Path Review Flashcards
What are the six subtypes of ameloblastoma?
“PDF BAG”
Plexiform
Desmoplastic
Follicular
Basal Cell
Acanthomatous
Granular
Histology: Tumor islands with central cystic degeneration (stellate reticulum-like appearance) and “reverse polarization” of cells at the periphery of the tumor islands.
Ameloblastoma
Reverse polarity of nuclei is pathognmonic
Palisaded ameloblastlike cells and inner zone of triangular-shaped cells resembling stellate reticulum in bell stage
Follicular Ameloblastoma
Epithelium that proliferates in a fishnet pattern
Plexiform ameloblastoma
Radiolucency with multiloculated, soap-bubble appearance
Ameloblastoma
Solid or multicystic ameloblastoma. Tx?
Resection with 1cm margin and one anatomical barrier
Unicystic ameloblastoma. Tx?
Unicystic: enucleation and curettage +/- peripheral ostectomy or liquid nitrogen cryotherapy.
Mural subtype: consider resection with 1cm margin
Peripheral ameloblastoma. Tx?
Excision. Low to no chance for recurrence.
What is the 2/3rd rule of adenomatoid odontogenic tumor (AOT)?
2/3 canine teeth
2/3 female
2/3 maxilla
2nd and 3rd decade of life
Pear-shaped radiolucency with speckled opaque foci, potentially associated with an impacted tooth (canine)?
Adenomatoid odontogenic tumor (AOT)
Columnar epithelium in a duct-like pattern
Adenomatoid odontogenic tumor (AOT)
Histology: Lisegang rings, psammomalike calcifications, amyloid
Calcifying epithelial odontogenic tumor (CEOT) or Pindborg tumor.
If there are clear cells present, it is typically more aggressive with cortical perforation
Well-defined mixed radiolucent/radioopaque unilocular or multolocular lesion with a snowflake pattern (“driven snow” )
Calcifying epithelial odontogenic tumor (CEOT) or Pindborg tumor.
Calcifying epithelial odontogenic tumor (CEOT) or Pindborg tumor. Tx?
Resection with 1cm margins - generally has low recurrence. 14-20% recurrence with enucleation.
Triangular or pear-shaped radiolucency associated with an erupted tooth
Squamous odontogenic tumor (SOT). Presents similarly to AOT, except the tooth is erupted. Most common in the anterior maxilla and/or posterior mandible.
Variable-sized nests of cytologically bland squamous epithelium in a moderately cellular fibrous stroma
Squamous Odontogenic Tumor (SOT)
Predominance of stellate and spindle cells surrounded by mucoid material.
Odontogenic myxoma
Ill-defined radiolucent lesion with soap-bubble or honeycomb pattern, multilocular
Ameloblastoma
A mutation in which of the following genes is responsible for fibrous dysplasia?
a. GNAQ
b. GNAS
c. PTCH1
d. SH3BP2
e. RUNX2
b. GNAS
Mutations in GNAQ (A) are seen in conditions such as Sturge Weber Angiomatosis and blue nevi. PTCH1 (C) is mutated in sporadic cases of odontogenic keratocysts and more frequently in OKCs in the setting of Gorlin Syndrome. SH3BP2 (D) mutations are seen in Cherubism, RUNX2 (E) mutations are seen in Cleidocranial Dysplasia. RUNX2 is also referred to as CBFA1.
Which of the following statements regarding fibrous dysplasia is FALSE?
a. It is a tumor-like condition often diagnosed in childhood
b. Patients may present with expansion of one or more bones
c. It does not have any extra-skeletal (osseous) manifestations
d. Surgical recontouring should be avoided until skeletal maturity is achieved
e. All of the above are true
c. It does not have any extra-skeletal (osseous) manifestations
Some forms of polyostotic fibrous dysplasia have extra-skeletal manifestations. These include Jaffe Lichtenstein Syndrome (Café au lait pigmentation), McCune Albright Syndrome (Café au lait pigmentation + endocrine disturbances), and Mazabraud Syndrome (intramuscular myxomas).
