18: Plastics, Skin, and Soft Tissue Flashcards
What cell type originates from the bone marrow and act as antigen-presenting cells in the skin and mucosa?
Langerhan cell
[They have a role in contact hypersensitivity reactions (type IV).]
Which type of melanoma is the least aggressive, exhibits minimal invasion, and usually grows radially first?
Lentigo maligna melanoma
[UpToDate: Lentigo maligna is a slowly evolving type of melanoma in situ that typically occurs in the sun-damaged skin of the face and neck of older individuals. The risk of progression to invasive lentigo maligna melanoma ranges from 5 to 20 percent. The development of darker pigmentation, sharper borders, or elevated or nodular areas are clinical signs of progression. There are no randomized trials evaluating treatment for lentigo maligna. We suggest surgical excision with margins of 5 to 10 mm, depending upon lesion size and location (Grade 2C).]
Are bone sarcomas typically found early or late?
Late
[Most are metastatic at the time of diagnosis.]
[UpToDate: At the time of presentation, between 10 and 20 percent of patients have demonstrable macrometastatic disease and are classified as stage III according to the staging system used by the Musculoskeletal Tumor Society. Distant metastases most commonly involve the lungs, but can also involve bone.
Occult micrometastases are presumed to be present in the majority of those who appear to have clinically localized disease, since before the era of adjuvant chemotherapy, over 80 percent of patients with osteosarcoma developed metastatic disease despite achieving local tumor control. It was postulated that these patients had subclinical metastases that were present at the time of diagnosis. With routine use of systemic adjuvant chemotherapy, at least two-thirds of children and adolescents with nonmetastatic osteosarcoma will be long-term survivors, implying the success of chemotherapy in eradication of micrometastases. Prognosis is worse in adults with osteosarcoma, particularly those over the age of 65.]
Match the risk factor for the type of sarcoma:
- Asbestos
- PVC and arsenic
- Chronic lymphedema
- Asbestos: Mesothelioma
- PVC and arsenic: Angiosarcoma
- Chronic lymphedema: Lymphangiosarcoma
What is the treatment for disseminated Kaposi’s sarcoma?
Interferon-alpha
[HAART is the best treatment for AIDS-related Kaposi’s sarcoma. Surgery may be required for severe intestinal hemorrhage.]
[UpToDate: Recombinant interferon alfa (IFNa) is approved for treatment of AIDS-associated KS in the US. While the mechanism of antitumor action of IFNa in KS is not known, it may involve direct antiproliferative effects, antiviral effects, inhibition of angiogenesis, and modulation of host cellular and humoral immune responses
Intralesional injection of interferon alfa, alone or in combination with interleukin-2, has also been reported to induce regression of classical KS lesions. The studies cited evaluated the effects of twice- or thrice-weekly injections over a period of four to six weeks, which is unlikely to be practical for routine treatment of individuals with multiple cutaneous lesions.]
What is the most common subtype of childhood rhabdomyosarcoma (The most common soft tissue sarcoma in kids)?
Embryonal subtype
[alveolar subtype has the worst prognosis.]
[UpToDate: The embryonal subtype is the most common, accounting for 59 percent of all RMS cases. Most (50 percent) are of the classic subtype, and the botryoid and spindle cell variants comprise 6 and 3 percent, respectively. Alveolar RMS represents 21 percent of all cases, while the remainder are classified as undifferentiated (8 percent), pleomorphic/anaplastic (1 percent), or NOS (11 percent).
The morphologic appearance of the tumor cells comprising alveolar and embryonal RMS is nonspecific. The cells have scant cytoplasm and a centrally placed round nucleus that occupies the majority of the cell. It is the organizational architecture of the tumor that distinguishes alveolar from embryonal subtypes.
Classic embryonal RMS is composed of typical rhabdomyoblasts arranged in sheets and large nests, with infrequent intermixed fusiform cells, and no suggestion of an alveolar architectural pattern. The typical rhabdomyoblast has moderate to deeply eosinophilic cytoplasm, representing poorly-formed myofilaments. Myofilaments with cross-striations are usually present only in the well-differentiated spindle cell subtype, which is so named because the cells have a characteristic elongated spindle-like appearance. Spindle cell RMS often presents in a paratesticular location.]
What is the description of a stage III pressure sore?
Full-thickness skin loss with subcutaneous fat exposure
[Treatment: Sharp debridement and it will likely need a myocutaneous flap.]
Arsenical keratosis is associated with which type of skin cancer?
Squamous cell carcinoma
[UpToDate: Different types of arsenic-related skin lesions have been described in the West Bengal and Bangladesh chronic poisonings. Hyperpigmentation or hypopigmentation can be an early manifestation. Hyperkeratoses and scaling, particularly diffusely on the palms and soles, also are quite characteristic. Eczematous lesions have also been described. Skin manifestations, particular maculopapular eruptions, have been described following an acute curry-poisoning incident, and included maculopapular eruptions (sometimes in intertriginous areas), nail changes (Mee’s or Beau’s Lines), and periungual pigmentation.
Ingestion of inorganic arsenic increases the risk of developing skin cancers. Lesions commonly described are multiple squamous cell carcinomas, arising from the arsenic hyperkeratotic warts, as well as basal cell carcinomas arising from cells not associated with hyperkeratinization.]
What 2 treatments can be given for systemic melanoma?
- IL-2
- Tumor vaccines
[UpToDate: High-dose interleukin-2 (IL-2) was the first treatment to modify the natural history of patients with metastatic melanoma and may have resulted in cure in a small fraction of patients. However, its severe toxicity limited its application to carefully selected patients treated at centers with experience in managing the side effects of treatment.
More recent research led to the development of immunotherapy using checkpoint inhibitors (the anti-programmed cell death 1 [PD-1] antibodies [pembrolizumab, nivolumab] and the anti-cytotoxic T-lymphocyte-associated protein 4 [CTLA-4] antibody [ipilimumab]) and targeted therapy (inhibition of the BRAF and/or MEK genes). Both checkpoint inhibitor immunotherapy and targeted therapy prolong progression-free and overall survival compared with chemotherapy, which has not been proven to increase overall survival.]
What percent of basal cell carcinoma of the skin occurs on the head and neck?
80%
[UpToDate: Approximately 70 percent of BCCs occur on the face, consistent with the etiologic role of solar radiation. Fifteen percent present on the trunk, and only rarely is BCC diagnosed on areas like the penis, vulva, or perianal skin.]
Which 2 non-cytotoxic agents can be used for desmoid tumors if surgery is not a viable option?
- Sulindac
- Tamoxifen
[UpToDate: Options for systemic therapy include noncytotoxic therapy (a nonsteroidal antiinflammatory agent [NSAID], tamoxifen), radiation therapy, targeted therapy with imatinib, or cytotoxic chemotherapy.]
What is the treatment for a glomus cell tumor?
Tumor excision
[Glomus cell tumors are benign.]
