nodal and distal met involvement respectively.

Answer 164

►D The most common malignancy to present with cutaneous metastases in men is from melanoma, followed by squamous cell cancer of the head and neck. In women, breast cancer is most likely to present with cutaneous metastases. Special presentations of cutaneous metastases include alopecia neoplastica (scarring alopecia) and zosteriform metastases.

Answer 165

►A Angiosarcoma may occur in association with chronic lymphedematous states. Stuart-Treves syndrome applies to angiosarcoma arising in an area of chronic lymphedema, like upper arm lymphedema after mastectomy.

Answer 166

►B Imiquimod induces the synthesis and release of cytokines such as interleukins 1, 5, 6, 8, 10, and 12, among others.

Answer 167

►B Ferguson Smith is familial (AD), and is characterized by regressing keratoacanthomas beginning in childhood. Grybowski is a non-familial disorder in which patients develop generalized eruptive keratoacanthomas beginning between age 40 to 60. Keratoacanthoma centrifugum marginatum is most commonly a solitary lesion occurring later in life. Muir Torre is a syndrome characterized by gastrointestinal or genitourinary neoplasms which precede development of sebaceous neoplasms and keratoacanthomas by one to two decades. Rombo is not associated with keratoacanthomas, but is associated with multiple basal cell carcinomas, atrophoderma vermiculatum, hypotrichosis, and milia.

Answer 168

►B Pilomatricomas are the most common superficial pediatric tumor. The occurrence of multiple pilomatricomas has been most closely associated with the development of myotonic dystrophy(Steinert Disease). The onset of myotonic dystrophy, an autosomal dominant disorder with variable penetrance, can occur before or after the onset of lesions. Cigliano et al. reported myotonic dystrophy in 1 out of 2 patients with multiple pilomatricomas; Julian et al. reported myotonic dystophy in 1 out of 4 patients. The other syndromes associated with multiple pilomatricomas include Rubenstein-Taybi, Turner syndrome, Gardner syndrome, and sarcoidosis.

Answer 169

►C Epithelioid sarcoma is a rare soft tissue sarcoma that most commonly develops in young adults, males greater than females, with a predilection for the distal upper extremities, namely hands and forearms. Most tumors present as firm-to-hard palpable masses, either in the deep soft tissue or in the dermis. The superficial lesions can present with ulceration. Five year survival and ten year survival rate for patients with epithelioid sarcoma are approximately 50-70% and 42-55% respectively (Journal of bone and Joint Surgery (Am), 70-A: 862-870, 1988). Female patients have a more favorable outcome. Proximal lesions have been shown to have worse outcomes compared to distal lesions.

Answer 170

►E Squamous cell carcinomas are the most common carcinoma of the oral cavity. The most common locations for this tumor are the lateral and ventral surfaces of the tongue and the floor of the mouth.

Answer 171

►D Desmoplastic trichoepithelioma presents as small, firm, umbilicated papule on the face of young adults.

Answer 172

►A Patients that have multiple keratoacanthoma in childhood have Ferguson-Smith type of KA. This is autosomal dominant and are self-healing.

Answer 173

►E Attached picture is disseminated superficial actinic porokeratosis (DSAP) which is the most common type of all porokeratosis, with multiple thin papules appearing most commonly on the legs of adult women. Many treatments have been used for this condition which include: cryotherapy, topical 5-fluorouracil (5-FU), topical retinoids, CO2 laser, and dermabrasion. Although surgical excision might be used for treatment of other forms of porokeratosis, it is not advised in this case because of number of lesions and increase risk of scarring. Other forms of porokeratosis are: porokeratosis of Mibelli, punctate porokeratosis, linear, and Porokeratosis palmaris et plantaris disseminata.

Answer 174

►E Fibroepithelioma of pinkus variant of BCC is often a fleshy or pink colored nodule. The most common location of a fibroepithelioma of pinkus is the lumbosacral region. Superficial variants are more common on the trunk and extremities. BCC makes up the most common non melanotic skin cancer at 75%.