An ossifying fibroma is most likely to be diagnosed in which of the following locations?
a. Anterior maxilla
b. Mandibular premolar-molar region
c. Anterior mandible
d. Maxillary premolar-molar region
e. Temporomandibular joint space
d. Maxillary premolar-molar region
- Patients with radiographic findings resembling florid cemento-osseous dysplasia but with generalized enlargement of the affected jawbones should be evaluated for which of the following conditions?
a. Cherubism
b. Multiple Myeloma
c. Cleidocranial dysplasia
d. Osteosarcoma
e. Paget’s Disease of Bone
e. Paget’s Disease of Bone
Late stage florid cemento-osseous dysplasia may present radiographically as hyperdense bony lesions resembling the “cotton wool” changes seen in Paget’s Disease of Bone.
A “ground-glass” pattern of bone may be seen in which of the following conditions?
a. Fibrous dysplasia
b. Periapical cemento-osseous dysplasia
c. Focal cemento-osseous dysplasia
d. Florid cemento-osseous dysplasia
e. Ossifying fibroma
a. Fibrous dysplasia
An 8-year-old female presenting with generalized maxillary enlargement, precocious puberty, and pigmented skin lesions should be evaluated for which of the following conditions?
a. Mazabraud Syndrome
b. Jaffe Lichteinstein Syndrome
c. McCune Albright Syndrome
d. Hyperparathyroidism Jaw Tumor Syndrome
e. Paget’s Disease of Bone
c. McCune Albright Syndrome
The question stem is describing the features of McCune Albright Syndrome, a form of polyostotic fibrous dysplasia which presents with café au lait skin pigmentations and endocrine disturbances. These endocrine disturbances most frequently manifest as precocious puberty.
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
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.
You perform an incisional biopsy on a unilocular radiolucent lesion of the left maxilla in the lateral incisor-canine area which is causing divergence of these tooth roots. A representative image of the biopsy specimen is shown below. What is the best diagnosis?
A. Lateral periodontal cyst
B. Odontogenic keratocyst
C. Glandular odontogenic cyst
D. Ameloblastoma
E. Calcifying odontogenic cyst
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.”
All of the following have a site predilection for the posterior mandible except one. Which is the EXCEPTION?
a. Odontogenic keratocyst
b. Ameloblastoma
c. Odontogenic myxoma
d. Calcifying odontogenic cyst
e. Ameloblastic fibroma
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.
Which of the following is NOT a feature of Gorlin Syndrome (Nevoid Basal Cell Carcinoma Syndrome)?
a. Multiple odontogenic keratocysts
b. Bifid or splayed ribs
c. Autosomal recessive pattern of inheritance
d. Palmar and plantar pits
e. Calcification of the falx cerebri
c. Autosomal recessive pattern of inheritance
Gorlin Syndrome has an autosomal dominant pattern of inheritance. All other choices listed are features of Gorlin Syndrome.
Cells displaying aberrant keratinization are a feature of which of the following?
a. Calcifying odontogenic cyst
b. Glandular odontogenic cyst
c. Adenomatoid odontogenic tumor
d. Odontogenic keratocyst
e. Calcifying epithelial odontogenic tumor
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.
The most common odontogenic cyst is the ____
a. Dentigerous cyst
b. Radicular cyst
c. Odontogenic keratocyst
d. Lateral periodontal cyst
e. Glandular odontogenic cyst
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.
You perform an excisional biopsy on a radiolucent lesion of the mandibular premolar region. A representative section from the specimen is shown below. What is the best diagnosis?
a. Lateral periodontal cyst
b. Odontogenic keratocyst
c. Glandular odontogenic cyst
d. Ameloblastoma
e. Calcifying odontogenic cyst
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.”
Which of the following lesions has the highest propensity to cross the midline?
a. Dentigerous cyst
b. Radicular cyst
c. Odontogenic keratocyst
d. Lateral periodontal cyst
e. Glandular odontogenic cyst
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.