[UpToDate: Treatment of glomus tumors is surgical excision. The location of the glomus tumor under the nail plate must be marked before anesthetic injection or tourniquet application, since exsanguination precludes the visualization of the tumor. To expose the tumor, a partial nail plate avulsion, such as a trap door avulsion or a lateral curled nail avulsion, is preferred.
In the trap door avulsion, the nail plate remains attached proximally over the proximal nail matrix, with full access to the underlying hyponychium, nail bed, and distal nail matrix. This avulsion technique is less frequently associated with postoperative complications, such as paronychia and pterygium than complete avulsion.
When complete exposure of the proximal matrix and eponychium is necessary, the lateral curled nail plate avulsion is ideal. The lateral curled avulsion is best performed using a hemostat, first to undermine the isolated lateral portion of the nail apparatus and then to clamp and roll the loosened nail plate away from the nail sulcus.
Following nail plate avulsion, the tumor is dissected from the surrounding tissues with blunt curved scissors. The nail bed defect can be closed with absorbable sutures if larger than 3 to 4 mm. The avulsed portion of the nail plate is often replaced and secured to the lateral and distal nail folds to protect the surgical site. Although the nail plate will not reattach permanently, it will protect the wound for several weeks until it is pushed out by a newly growing nail plate. The entire glomus tumor must be enucleated to prevent recurrence. Glomus tumors that occur in or under the nail matrix are the most difficult to excise and have the highest risk of recurrence because of incomplete removal.
A nail bed margin approach has also been proposed. A nail bed margin incision on the side of the tumor is performed under an operating microscope and the nail bed is dissected and elevated to expose the tumor. The tumor is carefully enucleated and resected completely using microsurgical scissors to minimize damage to the nail bed. The nail bed flap is then placed back into its original position and sutured.]
Which type of sweat gland produces milky sweat?
Apocrine sweat glands
[Highest concentration of glands in palms and soles; most sweat is the result of sympathetic nervous system via acetylcholine.]
What are the recommended negative margins for resection of basal cell carcinoma of the skin?
0.3-0.5 cm margins
[XRT and chemotherapy may be of limited benefit for inoperable disease, metastases, or neuro/lymphatic/vessel invasion.]
[UpToDate: Because surgical excision of truncal, extremity, or small facial BCCs on the head or neck with 4 to 5 mm margins has been associated with five-year cure rates exceeding 95 percent, 4 mm surgical margins are commonly used for the excision of these lesions.
However, the results of a 2010 meta-analysis of 89 studies on conventionally excised BCCs that excluded studies of previously excised or irradiated lesions and data on morpheaform BCCs (all features associated with increased risk for tumor recurrence) suggest that 3 mm surgical margins may be only slightly less efficacious. Rates of pathologically confirmed complete excisions were similar for surgical margins between 3 and 5 mm, and mean recurrence rates for BCCs excised with 5 mm, 4 mm, 3 mm, and 2 mm surgical margins were 0.4, 1.6, 2.6, and 4 percent, respectively.]
What is the treatment for a desmoid tumor?
Surgery if possible
[Chemotherapy (sulindac, tamoxifen) if vital structures are involved or too much bowel would need to be resected.]
[UpToDate:
Extraabdominal and abdominal wall tumors
- Observation is an appropriate option in asymptomatic patients who may be reliably followed. If desmoids remain unchanged or shrink, observation may be continued. If the tumor increases in size or becomes symptomatic, if there is imminent risk to adjacent structures, or if the desmoid creates cosmetic concerns, treatment should be pursued.
- We suggest complete surgical excision as the treatment of choice for a potentially resectable extraabdominal (extremity, trunk, breast) or abdominal wall desmoids in a patient who is medically able to tolerate surgery and if resection can be accomplished without major functional or cosmetic deficit (Grade 2B). Controversy remains as a number of patients will fare well without surgical intervention, so a more conservative approach appears rational for relatively static lesions.
- Primary radiation therapy (RT) is an appropriate option for patients who need treatment but are not good surgical candidates, those who decline surgery, and those for whom surgical morbidity would be excessive. An alternative approach is initiation of systemic treatment in these patients, especially if they are young and there are concerns about the potential for late toxicity from RT.
- For a recurrent desmoid tumor, our preference is observation or surgical resection. However, systemic therapy or RT alone are reasonable alternative treatments in selected patients who are thought to have a higher morbidity from repeat operation and an increased probability of positive margins. For patients who undergo surgery for a recurrent desmoid, we suggest observation or postoperative RT if the margins are positive (Grade 2C).
Intraabdominal desmoid, Gardner’s syndrome
- For patients who have large intraabdominal desmoid tumors, particularly in the setting of Gardner’s syndrome, surgery is still a standard approach for resectable tumors. However, the infiltrative nature of the desmoid in this situation often precludes surgery, and the surgical margins are often positive. Medical therapy in lieu of surgery is a viable option for patients with more difficult tumors such as those involving the mesentery, major vessels, or other critical structures.
- Surgery with or without RT is an appropriate option if there is no response to medical therapy. In this situation, consideration should be given to use of intraoperative electron beam therapy as a component of the treatment.
- Management of recurrence of an intraabdominal tumor in patients with Gardner’s syndrome is challenging because recurrences tend to become more frequent and aggressive with each surgical intervention. Most of these patients are managed with systemic therapy rather than additional local measures.]
What should be done about clinically positive nodes in basal cell carcinoma of the skin?
Regional adenectomy
What resection margins are required for melanoma in situ or thin lentigo maligna (Hutchinson’s Freckle)
0.5 cm margins are ok
[This type of melanoma is just in the superficial papillary dermis.]
[UpToDate: Lentigo maligna is a slowly evolving type of melanoma in situ that typically occurs in the sun-damaged skin of the face and neck of older individuals. The risk of progression to invasive lentigo maligna melanoma ranges from 5 to 20 percent. The development of darker pigmentation, sharper borders, or elevated or nodular areas are clinical signs of progression. There are no randomized trials evaluating treatment for lentigo maligna. We suggest surgical excision with margins of 5 to 10 mm, depending upon lesion size and location (Grade 2C). If available, surgical techniques that allow complete margin control such as staged excision with permanent sections (“slow” Mohs) are a preferred option. Among nonsurgical treatments for lentigo maligna, options include radiation therapy with multifractionated high voltage regimens or grenz rays or soft x-rays for large lesions in older patients for whom surgical removal and reconstruction would be difficult.]
Which epidermal cell type is of neuroectodermal (neural crest cell) origin?
Melanocytes
Which cells types are contained in a xanthoma?
Lipid-laden cells and histiocytes
[Treatment is excision]
[UpToDate: For xanthomas occurring in association with hyperlipidemia, it is hypothesized that when serum levels of lipoproteins are substantially elevated, extravasation of lipoproteins through dermal capillary blood vessels with subsequent engulfment by macrophages leads to the lipid-laden cells found in xanthomas, which has been demonstrated using electron microscopy.
Cutaneous xanthomas associated with hyperlipidemia often improve with treatment of the underlying lipid abnormality. Surgical and destructive treatments are the primary therapeutic options for xanthelasma in normolipidemic patients and for verruciform xanthomas.]
