Ch11 Salivary - Malignant Flashcards by Austin Shackelford

What is the % range of MucoEp that show a reciprocal translocation? What are the choromosomes? What is the resultant fusion oncogene?

38-82% (big range lol)…..t(11,19)….CRTC1-MAML2

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Which grade(s) of MucoEp are more commonly found to have the CRTC1-MAML2 gene fusion?

low and intermediate grades of mucoep

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MucoEp age range

20-70 years

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What is the most common malignant salivary gland tumor in children?

mucoep

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What are the three lesional cells in mucoep?

mucus producing cells, epithelial cells, and intermediate cells (progenitors for mucus and epi)

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What are the 3 tools used to grade mucoep?

amount of cyst formation 2. degree of cytologic atypia 3.relative # of mucous, epidermoid, and intermediate cells

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____-grade MucoEp tumors show prominent cyst formation, minimal cellular atypia, and a relatively high proportion of mucous cells

Low-grade

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_____-grade MucoEp tumors consist of solid islands of squamous and intermediate cells, which can demonstrate considerable pleomorphism and mitotic activity. Mucus-producing cells may be infrequent, and the tumor sometimes can be difficult to distinguish from squamous cell carcinoma.

High-grade

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What is the cure rate for low-grade mucoep?

90-98%

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What is the survival rate for high-grade mucoep?

30-54%

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Do mucoeps with the CRTC1-MAML2 gene fusion have a better or worse prognosis?

BETTER

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Two theories for intraosseous MucoEp:

1.developmentally entrapped salivary tissue 2. odontogenic epithelium (dentigerous cysts have mucous producing cells)

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Intraosseous mucoep: age, sex, location

middle aged adults, female, mandible - molar/ramus

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Intraosseous mucoep: clinical finding

cortical swelling

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Intraosseous mucoep: radiographic findings

uni or multi-locular RL w/ well defined borders

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Intraosseous mucoep: Conservative tx recurrence? Aggressive tx recurrence?

conserv: 40%, aggressive: 13%

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Intraosseous mucoep: % of cases that met

12%

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Acinic cell carcinoma is a salivary gland malignancy with cells that show ______ acinar differentiation

serous acinar

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Many cases previously reported as acinic cell carcinoma, but which are poor in zymogen granules, would be reclassified today as __________

mammary analogue secretory carcinoma

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What % range of acinic cell carcinoma occurs in the parotid?

85-90%

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Acinic cell carcioma: age 20-70 years, mean age is ?

mid 40’s - early 50’s

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Are acinic cell carcinomas encapsulated?

The tumor often is well circumscribed and sometimes may even appear encapsulated; however, some tumors exhibit an infiltrative growth pattern

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What are the 4 patterns of acinic cell carcinoma?

solid 2. microcystic 3. papillary-cystic 4. follicular

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acinic cell carcinoma is associated with one of the better prognoses of any of the malignant salivary gland tumors. Approximately ___ - ____% of patients have recurrences locally, and metastases develop in __ - __% of patients. About __% of patients will die of their disease.

10-20% recurrence….8-11% mets…..10% die

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What is the chomosomal translocation and the resulting gene fusion associated with mammary analogue secretory carcinoma?

t(12;15)(p13;q25)....ETV6-NTRK3

What were MASC (mammary an sec ca) probably initially diagnosed as due to cytology before the discovery of the translocation in 2010?

Acinic Cell Carcinoma

What is the most common location for MASC?

parotid

MASC: age? sex?

mean age 47 years, MALES (even though its a mammary cancer?!)

What are 3 positive stains in MASC? What are 2 methods for finding the gene fusion?

S100, vimentin, mammaglobin....FISH break apart probe for ETV6 or detection for the ETV6-NTRK3 fusion gene via RT-PCR

mammary analogue secretory carcinoma appears to be a \_\_\_-grade malignancy with a _______ prognosis

low-grade...generally favorable

Carcinoma ex PA is a malignant transformation of the _______ component of a prev benign PA

epithelial

Carcinoma ex PA: age range

60-80 years old (mean age us 15 years older than PA - evidence that it developed from a benign PA)

Carcinosarcoma top 3 locations

parotid \> submandibular gland, minor salivary glands

Where are the 2 most common sites for a metastasizing mixed tumor? 3 other possible sites?

bones, lung....regional lymph nodes, skin, liver

What are the 2 most characteristic cytologic signs of carcinoma ex PA?

1. cellular pleomorphism 2. abnormal mitotic activity

What are the 5 architectural histologic possibilites of Carcinoma ex PA?