Answer 175

►B Merkel cell carcinoma has been found to be associated with the merkel cell polyomavirus (MCPyV). Herpes virus causes diseases such as HSV, VZV, EBV, CMV, roseola, and Kaposi's sarcoma. Paramyxovirus causes measles. Flavivirus causes diseases such as west nile virus, dengue, and yellow fever. Enteroviruses are not associated with merkel cell carcinoma.

Answer 176

►E The Grzybowski type of keratoacanthoma is typically diagnosed in adulthood, with the sudden appearance of hundreds of lesions in a disseminated fashion. The lesions are generally 2 -3mm in diameter and can be found anywhere on the body including the palms, soles, larynx, and oral mucosa.

Answer 177

►A The most reliable prognostic factors in MM are Breslow‘s depth and ulceration of the primary tumor. Breslow depth is the thickness of the melanoma measured from the granular layer to the deepest point of tumor invasion.

Answer 178

►A The risk of SCC in an organ transplant population is elevated by 65x. These patients should have a year skin examination.

Answer 179

►E Pinkus BCC (also known as fibroepithelioma) appears as a pedunculated, dome-shaped papule frequently located on the back (lumbosacral area).

Answer 180

►D The size of a BCC is related to its metastasis risk. Invasion of bone, cartilage, and muscle is not common, however, spread does occur along perichondrium, periosteum, fascia, or tarsal plate.

Answer 181

►C The patient has basal cell nevus syndrome, or Gorlin syndrome, which is characterized by the appearance of multiple basal cell carcinomas in childhood, odontogenic keratocysts of the jaw, and skeletal defects (i.e macrocephaly, hypertelorism, frontoparietal bossing, spina bifida, or rib abnormalities). Tumors associated with this disease include medulloblastoma and meningioma (not pheochromocytoma). It is caused by a mutation in the PTCH gene located on 9q22 and is inherited in an autosomal dominant manner.

Answer 182

►B Mycosis fungoides is a rare form of cutaneous T-cell lymphoma, the etiology of which is not completely known. Adult T Cell Lymphoma is also a type of T cell neoplasm that has been linked to HTLV-1. Adult T-cell lymphoma may have an acute and chronic, smoldering form. The chronic, smoldering form can be difficult to distinguish from mycosis fungoides.

Answer 183

►A SCCs that measure 1cm on the chest are not an indication for Mohs micrographic surgery. Lesions must measure 2cms on the chest and extremities, or 1cm for those located on the face to be considered for Mohs surgery. Due to the aggressive growth patter and subclinical spread of morpheaform and recurrent BCCs are always and indication for Mohs surgery.

Answer 184

►A Epithelioma cuniculatum is a plantar SCC that is locally aggressive but rarely metastasizes, Oral florid papillomatosis is the a type that is found in the mouth. Buschke-Lowenstein is the type that you find in the genital area.

Answer 185

►C The most common neoplasm to arise in patients who have been treated with long -term PUVA therapy is squamous cell carcinomas. In a study by Stern, et.al. a 12.8 fold risk was found for the development of squamous cell cancers in patients who received high doses than those that received low doses.

Answer 186

►A CK7 will stain keratinocytes. Vimentin, S-100 and HMB-45 stains melanocytic lesions.

Answer 187

►E Desmoplastic trichoepitheliomas is one of the differential diagnosis for MAC. MAC show deep subcutaneous and perineural invasion, as well as CEA positive staining, all features that may help differentiate it from desmoplastic trichoepitheliomas. Both, MAC and desmoplastic trichoepitheliomas are commonly located on the face.

Answer 188

►B The most common locations of MM in women are the back, lower legs and upper extremities.

Answer 189

►B Superficial spreading melanoma may be located anywhere, but the back is the most common site in male patients.

Answer 190

►E Localized hypohidrosis is a feature found in Bazex syndrome but not Rombo syndrome. Another differentiating feature is that Rombo syndrome classically has vermiculate atrophoderma, while Bazex has follicular atrophoderma.

Benign and Malignant Neoplasms Flashcards

(214 cards)