Xeroderma pigmentosum is a risk factor for which form of skin cancer?
Melanoma
Rank the following types of skin cancer in order of most to least frequently metastatic?
- Squamous cell carcinoma
- Basal cell carcinoma
- Melanoma
- Melanoma
- Squamous cell carcinoma
- Basal cell carcinoma
What is the treatment for hidradenitis?
Antibiotics and improved hygiene
[May need surgery to remove skin and associated sweat glands.]
[UpToDate: The level of disease severity strongly influences the approach to the treatment of Hidradenitis suppurativa/acne inversa (HS/AI). Patients with Hurley stage I HS/AI present with single or multiple nodules and abscesses without associated sinus tracts or scarring. For these patients, we suggest daily treatment of the involved areas with topical clindamycin (Grade 2B). We manage acute, inflamed lesions with punch debridement (mini-unroofing). If punch debridement is not feasible, topical resorcinol is an alternative patient-administered treatment that we often employ for the treatment of acute lesions. Intralesional corticosteroids and short courses of systemic antiinflammatory antibiotic therapy also can be beneficial for reducing symptoms.
Patients with Hurley stage II HS/AI exhibit recurrent inflamed nodules and abscesses, some of which lead to sinus tracts and scarring. We approach treatment with a combination of medical and surgical therapy. For the medical treatment of the inflammatory component of Hurley stage II disease, we suggest treatment with systemic antibiotic therapy (Grade 2C). We usually treat with doxycycline and continue treatment for two to three months or more. Combination therapy with clindamycin and rifampin is typically reserved for patients who fail to respond to other antibiotic regimens. Concurrent use of anti-androgenic agents may provide additional benefit.
Incision and drainage does not alter the clinical course of HS/AI and should only be performed when immediate relief of pain from a tense abscess is required. Incision and drainage should not be used for routine management of HS/AI. Punch debridement (for small acute, nodular lesions) and unroofing (for larger areas of involvement) are our preferred surgical interventions.]
What is the most common type of benign skin cyst?
Epidermal inclusion cyst
[Has completely mature epidermis with creamy keratin material.]
[UpToDate: Epidermoid cysts, also called epidermal cysts, epidermal inclusion cysts, or, improperly, “sebaceous cysts,” are the most common cutaneous cysts. They can occur anywhere on the body and typically present as skin-colored dermal nodules, often with a clinically visible central punctum. The size ranges from a few millimeters to several centimeters in diameter. Infected, fluctuant cysts tend to be larger, more erythematous, and more painful than sterile inflamed cysts, although an intense inflammatory response to cyst rupture may also present as a fluctuant nodule.
Epidermoid cysts unusual in number and location (extremities rather than face, base of ears, and trunk) may be seen in the setting of Gardner syndrome, a rare inherited condition characterized by familial adenomatous polyposis of the colon associated with a number of extracolonic abnormalities.
The cyst wall consists of normal stratified squamous epithelium derived from the follicular infundibulum. The cyst may be primary or may arise from the implantation of the follicular epithelium in the dermis as a result of trauma or comedone. Lesions may remain stable or progressively enlarge. Spontaneous inflammation and rupture can occur, with significant involvement of surrounding tissue. There is no way to predict which lesions will remain quiescent and which will become larger or inflamed.]
Which mechanoreceptor (sensory nerve) in the skin is responsible for sensing pressure?
Pacinian corpuscles
Soft tissue sarcoma chemotherapy is based with which drug?
Doxorubicin
[UpToDate: The only single agents that are consistently associated with response rates of more than 20 percent in metastatic STS are doxorubicin, epirubicin, and ifosfamide. Even for these agents, the range of objective activity between various small (and even larger) trials is impressive, demonstrating the variability in disease sensitivity and the fact that any given STS patient population could serve as an important confounding variable for interpretation of drug efficacy.
The sensitivity of STS to systemic chemotherapy was first demonstrated with single agent doxorubicin in the early 1970s, and subsequent studies suggested a dose-response relationship. The threshold dose for optimal activity appears be ≥60 mg/m2 per cycle, usually administered once every three weeks, with lower doses associated with inferior antitumor activity. It is difficult to demonstrate a clinically meaningful dose-response relationship with single agent doxorubicin at doses beyond 75 mg/m2 per cycle, which has become the standard dose.
Even in modern multi-institutional series using 70 to 80 mg/m2 per cycle, there is significant variability in reported response rates, which range from 10 to 25 percent. The vast majority are partial, rather than complete, responses.
Doxorubicin is associated with reversible myelosuppression, mucositis, alopecia, nausea and vomiting, and both acute and chronic cardiotoxicity. This drug has a relatively narrow therapeutic index; even small variations of dose beyond 75 mg/m2 can drastically worsen patient tolerance, and even 75 mg/m2 is considered by many patients to be significantly more toxic than 60 mg/m2. Infusional, rather than bolus, administration reduces the likelihood of cardiotoxicity. Other methods to diminish cardiotoxicity include the concomitant use of the cardioprotectant dexrazoxane or the use of liposome-encapsulated doxorubicin.]
What is the diagnostic approach to a > 2 cm suspicious skin lesion or any sized suspicious skin lesion in an aesthetically sensitive area?
Incisional biopsy (punch biopsy)
[If pathology comes back as melanoma then need resection with margins.]
What is the most common malignancy in the United States?
Basal cell carcinoma
[4 times more common than squamous cell skin cancer.]
[UpToDate: Estimates of the incidence of BCC are imprecise since there is no cancer registry that collects data on BCC. The American Cancer society estimates that more than two million nonmelanoma skin cancers were treated in the United States in 2006, of which the majority would have been BCC. A population-based study with several methodologic limitations estimated a higher number of lesions, reporting that approximately 3.5 million nonmelanoma skin cancers were treated in the United States during the same year.]
Which is premalignant: Actinic keratoses or Seborrheic keratoses?
Actinic keratoses
[Seborrheic keratoses are not premalignant and often occur on the torso in elderly populations. Actinic keratoses are premalignant and occur in sun-damaged areas. Actinic keratoses need an excisional biopsy if suspicious.]
[UpToDate: The likelihood of progression of an individual AK to SCC is low. Estimates of annual rates of transformation have ranged from 0.03 to 20 percent. The following data come from two of the largest studies:
- An Australian study that evaluated 1689 adults over the age of 40 who had more than 20,000 AKs found the risk for transformation of an individual AK to SCC within one year to be less than 0.1 percent.
- Analysis of data from a cohort of patients in a United States trial designed to investigate the use of topical tretinoin for skin cancer prevention in patients with at least two prior keratinocyte carcinomas revealed a rate of transformation to invasive or in situ SCC of 0.6 percent in 6015 AKs followed for one year and a rate of 2.6 percent in 1480 lesions followed for four years.
Progression of AKs to basal cell carcinoma (BCC) occurred in 0.5 percent of AKs within one year and in 1.6 percent within four years. However, it is unclear whether this finding was related the misdiagnosis of early BCCs as AKs or was true disease progression.