The gingival cyst of the adult is the soft tissue equivalent of the:
a. Gingival cyst of the newborn
b. Odontogenic keratocyst
c. Dentigerous cyst
d. Lateral periodontal cyst
e. Calcifying odontogenic cyst
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.
Palisading of the basal cell layer and a corrugated, parakeratin lining are characteristic of which of the following?
a. Dentigerous cyst
b. Radicular cyst
c. Odontogenic keratocyst
d. Lateral periodontal cyst
e. Glandular odontogenic cyst
c. Odontogenic keratocyst
Which of the following is the most common developmental odontogenic cyst?
a. Dentigerous cyst
b. Radicular cyst
c. Odontogenic keratocyst
d. Lateral periodontal cyst
e. Glandular odontogenic cyst
a. Dentigerous cyst
The inheritance pattern of Gorlin Syndrome (Nevoid Basal Cell Carcinoma Syndrome) is:
a. Autosomal dominant
b. Autosomal recessive
c. X-lined dominant
d. X-lined recessive
e. None of the above
a. Autosomal dominant
Most examples of “primordial cysts” resemble what on histologic examination?
a. Odontogenic keratocyst
b. Dentigerous cyst
c. Glandular odontogenic cyst
d. Lateral periodontal cyst
e. Calcifying odontogenic cyst
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.
All of the following are considered developmental odontogenic cysts except one. Which is the EXCEPTION?
a. Buccal bifurcation cyst
b. Odontogenic keratocyst
c. Dentigerous cyst
d. Lateral periodontal cyst
e. Glandular odontogenic cyst
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.
The Gorlin Cyst is an alternative/former name for which of the following?
a. Lateral periodontal cyst
b. Odontogenic keratocyst
c. Calcifying odontogenic cyst
d. Dentigerous cyst
e. Glandular odontogenic cyst
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).
Which of the following odontogenic cysts is most likely to undergo malignant transformation?
a. Residual cyst
b. Dentigerous cyst
c. Odontogenic keratocyst
d. Buccal bifurcation cyst
e. Glandular odontogenic cyst
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.
The buccal bifurcation cyst is most commonly seen in association with:
a. Maxillary first molar
b. Maxillary second molar
c. Mandibular first molar
d. Mandibular second molar
e. Mandibular third molar
c. Mandibular first molar
Which of the following lesions contains ghost cells?
a. Calcifying odontogenic cyst
b. Glandular odontogenic cyst
c. Odontogenic keratocyst
d. Dentigerous cyst
e. Radicular cyst
a. Calcifying odontogenic cyst
Which of the following is a true statement regarding the differences between an odontogenic keratocyst and an orthokeratinized odontogenic cyst?
a. An odontogenic keratocyst has a lower recurrence rate than an orthokeratinized odontogenic cyst
b. An odontogenic keratocyst may be seen in Gorlin Syndrome, while an orthokeratinized odontogenic cyst will not
c. An odontogenic keratocyst is radiolucent, while an orthokeratinized odontogenic cyst is mixed radiolucent-radiopaque
d. An odontogenic keratocyst can be distinguished from an orthokeratinized odontogenic cyst based on radiographic presentation alone
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.
Epstein Pearls are usually seen on the:
a. Palate
b. Gingiva
c. Tongue
d. Buccal mucosa
e. Floor of mouth
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.
A botryoid odontogenic cyst is a name for a multilocular version of the:
a. Odontogenic keratocyst
b. Lateral periodontal cyst
c. Orthokeratinized odontogenic cyst
d. Dentigerous cyst
e. Calcifying odontogenic cyst
b. Lateral periodontal cyst
Name three clear cell tumors
CEOT, mucoepidermoid CA, and renal cell CA
Name four ghost cell tumors
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
What are the three benign pathologies that are resected with a margin of at least 1cm?
