Path Review Flashcards

Question

You are evaluating a 40-year-old African American female for evaluation of a non-expansile, mixed radiolucent-radiopaque lesion of the anterior mandible. She shows you a prior biopsy report of the lesion with a final diagnosis of “benign fibro-osseous lesion.” What is the most likely diagnosis for this lesion? a. Fibrous dysplasia b. Periapical cemento-osseous dysplasia c. Focal cemento-osseous dysplasia d. Florid cemento-osseous dysplasia e. Ossifying fibroma

Answer 1

b. Periapical cemento-osseous dysplasia The histologic features of all forms of benign fibro-osseous lesions are relatively similar, therefore clinical and radiographic correlation is required to arrive at a definitive diagnosis. The question stem describes features most characteristic of periapical cemento-osseous dysplasia.

Answer 2

B. Odontogenic keratocyst The image shows the architecture of a cyst, including a lumen, epithelial lining, and fibrous connective tissue wall, therefore choice D is incorrect. The exact type of cyst is determined by the features of the epithelial lining. The epithelial lining shown is ~6-8 cell layers thick, has a palisaded basal cell layer, and is covered by a corrugated surface of parakeratin. Therefore, the correct answer is B. A lateral periodontal cyst (A) will have a thinner lining (1-3 cells thick) with focal thickenings and no keratin. A glandular odontogenic cyst (C) will have a lining of variable thickness and other features including mucous cells, apocrine snouting, microcysts, and cilia. A calcifying odontogenic cyst (E) will have aberrant keratinocyte formation known as “ghost cells.”

Answer 3

d. Calcifying odontogenic cyst The calcifying odontogenic cyst is seen most frequently in the anterior region (incisor-canine area) of the jaws, and has an equal predilection between the maxilla and the mandible.

Answer 4

c. Autosomal recessive pattern of inheritance Gorlin Syndrome has an autosomal dominant pattern of inheritance. All other choices listed are features of Gorlin Syndrome.

Answer 5

a. Calcifying odontogenic cyst “Ghost cells” are eosinophilic epithelial cells with pink cytoplasm but lacking a nucleus. They are seen in the epithelial lining of calcifying odontogenic cysts.

Answer 6

b. Radicular cyst The most common odontogenic cyst overall is the radicular cyst, which is a type of inflammatory cyst. The most common developmental odontogenic cyst is the dentigerous cyst.

Answer 7

a. Lateral periodontal cyst The image shows the architecture of a cyst, including a lumen, epithelial lining, and fibrous connective tissue wall, therefore choice D is incorrect. The exact type of cyst is determined by the features of the epithelial lining. The lining shown is thin (1-3 cell layers thick) with focal thickenings. The diagnosis is therefore A, lateral periodontal cyst. An odontogenic keratocyst (B) will have a lining that is ~6-8 cell layers thick, has a palisaded basal cell layer, and is covered by a corrugated surface of parakeratin. A glandular odontogenic cyst (C) will have a lining of variable thickness and other features including mucous cells, apocrine snouting, microcysts, and cilia. A calcifying odontogenic cyst (E) will have aberrant keratinocyte formation known as “ghost cells.”

Answer 8

e. Glandular odontogenic cyst Although choice C may also cross the midline if it becomes large enough in size, the lesion with the greatest propensity of the choices listed is E. The glandular odontogenic cyst and central giant cell granuloma are the two radiolucent lesions most likely to cross the midline.

Answer 9

d. Lateral periodontal cyst The gingival cyst of the adult is the soft tissue analogue of the lateral periodontal cyst, in that it has the same/similar histologic features but lacks bony involvement. The gingival cyst of the newborn is a keratin filled cyst seen on the alveolar ridge of newborn children, which will rupture and resolve spontaneously. The soft tissue equivalent of the dentigerous cyst is the eruption cyst/hematoma.

Answer 10

c. Odontogenic keratocyst

Answer 11

a. Dentigerous cyst

Answer 12

a. Autosomal dominant

Answer 13

a. Odontogenic keratocyst The “primordial cyst” by definition is a cyst that forms in place of a tooth (i.e. although there is no tooth at the site of the cyst, the patient reports no history of prior dental extractions in the area). Most of these “primordial cysts” are odontogenic keratocysts on microscopic examination.