Although few AKs progress to SCC, data from these studies suggest that approximately 60 percent of cutaneous SCCs arise from preexisting AKs. This observation supports a close relationship between these lesions.]
Which test should be ordered before performing a biopsy on patients with a suspected soft tissue sarcoma?
MRI to rule out vascular, neuro, or bone invasion
[UpToDate: Histologic examination of a soft tissue mass is essential for diagnosis and treatment planning. There are several methods for obtaining a biopsy, but ideally, the biopsy should be performed after an MRI has been obtained as post-procedural edema may make the MRI difficult to interpret.]
What is the diagnostic approach to a <2 cm skin lesion suspicious for melanoma?
Excisional biopsy (tru-cut core needle biopsy)
[If pathology comes back as melanoma then need resection with margins.]
What is the most common soft tissue sarcoma?
Malignant fibrous histiosarcoma (also known as pleomorphic undifferentiated sarcoma)
[#2 is liposarcoma]
Which disease is associated with neuromas?
Neurofibromatosis (Von Recklinghausen’s disease)
Which ultraviolet subtype is responsible for chronic sun damage?
UV-B
What is the most important determinant of transverse rectus abdominus myocutaneous (TRAM) flap viability?
Periumbilical perforators
[UpToDate: The skin and subcutaneous fat of the lower abdomen receive their blood supply from the underlying muscle via perforating vessels from the superior and inferior epigastric arteries, which arborize through the rectus abdominus muscle.]
What is the description of a stage IV pressure sore?
Involves bony cortex and muscle
[Treatment: Myocutaneous flap.]
Cafe-au-lait spots, axillary freckling, and peripheral nerve/CNS tumors are characteristics of which disease?
Von Recklinghausen’s disease (Neurofibromatosis type 1)
Which type of sweat gland produces aqueous sweat?
Eccrine sweat glands
[Responsible for thermal regulation, usually hypotonic.]
Do clinically positive nodes always need to be resected in melanoma?
Yes
[Clinically positive nodes are usually nontender, round, hard, and 1-2 cm in size]
[UpToDate: Lymphatic mapping with sentinel lymph node biopsy (SLNB) is the standard clinicopathologic approach to evaluate regional lymph nodes in patients without clinical evidence of lymph node involvement. For patients with clinically negative regional lymph nodes, the decision whether or not to perform an SLNB is based upon the likelihood of regional lymph node involvement. For patients with a melanoma 0.75 mm thick or greater, we recommend performing an SLNB. This criterion includes patients with intermediate and thick primary lesions, as well as those with thin melanomas 0.75 to 1.00 mm thick. For patients with a melanoma less than 0.75 mm thick and one or more factors placing the patient at increased risk for nodal involvement (ulceration of the primary tumor, a mitotic rate ≥ 1/mm2, or lymphovascular invasion), we favor performing an SLNB. However, the absolute risk of a positive sentinel lymph node is small in this group, and a detailed discussion with the patient is needed to discuss the potential risks and benefits. SLNB is not indicated for patients with a melanoma <0.75 mm and no other factors placing the patient at increased risk for nodal involvement.
For patients with a positive SLNB, we suggest completion lymph node dissection (Grade 2B). In patients with a positive SLNB, adjuvant therapy with interferon alfa should be limited to patients with a life expectancy of ten years in the absence of serious comorbidity. Participation in a clinical trial is also an option, especially for patients not considered candidates for or uninterested in receiving interferon alfa.
For patients who present with clinically apparent regional lymph node involvement that is confirmed cytologically (fine needle aspirate) or histologically, we recommend therapeutic regional lymphadenectomy (Grade 1B). Partial dissection or lymph node sampling is not considered an acceptable alternative because of the propensity of melanoma to spread microscopically to other nodes in the basin.]
The dermis is primarily composed of what?
Structural proteins such as collagen
What additional treatment is required for all anterior head/neck melanomas > 1 mm deep?
Superficial parotidectomy
[20% metastasis rate to parotid.]
Transverse rectus abdominus myocutaneous (TRAM) flap relies on which vessels for blood supply?
Superior epigastric vessels
[Complications include flap necrosis, ventral hernia, bleeding, infection, abdominal wall weakness.]
[UpToDate: One of the most commonly used autologous tissue reconstructions is the TRAM (transverse rectus abdominus myocutaneous) flap. The flap is comprised of an ellipse of lower abdominal skin, subcutaneous fat, and muscle. The skin and subcutaneous fat of the lower abdomen receive their blood supply from the underlying muscle via perforating vessels from the superior and inferior epigastric arteries, which arborize through the rectus abdominus muscle. The pedicled TRAM flap is based on the superior epigastric artery and vein, and is rotated into the breast pocket with the superior portion of the muscle (and blood supply to the flap) still attached to the costal margin. TRAM flaps use a transversely oriented skin island from the lower abdomen. This design incorporates an abdominoplasty (“tummy tuck”) as part of the donor site closure. Using a single rectus muscle, up to 75 percent of the infraumbilical skin and fat can be harvested for the pedicled TRAM flap.]
What is the most aggressive form of basal cell carcinoma of the skin?
Morpheaform type
[It has collagenase production.]
[UpToDate: Morpheaform or sclerosing BCCs constitute 5 to 10 percent of BCCs. These lesions are typically smooth, flesh-colored, or very lightly erythematous papules or plaques that are frequently atrophic; they usually have a firm or indurated quality with ill-defined borders. Some authors group morpheaform, infiltrative, and micronodular as “aggressive-growth” BCC, because they behave similarly. Infiltrative and micronodular subtypes are less common than the morpheaform BCC.]
What is the subtype of childhood rhabdomyosarcoma (The most common soft tissue sarcoma in kids) that has the worst prognosis?
Alveolar subtype
[Embryonal subtype is the most common.]
[UpToDate: In the past, the presence of any alveolar pattern was sufficient to categorize a tumor as an alveolar RMS. However, in most recent protocols the Soft Tissue Sarcoma committee of the Children’s Oncology Group has recommended that the lesion must have a predominant (>50 percent) alveolar component (or bear the characteristic translocations t(1;13) or t(2;13)) to be subtyped as alveolar.
The typical appearance is that of fibrovascular septae that are lined with densely packed ovoid to round tumor cells and separated by pseudo-alveolar spaces, which vaguely resemble pulmonary alveoli. Frequently, the “loosely adherent” rhabdomyoblasts are shed into these pseudo-alveolar spaces. Less well-differentiated alveolar RMS may feature only a suggestion of fine fissuring or microalveoli; molecular techniques may aid in the diagnosis of such cases.]
What are the 2 most common sites for Kaposi’s sarcoma?
- Oral mucosa
- Pharyngeal mucosa
[Symptoms in these regions include bleeding and dysphagia. Kaposi’s sarcoma is a vascular sarcoma.]