Ameloblastoma, CEOT and odontogenic myxoma
Histology: “Suprabasalar epithelium shows stellate reticulum with ghost cell formation”
Calcifying Cystic Odontogenic Tumor (Gorlin Cyst)
You perform an excisional biopsy of the tongue lesion shown below. Based on the clinical image and the associated photomicrograph, what is the best diagnosis?
a. Schwannoma
b. Lipoma
c. Squamous cell carcinoma
d. Granular cell tumor
e. Rhabdomyoma
d. Granular cell tumor
You perform an incisional biopsy of a submucosal palatal swelling. The pathology report describes Antoni A and Antoni B microscopic patterns. What is the best diagnosis?
a. Neurofibroma
b. Schwannoma
c. Granular cell tumor
d. Leiomyoma
e. Rhabdomyosarcoma
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.
The most common soft tissue malignancy involving the head and neck region in children is the:
a. Rhabdomyosaroma
b. Leiomyosarcoma
c. Malignant peripheral nerve sheath tumor
d. Liposarcoma
e. Fibrosarcoma
a. Rhabdomyosaroma
- You perform a biopsy of a palatal lesion in an HIV+ individual. A representative microscopic image is shown below. Which of the following is true regarding this lesion?
a. It is best diagnosed as Kaposi Sarcoma
b. It is caused by HHV-8
c. It may also be seen in patients who are status-post organ transplant
d. All of the above are true
d. All of the above are true
Histology: “dilated capillaries, multi-nucleated giant cells and hemosiderin pigmentation”
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)
What are five giant cell lesions?
Hyperparathyroidism, CGCG, Cherubism, and Aneurismal Bone Cyst, Langerhans Cell Histiocytosis
Lesions are poorly related to one another, but appear similar under light microscope.
Histology: “eosinophilic, perivascular cuffing of collagen”
Characteristic finding of cherubism, although frequently absent.
3 Types of Cherubism:
- Bilateral mandibular rami
- Entire mandible, with condylar sparing
- 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
Birbeck granules under electron microscopy is diagnostic for which giant cell lesion?
Langerhans Cell Histiocytosis
Also stains positive with CD1a and S100 (image)
Histology: pseudo-encapsulated, whorly spindle cells, antoni A and B forms, and verocay bodies
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
Histology: unencapsulated lesion that blends with adjacent tissue, has spindle-shaped cells with wavy nuclei, and abundant mast cells
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
Histology: “osteoid and primitive woven bone amidst fibrovascular connective tissue stroma”
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
Histology: “spindle cell tumor showing herring bone pattern and interfascicular pattern”
Fibrosarcoma
Tx: resection with 1-2cm margins. Questionable benefit of chemo or radiation therapy. Stains positive for vimectin.
Histology: multicystic lesion partially lined by respiratory epithelial cells with mucous pooling
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
Histology: cystic pathology lined by parakeratinized stratified squamous epithelium (6-8 cells thick). Areas of separation from the basal membrane with inconspicuous rete ridges
OKC
Radiograph: well-defined radiolucency of the posterior mandible with scalloping around the tooth roots
Traumatic Bone Cyst
Does not represent a true cyst as there is no epithelial lining. Clinically appears as an empty cavity +/- clear serosanginous fluid.
Histology: a combination of ductal cells, chondromyxoid matrix and myoepithelial cells
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.
Histology: papillary cystic growth of oncocytic ductal cells in a lymphoid stroma
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
Histology: interconnecting strands of ductal cells arranged in a double row`
Canalicular Adenoma
Subtype of monomorphic adenoma
75% occur in the upper lip.
Histology: papillary cystic growth of oncocytic ductal cells with round eosinophilic polygonal cells with swollen cytoplasm and centralized nucleus
Oncocytoma
Subtype of monomorphic adenoma
Histology: mucous, epidermoid and glandular cells arranged in a solid/cystic pattern
Mucoepidermoid Carcinoma
Note that increased numbers of epidermoid cells are seen in high-grade MEC vs increased glandular and mucous cells in low-grade
What is a Clark level III for melanoma?
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