Answer 14

a. Buccal bifurcation cyst The buccal bifurcation cyst is a type of inflammatory odontogenic cyst that is most often seen in association with erupting mandibular first molars. Other examples of inflammatory odontogenic cysts include radicular and residual cysts.

Answer 15

c. Calcifying odontogenic cyst The Gorlin cyst is another name for the calcifying odontogenic cyst. The Gorlin cyst/COC is NOT associated with Gorlin Syndrome (Nevoid Basal Cell Carcinoma Syndrome).

Answer 16

a. Residual cyst Although still exceedingly rare, the epithelial lining of a residual cyst may undergo malignant change, from which a squamous cell carcinoma may develop.

Answer 17

c. Mandibular first molar

Answer 18

a. Calcifying odontogenic cyst

Answer 19

b. An odontogenic keratocyst may be seen in Gorlin Syndrome, while an orthokeratinized odontogenic cyst will not An odontogenic keratocyst has a higher rate of recurrence than an orthokeratinized odontogenic cyst. Both will present as radiolucent lesions. A biopsy is required to definitively distinguish between the two entities.

Answer 20

a. Palate Epstein Pearls are keratin filled inclusion cysts seen on the midline of the hard palate in newborns. They will rupture and resolve spontaneously. Similar appearing lesions are referred to as Bohns Nodules when they involve the lateral palate and gingival cyst of the newborn when they involve the alveolar ridge.

Answer 21

b. Lateral periodontal cyst

Answer 22

CEOT, mucoepidermoid CA, and renal cell CA

Answer 23

Craniopharyngioma, ameloblastic fibro-odontoma, calcifying odontogenic cyst (gorlin cyst), ghost cell odontogenic CA Ghost cells are enlarged eosinophilic epithelial cells with eosinophilic cytoplasm without a nucleus

Answer 24

Ameloblastoma, CEOT and odontogenic myxoma

Answer 25

Calcifying Cystic Odontogenic Tumor (Gorlin Cyst)

Answer 26

d. Granular cell tumor

Answer 27

b. Schwannoma Antoni A and B are the characteristic patterns seen in a schwannoma. Antoni A is the more cellular zone which contains a palisaded arrangement of Schwann cells and intervening acellular areas known as Verocay bodies. Antoni B is less cellular and less organized and does not contain Verocay bodies.

Answer 28

a. Rhabdomyosaroma

Answer 29

b. Branchial cleft cyst

Answer 30

b. Metastatic oropharyngeal squamous cell carcinoma Most cystic lateral neck masses diagnosed in adults represent cystic metastases from an oropharyngeal primary SCC. A diagnosis of a branchial cleft cyst in a patient older than 40 years should be viewed with caution.

Answer 31

e. Multiple Endocrine Neoplasia Syndrome 2 MEN2B results in tumors of neuroendocrine tissues including pheochromocytomas and medullary thyroid carcinoma. Patients with MEN2B also have a Marfanoid build and multiple oral mucosal neuromas, the latter of which represents an early clinical feature and may aid in diagnosis.

Answer 32

d. Bilateral acoustic schwannomas Features of NF1 include pigmented iris hamartomas (Lisch nodules), axillary freckling (Crowe Sign), café au lait pigmentation, multiple neurofibromas, and an AD pattern of inheritance. Bilateral acoustic schwannomas are a feature of NF2.

Answer 33

d. All of the above are true

Answer 34

b. Nasopalatine duct cyst The cyst of the incisive papilla is the peripheral or soft-tissue only varient

Answer 35

b. It is limited to the gingiva

Answer 36

b. Lymphangioma A cystic hygroma is another term for a macrocystic lymphangioma of the lateral neck.

Answer 37

d. Alveolar soft part sarcoma

Answer 38

c. Synovial Sarcoma

Answer 39

b. Dermoid cyst

Answer 40

e. Granular cell tumor The most common site of occurrence for a GCT throughout the entire body is the tongue.

Answer 41

a. Schwannoma

Answer 42

b. Dermoid cyst

Answer 43

a. Primary or c. Tertiary Primary: typically from an adenoma of the parathyroid Tertiary: usually refractory hyperparathyroidism secondary to chronic renal disease or a paraneoplastic syndrome (ie oral SCC) Both present with similar Ca and PO4 findings due to the excessive release of parathyroid-related peptide secretion

Answer 44

b. Secondary Usually occurring due to low Vit D levels secondary to chronic renal disease resulting in poor Ca reabsorption.