[UpToDate: During the course of the disease (rarely initially), mucous membranes of mouth and gastrointestinal (GI) tract and regional lymph nodes may be affected. GI tract involvement is usually asymptomatic, but bleeding, diarrhea, protein-losing enteropathy, intussusception, and perforation have been reported. In general, GI tract/oral mucosal involvement is less common than with AIDS-related KS, affecting ≤10 percent of patients.
However, in one report, an extraordinary 82 percent of Greek patients (71 of 87) with biopsy-proven CKS who were investigated with upper gastrointestinal endoscopy had GI lesions; all 71 had stomach lesions, 19 had esophageal lesions, eight had lesions of the proximal duodenum, and two had both esophageal and duodenal lesions. Although this finding could be interpreted as suggesting the need for screening endoscopy in patients with newly diagnosed CKS, it also supports the view that the presence of asymptomatic GI involvement probably has little effect on prognosis. In practice, routine endoscopy is not performed in people diagnosed with CKS who do not have symptoms referable to the GI tract.]
What is the treatment for hyperhidrosis?
Thoracic sympathectomy if refractory to variety of antiperspirants
[Symptoms include increased sweating that is especially noticeable in the palms.]
[UpToDate: When choosing treatments for primary focal hyperhidrosis, the patient’s goals in therapy should be understood and the side effects associated with each therapy should be carefully discussed. Conservative measures should be tried before progressing to more invasive treatments. The severity and location of hyperhidrosis helps guide the choice of therapies.
Patients with axillary, palmar, or plantar hyperhidrosis should initially be treated with topical antiperspirants such as aluminum chloride hexahydrate in alcohol. Skin irritation is a common side effect. Iontophoresis is an alternative initial treatment for patients with palmar or plantar hyperhidrosis.
Botulinum toxin injection is an effective second-line treatment for axillary hyperhidrosis and microwave thermolysis is a less-widely available alternative. Palmar and plantar hyperhidrosis can also be treated with botulinum toxin, but the injections are quite painful on the hands and feet, and a significant percentage of patients develop temporary local muscle weakness.
Patients with axillary hyperhidrosis who cannot be managed effectively with first- or second-line treatments may benefit from suction curettage, a local surgical intervention. If this is not feasible or effective, our preferred next therapy is systemic medication. Endoscopic thoracic sympathectomy (ETS) is an additional option. The selection of these treatments should follow careful consideration of the associated risks and side effects.
Patients with palmar hyperhidrosis who cannot be managed effectively with antiperspirants, iontophoresis, or botulinum toxin are candidates for systemic medications or sympathectomy.
First-line therapies for craniofacial hyperhidrosis include topical antiperspirants. Patients who cannot be managed with topical therapy may benefit from botulinum toxin injections or oral medication.]
Melanoma of what depth requires that clinically negative nodes be confirmed negative with a sentinel lymph node biopsy?
> 1 mm deep
[UpToDate: SLNB is the standard approach for the management of patients with melanoma in whom there is a substantial risk of regional node metastasis. The likelihood of detecting metastatic deposits in a SLNB increases with the thickness of the primary lesion, providing a rationale for deciding which patients may benefit from this procedure. The generally accepted approach focuses on tumor thickness and related risk factors: SLNB is indicated for melanomas ≥1 mm thick. For lesions 1.01 to 2.0, 2.01 to 4.0, and >4 mm, the risk of regional lymph node metastasis is approximately 12, 28, and 44 percent, respectively. For thin melanomas (ie, <1 mm thick), the overall risk of regional node metastases in patients undergoing SLNB is estimated to be about 5 percent.
A retrospective review from the Sentinel Lymph Node Working Group of 1250 patients with thin melanoma who underwent SLNB provides the most extensive data in this group. Breslow thickness ≥0.75 mm, Clark level ≥IV, and ulceration were associated with lymph node metastases in 6.3, 7.0, and 11.6 percent of cases, respectively, and these factors were all statistically significant on multivariate analysis. In contrast, melanomas <0.75 mm thick had positive SLNBs less than 5 percent of the time regardless of the Clark level or the presence of ulceration. The low risk of a positive SLNB in patients with a primary lesion <0.75 mm and without any other risk factors has also been seen in earlier studies. In summary, for patients with a melanoma 0.75 mm thick or greater, we recommend performing an SLNB.]
Which type of collagen is predominant in skin?
Type-I collagen
[Composes 70% of dermis. Gives tensile strength.]
Which test should be ordered on all patients with a newly diagnosed soft tissue sarcoma?
Chest xray to rule out lung metastases
[UpToDate: Given the propensity for lung metastases, chest imaging is recommended for newly diagnosed patients with soft tissue sarcoma of the extremity/trunk. While CT scan is often preferred due to its greater sensitivity in detecting small lung nodules, it is unknown whether this provides benefit over chest X-ray (CXR) alone. Both modalities are considered highly appropriate for this purpose by the American College of Radiology.]
What is the treatment for localized Kaposi’s sarcoma?
XRT or intra-lesional vinblastine
[UpToDate: All forms of KS, including CKS, are very sensitive to radiotherapy (RT). However, because of the multifocal nature of the disease, the persistence of HHV-8 even with successful local lesion control, and the tendency for new lesions to develop in nonirradiated areas, there is no consensus as to the place of RT in the therapeutic armamentarium (particularly when to choose RT over systemic therapy) or the optimal radiation technique.
Intralesional injection of chemotherapy (most often vinblastine, but sometimes bleomycin) leads to local regression of cutaneous KS lesions. In our experience, such injections, while capable of eradicating injected tumors, can be painful and lead to local scarring.]
Which type of melanoma is very aggressive and appears on the palms and soles of African Americans?
Acral lentiginous
[UpToDate: Acral lentiginous melanomas are the least common variant of radial growth phase melanomas, comprising fewer than 5 percent of all melanomas. They arise most commonly on palmar, plantar, subungual, and occasionally, mucosal surfaces. The acral lentiginous subtype is the most common type of malignant melanoma among Asians and dark-skinned individuals, with a particular predilection for the soles of the feet. Clinically, an acral lentiginous melanoma appears as a dark brown to black, unevenly pigmented patch. Areas of regression manifest as foci of gray-white discoloration. If a lesion becomes raised or develops ulceration, the likelihood of invasion should be considered. However, some tumors that invade along the peri-eccrine adventitial dermis may appear flat clinically even if they have encroached upon subcutaneous adipose tissue.]
Which intra-abdominal tumor is associated with Gardner’s syndrome and retroperitoneal fibrosis, and often encases bowel, making it difficult to get an en bloc resection?
Intra-abdominal desmoid tumor
[UpToDate: Most desmoids arise sporadically, although between 5 and 15 percent are associated with familial adenomatous polyposis (FAP). FAP is caused by mutations in the APC (adenomatous polyposis coli) gene, located on chromosome 5q21-q22.]
How do keratinocytes get melanin?
Melanocytes have dendritic processes that transfer melanin to neighboring keratinocytes via melanosomes
[The density of melanocytes is the same among races; the difference is in melanin production.]
Peripheral palisading of nuclei and stromal retraction on pathology are characteristics of which kind of skin cancer?
Basal cell carcinoma
Visceral and retroperitnoeal sarcomas are most commonly which 2 types?
- Leiomyosarcoma
- Liposarcoma
[UpToDate: Retroperitoneal STS (RPS) are relatively uncommon, constituting only 10 to 15 percent of all STS. The average annual incidence of RPS is approximately 2.7 cases per million population. Patients usually present in their 50s, although the age range is broad. The frequency is approximately equal in men and women.
Approximately 80 percent of the neoplasms that arise within the retroperitoneal space are malignant. Furthermore, the majority of patients who present with a primary retroperitoneal, extravisceral, unifocal soft tissue mass will be found to have a sarcoma. In adults, the most common histologic types of RPS are liposarcomas and leiomyosarcomas, followed by undifferentiated/unclassified STS (a subset of which are pleomorphic undifferentiated sarcomas). Historically, undifferentiated/unclassified STS were included within the older term “malignant fibrous histiocytoma.” A variety of other histologic types may be observed, but they are much less common in the retroperitoneum than in other primary sites. Approximately one-half of all RPS are high-grade tumors, although this varies according to histology. The majority of retroperitoneal liposarcomas are low- to intermediate-grade lesions.]
How should keratoacanthomas be treated?
- If small, excise
- If large, biopsy and observe
[Always biopsy if not going to excise in order to rule out cancer. It will involute spontaneously over a few months.]
[UpToDate: We suggest surgical excision as the first-line treatment for KA (Grade 2C). Surgical excision provides pathologic confirmation of tumor removal. Conventional surgical excision may be performed on most lesions. Lesions in areas where tissue sparing is desired, such as the central face, can be treated with Mohs surgery.
Other effective options for the treatment of KA include electrodesiccation and curettage (ED&C), intralesional pharmacologic therapy, radiation therapy, and topical therapy. A disadvantage of these interventions compared with surgical excision is the lack of histopathologic confirmation of tumor removal.
For patients who elect to defer therapy and prefer to wait for lesion spontaneous resolution, close clinical follow-up should be implemented. Spontaneous resolution may take several months or longer. If lesions deviate from the expected clinical course, surgical excision to confirm the diagnosis is indicated.
The local therapies commonly utilized for the management of solitary KA may be impractical for patients with hundreds or thousands of lesions. For patients with numerous KAs, we suggest treatment with oral retinoid therapy (Grade 2C).]
Which mechanoreceptor (sensory nerve) in the skin is responsible for sensing warmth?
Ruffini’s endings
Which kind of skin cancer usually has a pearly appearance, rolled borders, and slow/indolent growth?
Basal cell carcinoma
What is the description of a stage I pressure sore?
Erythema and pain without skin loss
What is Bowen’s disease?
Squamous cell carcinoma in situ that is associated with HPV
[10% turn into invasive squamous cell carcinoma.]
[UpToDate: Cutaneous SCC in situ typically presents as a well-demarcated, scaly patch or plaque. Lesions are often erythematous but can also be skin colored or pigmented. SCC in situ lesions tend to grow slowly, enlarging over the course of years. Unlike the inflammatory disorders that may resemble SCC in situ, lesions are usually asymptomatic.]
What do the ABCDE signs of melanoma stand for?
- A: Asymmetry (angulations, indentations, notching, ulceration, bleeding)
- B: Borders that are irregular
- C: Color change (ie darkening. Blue color is the most ominous)
- D: Diameter increase
- E: Evolution over time
Where do lipomas most commonly occur?
Back, neck, between shoulders
[Lipomas are common but rarely malignant.]
[UpToDate: Lipomas present as soft, painless subcutaneous nodules ranging in size from 1 to >10 cm. They occur most frequently on the trunk and upper extremities and can be round, oval, or multilobulated. Frequently, patients may have more than one lipoma, and occasionally they may have a genetic condition (familial multiple lipomatosis) characterized by the development of multiple lipomas in several family members. Malignant transformation of a lipoma into a liposarcoma is rare.]
From which cells (epiphyseal, metaphyseal, diaphyseal) of the bone does osteosarcoma originate?
Metaphyseal cells
[Usually occurs in children.]
[UpToDate: Bone sarcomas account for approximately 6 percent of all childhood cancers, and osteosarcomas account for approximately 3 percent of childhood cancers overall. However, osteosarcoma is the most common primary malignant bone tumor affecting children and young adults. Osteosarcomas comprise 56 percent of all bone cancers in individuals under the age of 20, while Ewing sarcoma accounts for 34 to 36 percent, and chondrosarcomas are responsible for less than 10 percent.
In children, the peak incidence is between 13 and 16 years of age, a time that appears to coincide with the adolescent growth spurt. For unclear reasons, osteosarcomas are more common in boys than in girls, and in blacks and other races compared with Caucasians. The most common sites of osteosarcoma in children are the metaphyses of long bones, especially the distal femur, proximal tibia, and proximal humerus.]
What is the description of a stage II pressure sore?
Partial skin loss with yellow debris
[Treatment: Local measures, keep pressure off.]
Where are glomus cell tumors usually located?
Terminal aspect of the digit
[Benign]
[UpToDate: Glomus tumor is a rare, benign neoplasm composed of cells resembling smooth cells of the normal glomus body. It is usually located in areas of the skin that are rich in glomus bodies (eg, the subungual regions of digits or the deep dermis of the palm, wrist, forearm, and foot). Glomus tumors of the fingers and toes occur in approximately 5 percent of patients with neurofibromatosis type 1 (NF1) and are considered NF1-associated neoplasms.
Glomus tumor presents as a pink or purple vascular papule or nodule associated with paroxysmal pain, cold sensitivity, and tenderness. Subungual glomus tumors are particularly painful. Nail dystrophy and/or a bluish-red flush are typically seen.]
What is the usual initial treatment for verruca vulgaris (warts)?
Liquid nitrogen
[Warts are viral in origin, contagious, autoinoculable, and can be painful.]
[UpToDate: For patients with common or plantar warts, we suggest topical salicylic acid as first-line treatment (Grade 2A). Cryotherapy is an alternative first-line treatment; however, tolerance for associated pain and other side effects (eg, hypopigmentation) limits use in some patients.
The best approach to patients with severe or refractory cutaneous warts is unclear. Topical immunotherapy with contact allergens, intralesional bleomycin, or topical or intralesional 5-fluorouracil may be useful for such patients.
Flat warts are usually treated with cryotherapy or topical agents such as tretinoin, imiquimod, or 5-fluorouricil. Filiform warts are often most easily treated with cryotherapy or surgery.]
Which skin tumor is painful and composed of blood vessels and nerves?
Glomus cell tumor
[Benign]
[UpToDate: Glomus tumor is a rare, benign neoplasm composed of cells resembling smooth cells of the normal glomus body. It is usually located in areas of the skin that are rich in glomus bodies (eg, the subungual regions of digits or the deep dermis of the palm, wrist, forearm, and foot). Glomus tumors of the fingers and toes occur in approximately 5 percent of patients with neurofibromatosis type 1 (NF1) and are considered NF1-associated neoplasms.