Answer 45

Central Giant Cell Granuloma Presents as either aggressive (giant cell tumor) or non-aggressive (giant cell lesion) variants. Aggressive Criteria: one major criteria + 3 minor criteria Major Criteria: -size >5cm -recurrence after initial tx Minor Criteria: -rapid growth -tooth loosening +/- displacement -radiographic evidence of cortical thinning and/or perforation -tooth resorption Aggressive tx: en-bloc resection with 1cm margin Non-aggressive tx: enucleation + pharmacologic therapy (intralesional steroids, calcitonin therapy, INF therapy)

Answer 46

Peripheral Giant Cell Lesion One of the three P soft tissue lesions: -Pyogenic granuloma -Peripheral giant cell lesion -Peripheral ossifying fibroma

Answer 47

-Pyogenic granuloma -Peripheral giant cell lesion -Peripheral ossifying fibroma

Answer 48

Hyperparathyroidism, CGCG, Cherubism, and Aneurismal Bone Cyst, Langerhans Cell Histiocytosis Lesions are poorly related to one another, but appear similar under light microscope.

Answer 49

Characteristic finding of cherubism, although frequently absent. 3 Types of Cherubism: 1. Bilateral mandibular rami 2. Entire mandible, with condylar sparing 3. Maxilla and mandible Mutation in the SH3BP2 chromosome. Often left untreated, as it can spontaniously regress after puberty Associated with Jaffe-Campanacci syndrome. Noonan-like

Answer 50

Aneurismal bone cyst

Answer 51

Langerhans Cell Histiocytosis Also stains positive with CD1a and S100 (image)

Answer 52

Hand-schuller Christian or the multifocal unisystem presentation of langerhans cell histiocytosis

Answer 53

OF is encapsulated and will have a radiolucent rim (especially when smaller than 2-3cm), FD is not capsulated and follows the contours of the facial bones. Histologically, they are indistinguishable.

Answer 54

Schwannoma (neurilemmoma) Vestibular schwannoma is associated with NF2 (neurofibromin 2 gene on chromosome 22). For comparison, neurofibromas are UNENCAPSULATED, blend with adjacent tissue, have spindle-shaped cells with wavy nuclei, and abundant mast cells

Answer 55

Neurofibroma Associated with neurofibromatosis. Type 1 NF (Von Recklinnghausen disease) is the most common, presents with lisch nodules, coast of california cafe au lait spots, optic pathway gliomas, and crowe sign

Answer 56

Osteoid osteoma or osteoblastoma Osteoblastoma tends to be larger and more aggressive with constant discomfort. Osteoid osteoma presents with nocturnal pain, is smaller and RESPONDS TO NSAIDS

Answer 57

Gardner syndrome

Answer 58

Fibrosarcoma Tx: resection with 1-2cm margins. Questionable benefit of chemo or radiation therapy. Stains positive for vimectin.

Answer 59

Glandular Odontogenic Tumor Located in the anterior mandible as a painless swelling. Excised with a wide margin. Can be difficult to differentiate from a low-grade mucoepidermoid CA

Answer 60

Gingival cyst of adult

Answer 61

PTCH1 Also associated with Nevoid Basal Cell Carcinoma Syndrome (Gorlin Goltz Syndrome) and BCC

Answer 62

-Multiple OKCs -Numerous basal cell carcinomas (typically sun exposed areas) -Palmar pitting -Bifid Ribs -Calcified falx cerebri -Hypertelorism -Ovarian fibromas -Medulloblastomas

Answer 63

Traumatic Bone Cyst Does not represent a true cyst as there is no epithelial lining. Clinically appears as an empty cavity +/- clear serosanginous fluid.

Answer 64

Pleomorphic adenoma

Answer 65

Mucoepidermoid CA and Adenoid cystic carcinoma

Answer 66

Adenoid cystic CA, mucoepidermoid CA, and polymorphous low-grade adenocarcinoma (PLGA). ACC and high-grade mucoepidermoid CA are also known to have perineural SPREAD

Answer 67

Pleomorphic Adenoma Found in the parotid>palate>submandibular gland. The most common benign neoplasm of the salivary glands. Treatment is partial superficial parotidectomy vs superficial parotidectomy based on the ability to obtain margins with a 5mm cuff of normal tissue. In the submandibular gland the entire gland is removed, and in the palate there is a wide local excision with 1cm of periosteal sacrificing.