Glomus tumor presents as a pink or purple vascular papule or nodule associated with paroxysmal pain, cold sensitivity, and tenderness. Subungual glomus tumors are particularly painful. Nail dystrophy and/or a bluish-red flush are typically seen.]
Which genetic syndrome for soft tissue tumors predisposes to the below tumor?
- Angiomyolipoma
Tuberous sclerosis
[UpToDate: TSC is characterized by the development of a variety of benign tumors in multiple organs, including the brain, heart, skin, eyes, kidney, lung, and liver. In addition, there is an increased risk of malignancy in TSC. Nearly all patients with TSC have one or more of the skin lesions characteristic of the disorder. Most patients with TSC have epilepsy, and one-half or more have cognitive deficits and learning disabilities; other common manifestations include autism, behavioral problems, and psychosocial difficulties. Collectively, these are termed TSC-associated neuropsychiatric disorders (TAND). These problems are usually associated with brain lesions including glioneuronal hamartomas (also called tubers), periventricular giant cell astrocytomas, and abnormalities of cerebral white matter detected on neuroimaging studies. However, there is a wide variety of phenotypes between and within families regarding the number and severity of TSC manifestations.]
Hidradenitis is caused by an infection of which sweat glands?
Apocrine sweat glands
[Usually occurs in the axilla and groin.]
[UpToDate: The pathogenesis of hidradenitis suppurativa (acne inversa, HS/AI) is not fully understood. However, an explosion of research has led to greater understanding of the processes involved. As opposed to early theories that implicated apocrine glands as the primary contributors to HS/AI, most authors now support follicle-centered theories for the pathogenesis of HS/AI. Follicular occlusion, follicular rupture, and an associated immune response appear to be important events in the development of the clinical manifestations of HS/AI. The basic principles that underlie follicle-centered theories are reviewed below.
Follicular occlusion is the most likely event responsible for the initial development of HS/AI lesions. Follicular occlusion appears to result from a hormonally-induced increase in ductal keratinocyte proliferation (follicular epithelial hyperplasia), leading to a failure of keratinocytes to shed properly from the follicular epithelium, causing follicular hyperkeratosis and plugging. Further study is necessary to explore the pathways that promote follicular occlusion in this disease. Proposed contributors have included the effects of hormones and nicotine on the follicular epithelium. In addition, we propose that anoxia within the follicular duct secondary to follicular epithelial hyperplasia contributes to the disruption of normal terminal differentiation of follicular keratinocytes, leading to follicular plugging.]
Which mechanoreceptor (sensory nerve) in the skin is responsible for sensing cold?
Krause’s end-bulbs
Which skin lesion grows rapidly, has rolled edges, and appears as a crater filled with debris?
Keratoacanthoma
[Debris is keratin. It is not malignant but can be confused with squamous cell carcinoma. It involutes spontaneously over months.]
[UpToDate: Keratoacanthoma (KA) is a cutaneous tumor that most commonly presents as a dome-shaped nodule with a central keratin-filled crater. KA most frequently develops on hair-bearing, sun-exposed skin. Middle-aged and elderly adults with fair complexions are most frequently affected.
A distinguishing feature of KA is a clinical course characterized by rapid initial growth followed by a variable period of lesion stability and subsequent spontaneous resolution. The recognition of this unique characteristic in a tumor with clinical and histopathologic features that closely resemble cutaneous squamous cell carcinoma has led to significant debate over the classification of this lesion.]
Where in the epidermis are melanocytes located?
In the basal layer of epidermis
What is the most common location for a desmoid tumor to occur?
Anterior abdominal wall
[Desmoid tumors have a high risk of local recurrence, but no distal spread.]
[UpToDate: Most desmoid tumors present as a deeply seated painless or minimally painful mass with a history of slow growth. Intraabdominal desmoids can present with intestinal obstruction, bowel ischemia, or functional deterioration in an ileoanal anastomosis (typically in a patient who has undergone colectomy for familial adenomatous polyposis [FAP]).
Desmoid tumors can develop at virtually any body site, but three main anatomic sites are described: trunk/extremity, abdominal wall, and intraabdominal (bowel and mesentery). In patients with FAP, intraabdominal desmoids predominate.
In non-FAP-associated cases, the most commonly involved areas are the shoulder girdle, hip-buttock region, and the extremities, where the location is usually deep in the muscles or along fascial planes. Desmoid tumors may be multifocal at one site (typically the extremity), but they rarely occur at different regions in the same patient.]
Which mechanoreceptor (sensory nerve) in the skin is responsible for tactile sense?
Meissner’s corpuscles
Which type of melanoma is the most aggressive, usually grows vertically first, is commonly bluish-black with smooth borders, and occurs anywhere on the body?
Nodular melanoma
[Most likely to have metastasized at the time of diagnosis.]
[UpToDate: By definition, nodular melanomas are vertical growth phase melanomas. They constitute 15 to 30 percent of all melanomas. Clinically, a nodular melanoma appears as a darkly pigmented, pedunculated or polypoid nodule, although amelanotic variants are infrequently seen.]
Which type of head and neck sarcoma most commonly occurs in the pediatric population?
Rhabdomyosarcoma
[Head and neck sarcoma are hard to treat given difficulty in obtaining resection margins due to proximity of vital structures. Post op XRT for positive or close margins as negative margins may be impossible to obtain.]
[UpToDate: Rhabdomyosarcoma (RMS) is the most common soft tissue tumor of childhood, and responsible for approximately one-half of all soft tissue sarcomas in this age group. However, they are rare, representing only 3 to 4 percent of pediatric cancers overall. Approximately 350 new cases are diagnosed in the United States each year, and the annual incidence in children, adolescents, and young adults under the age of 20 is 4.3 cases per one million.
Two-thirds of cases are diagnosed in children younger than six years of age, and there is a small male predominance (male to female ratio between 1.3 and 1.5). The incidence in African American patients is higher than in whites, most notably in 15 to 19 year olds. The incidence appears to be lower in Asians when compared with predominantly Caucasian populations.]
What is the most common malignancy in AIDS?
Kaposi’s sarcoma
[Kaposi’s sarcoma is associated with immunocompromised state. Though it is the most common malignancy in AIDS, it is rarel the cause of death.]
[UpToDate: AIDS-related or epidemic KS is the most common tumor arising in HIV-infected persons. KS is considered an AIDS-defining illness in the Centers for Disease Control and Prevention (CDC) guidelines. In the United States, KS was over 20,000 times more common in persons with AIDS than in the general population prior to the widespread use of potent antiretroviral therapy (ART), although its incidence has declined substantially since that time.]
Which kind of skin cancer is usually red-brown with overlying erythema, and has a papulonodular appearance with crust and ulceration?
Squamous cell carcinoma
[May have surrounding induration and satellite nodules.]
Which genetic syndrome for soft tissue tumors predisposes to childhood rhabdomyosarcomas and many other tumors?
Li-Fraumeni syndrome
[UpToDate: Li-Fraumeni syndrome is an inherited autosomal dominant disorder that is manifested by a wide range of malignancies that appear at an unusually early age. Li-Fraumeni syndrome is also known as the Sarcoma, Breast, Leukemia, and Adrenal Gland (SBLA) cancer syndrome. This cancer predisposition syndrome is inherited as an autosomal dominant disorder and is associated with abnormalities in the tumor protein p53 gene (TP53).
The Li-Fraumeni syndrome was originally described in 1969, based upon the identification of second soft tissue sarcomas in five families with index cases of rhabdomyosarcoma. These observations were subsequently extended to 24 kindreds in which 151 individuals developed cancer. These patients were characterized by a variety of bone and soft tissue sarcomas and breast cancer, approximately 80 percent of which occurred prior to 45 years of age. Other tumors that occurred disproportionately in this series included brain tumors, leukemia, and adrenocortical carcinoma, and there were six patients with cancer that was linked to antecedent radiation therapy. Other reports have confirmed the high frequency of early age at onset in patients with Li-Fraumeni syndrome, as well as the wide range of cancers that are seen.]
How are soft tissue sarcomas staged?
Staging is based on grade rather than size
[UpToDate: The most widely used staging system for soft tissue sarcomas is the TNM system developed by the International Union Against Cancer (UICC) and the American Joint Committee on Cancer (AJCC). The system uses tumor size (T), depth (superficial or deep), lymph node involvement (N), presence or absence of distant metastases (M), and histologic grade (G) in determining the stage grouping for soft tissue sarcomas.]
Should isolated melanoma metastases be resected if resection can be done with a low risk procedure?
Yes
[Resection of metastases has provided some patients with long disease-free intervals and is the best chance for a cure.]
[UpToDate: Patients with oligometastatic disease (ie, limited to one or a limited number of sites) should be evaluated for the possible surgical metastasectomy. Although widespread metastatic disease develops in most cases, complete resection of metastatic disease is associated with prolonged survival in some cases. Systemic therapy is indicated for patients in whom surgical metastasectomy is not appropriate.
The primary systemic therapy approaches for patients with metastatic melanoma are checkpoint-inhibitor immunotherapy (primarily the anti-PD1 antibodies pembrolizumab and nivolumab in combination with the anti-CTLA4 antibody ipilimumab) and targeted therapy against the mitogen activated protein kinase (MAPK) pathway. The appropriate choice and sequence of treatments for an individual patient is based upon the extent of disease, molecular characteristics of the tumor (presence of a driver mutation in BRAF or KIT), and patient performance status and comorbidity.]
Which tumor is benign but very locally invasive and arises in fascial planes (most commonly the anterior abdominal wall)?
Desmoid tumor
[Desmoids can occur during or following pregnancy and can also occur after trauma or surgery.]
[UpToDate: Desmoid tumors (also called aggressive fibromatosis, deep musculoaponeurotic fibromatosis, and also formerly termed fibrosarcoma grade I of the desmoid type) are locally aggressive tumors with no known potential for metastasis or dedifferentiation. The term “desmoid” originates from the Greek word “desmos,” meaning band or tendon-like, and was first applied in the 1800s to describe tumors with a tendon-like consistency.
Although they lack the capacity to establish metastases, desmoids are locally aggressive and have a high rate of recurrence even after complete resection. Tumor-related destruction of vital structures and/or organs can be fatal, particularly when these tumors arise in patients with familial adenomatous polyposis (FAP; Gardner’s syndrome).]
Osteosarcoma has a higher incidence in which region of the body?
Near the knee
[UpToDate: Bone resections fall into one of three categories, depending upon the anatomic site and the extent of the involved bone that needs to be excised. Because most bone sarcomas arise in the metaphysis of the long bones near a joint, the majority of resections, for tumors in the lower extremity, include both the segment of tumor-bearing bone and the adjacent joint (osteoarticular resection). Most often, the incision is performed through the joint (intraarticular resection); however, when the tumor extends along the joint capsule or ligamentous structures or invades the joint, the entire joint can be resected (extraarticular resection) to avoid cutting through tumor.]
Which type of melanoma is the most common, exhibiting an intermediate risk of malignancy, and originates from a nevus or sun-exposed area?
Superficial spreading melanoma
[UpToDate: Superficial spreading melanoma is the most common subtype, comprising approximately 75 percent of all malignant melanomas. One-fourth of superficial spreading melanomas are found in association with a preexisting nevus, such as a dysplastic or congenital nevus, while the majority appear to arise de novo. These melanomas derive their name from histologic evidence of lateral (radial) growth for a period of time before vertical (invasive) growth occurs. Although superficial spreading melanoma has a predilection for the back in men and lower extremities in women, it can occur in any anatomic location, and at any age. Clinically, the superficial spreading type of melanoma appears as a variably pigmented plaque with an irregular border, ranging from a few millimeters to several centimeters in diameter. In fully evolved lesions, multiple shades of red, tan, brown, blue, black, gray, and white can be appreciated. The red zones are thought to represent areas of vessel ectasia and inflammation, and the white and gray regions reflect amelanotic or regressed foci. An asymmetric flare at the lesion periphery represents the advancing border of proliferating, neoplastic melanocytes.]
Merkel cell carcinoma is what type of tumor?
Neuroendocrine tumor
[Very aggressive, malignant tumor with early regional and systemic spread. It is red to purple in color and appears as a papulonodule or indurated plaque.]
[UpToDate: Merkel cell carcinoma (MCC) of the skin is a rare, aggressive cutaneous malignancy. MCC typically presents in older patients with light skin tones as a rapidly growing, painless, firm, non-tender, shiny, flesh-colored or bluish-red, intracutaneous nodule. Ulceration and crusting are infrequent. MCCs range in size from less than 1 cm to over 2 cm and are most often located in sun-exposed areas.]
Which genetic syndrome for soft tissue tumors predisposes to the below tumors?
- CNS tumors
- Peripheral sheath tumors
- Pheochromocytomas
Neurofibromatosis
[UpToDate: There are three major clinically and genetically distinct forms of neurofibromatosis: neurofibromatosis types 1 and 2 (NF1 and NF2) and schwannomatosis. NF1, also known as von Recklinghausen disease, is the most common type. The hallmarks of NF1 are the multiple café-au-lait macules and neurofibromas. The condition is called segmental NF1 when clinical features are limited to one area of the body.]
Neuron-specific enolase (NSE), cytokeratin, and neurofilament protein are all found in which skin cancer?
Merkel cell carcinoma (neuroendocrine tumor)
What is the second most common soft tissue sarcoma?
Liposarcoma
[#1 is malignant fibrous histiosarcoma (also known as pleomorphic undifferentiated sarcoma).]
What is the main cell type in the epidermis and from where does it originate?
Keratinocytes originate from the basal layer
[Keratinocytes provide a mechanical barrier. The epidermis is primarily cellular.]
What staging work up is required for all melanomas > 1 mm in depth?
CT chest/abdomen/pelvis, LFTs, and LDH
[Physical exam of all possible draining lymph nodes should also be performed.]