Answer 68

Papillary Cystic Lymphomatosum (Warthin's Tumor) Most commonly found in the parotid tail. 10% bilateral, 10% associated with smoking. Concentrates technetium 99, which can be used to aid in diagnosis. Tx: partial superficial parotidectomy vs total superficial parotidectomy

Answer 69

Canalicular Adenoma Subtype of monomorphic adenoma 75% occur in the upper lip.

Answer 70

Oncocytoma Subtype of monomorphic adenoma

Answer 71

Basal Cell Adenoma Most common subtype of monomorphic adenoma 75% occur in the parotid gland. Within major glands they display encapsulation (no capsule in minor salivary glands).

Answer 72

-Positive clinical nodes: modified radical neck dissection (levels II-V) is indicated -High risk/grade pathology (high grade mucoepidermoid CA, SSC, adenocarcinoma, malignant mixed tumor or oncocytoma) -T3 or T4 lesion -Age >53yo -Perilymphatic invasion -Extraglandular spread

Answer 73

Mucoepidermoid Carcinoma Parotid>Palate>Submandibular gland

Answer 74

Mucicarmine

Answer 75

a. Epidermoid cells

Answer 76

c. Wide local excision d. Superficial parotidectomy

Answer 77

a. Possible neck dissection b. Possible post-operative radiation therapy c. Wide local excision d. Superficial parotidectomy

Answer 78

Mucoepidermoid Carcinoma Note that increased numbers of epidermoid cells are seen in high-grade MEC vs increased glandular and mucous cells in low-grade

Answer 79

a. Solid Cribriform is the most common,

Answer 80

N2c Bilateral neck dissection is indicated

Answer 81

"I 'TOLD' you we needed a neck dissection!" T: T2 or higher O: occult metastasis to a regional node 20%+ probability L: location is high risk (FOM, tongue, retromolar trigone) D: DOI 3-4mm in the tongue

Answer 82

-Removal of all ipsilateral cervical lymph nodes -Levels: I-V -Removal of SCM, spinal accessory nerve and internal jugular vein

Answer 83

Similar to radical neck dissection (levels I-V) with preservation of critical structures. Type I: preserve the spinal accessory nerve (SAN) Type II: preserve SAN and internal jugular vein (IJV) Type III: preserve SAN, IJV and SCM "NVM" acronym: nerve, vein, muscle. Each level spares the previous structure and adds another.

Answer 84

Selective neck dissection aims to preserve critical structures like the SAN, IJV and SCM and limits the lymph node groups that would otherwise be included in a radical neck dissection. For oral SCC, levels I-III (supraomohyoid) are often included. For oropharyngeal CA, hypopharyngeal and laryngeal CA, levels II-IV are typically included.

Answer 85

"Expect Some Nausea, Vomiting, and Pain" E: Extracapsular nodal spread or positive margins at the primary tumor S: Size (T3-T4 primary tumor) N: N2-N3 or level IV-V nodes V: Vascular embolism P: Perineural invasion

Answer 86

b. Basal Cell Carcinoma 80% of all skin cancers are basal cell carcinomas. Associated with PTCH gene mutations, and thus are seen with Gorlin's Syndrome. 9-14% of skin malignancies are SCC. 4% of skin malignancies are melanoma Actinic Keratosis is a pre-malignant lesion with a 2-10% likelihood of malignant transformation to SCC.

Answer 87

g. Nodular Nodular - 79%. Bleeds easily, face>trunk Superficial - 15% Erythematous scaly plaque, trunk>face Morpheaform - 6% Resembles a scar Micronodular - <1%

Answer 88

a. Superficial spreading Superficial spreading: most common. Appears as dark/pigmented macules. Nodular: second most common. Raised blue-black-red papule or nodule. Does not have a radial growth stage, does not follow the ABCDE rule. Acral lentiginous: occurs on palms and soles (hutchinson sign - pigmentation under the nail bed) Lentigo maligna: least common, but most common type on the face or sun-exposed areas. Common in older individuals.

Answer 89

Abutting the papillary/reticular dermis Clark Levels: I - Confined to epithelium II - Invading the papillary dermis III - Abutting the papillary/reticular dermis IV - Reticular dermis V - Sub-cutaneous

Answer 90

a. Dapsone May be used for oral MMP that cannot be adequately managed with local therapy (topical steroids - triamcinolone). For ocular involvement, systemic steroids are indicated.

Path Review Flashcards

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