Most common: 1. poorly diff adenoca....2.myoepithelial ca 3. polymorphous adenoca 4.mucoep 5.adenoid cystic ca

What are the three histologic subcategories of carcinoma ex PA? What are the criteria for each?

1.invasive (\>1.5mm of invasion beyond tumor capsule) 2. minimally invasive (\<1.5mm invasion) 3. noninvasive (a small malignant focus w/in a benign PA)

LOL. what are the two alternate terms for noninvasive carcinoma ex pleomorphic adenoma?

1. carcinoma in situ ex mixed tumor 2. intracapsular carcinoma ex pleomorphic adenoma

Carcinosarcoma: which biphasic component typically predominates? What is the most common subtype of this dominating feature?

sarcomatous....chondrosarcoma (can also show osteosarcoma, fibrosarcoma, liposarcoma, rhabdomyosarcoma, or malignant fibrous histiocytoma)

Carcinosarcoma: the epithelial component usually consists of what 2 forms?

poorly diff adenocarcinoma or undifferentiated carcinoma

Metastasizing mixed tumor: microscopic features \_\_\_\_\_\_\_\_\_, within both the primary and the metastatic sites. WHAT type of changes are NOT observed?

benign pleomorphic adenoma.....Malignant histopathologic changes are NOT observed

Carcinoma ex PA: prognosis is guarded; the overall 5-year survival rate ranges from \_\_\_-\_\_\_%, prog is closely related to \_\_\_\_\_\_

25-65%....histo subtype (polymorphous adenoca = 90% survival)

Carcinosarcoma: prognosis is poor, with around \_\_\_% of patients either dying from their disease or developing recurrent local tumor or metastases

75%

Metastasizing mixed tumor: mortality rate of \_\_\_% has been reported

40%

What skin adnexal tumor does adenoid cystic carcinoma resemble histologically?

cylindroma

Adenoid cystic ca can occur in any salivary site, but 40-45% occur in \_\_\_\_\_\_\_....the remaining are evenly found in which two sites?

minor salivary glands (palate most common)...evenly between parotid and submandibular gland

What is the most common maligancy of the submandibular gland?

adenoid cystic carcinoma

Adenoid cystic carcinoma: age, sex

middle aged adults (rare under 20 years), FEMALE (1.4:1)

What is an important clinical finding for adenoid cystic carcinoma?

PAIN (consistent, low-grade, dull ache that gradually increases intensity), often preceeds clinical swelling

Adenoid cystic carcinoma: what are the 2 cell populations?

myoepithelial and ductal cells

Adenoid cystic carcinoma: 3 major histo patterns?

1. cribriform 2.tubular 3.solid (usually a combo is seen and final dx is based on predominant pattern)

Adenoid cystic carcinoma: cribriform pattern is the most classic and best- recognized appearance, characterized by islands of basaloid epithelial cells that contain multiple cylindrical, cystlike spaces resembling "\_\_\_\_\_\_\_"

Swiss cheese

Adenoid cystic carcinoma: cribriform pattern - The tumor cells are small and cuboidal, exhibiting deeply ________ nuclei and LITTLE cytoplasm.

basophilic

Adenoid cystic carcinoma: A highly characteristic histologic feature is its tendency to show \_\_\_\_\_\_\_\_\_

perineural invasion (NOT pathognomonic, other salivary malignancies do this too - esp polymorph adenoca

Accoriding to Neville: what two IHC stains are helpful to dx adenoid cystic ca? how many others are mentioned as helpful? (no names needed, just give a #)

CD43 and c-kit (CD117)...7 others (vimentin, collagen IV, laminin, integrins, Ki-67, smooth muscle actin, and various cytokeratins)

What two IHC stains are most helpful for differentiating adenoid cystic from polymorphous adenocarcinoma? why?

p63 and p40: concordant staining in adenoid cystic carcinoma (either BOTH positive or BOTH negative)....discordant staining in polymorphous adenoca (+p63 / -p40)....Since polymorphous adenocarcinoma is not of pure myoepithelial origin, the less specific stain (p63) should stain positive and the more specific stain (p40) should stain negative

Adenoid cystic carcinoma: Because metastasis to regional lymph nodes is uncommon (\_\_-\_\_% of cases), neck dissection usually is NOT indicated

6-10%

Adenoid cystic carcinoma: 5-year survival rate \_\_% to \_\_%, 10-year survival \_\_% to \_\_%, and by 20 years, only \_\_-\_\_% of patients are still alive

5-year survival rate 77% to 82% (THINK 3/4), 10-year survival 60% to 68% (THINK 2/3), by 20 years, only 35% to 52% (THINK 1/3-1/2) of patients are still alive

Adenoid cystic carcinoma: Tumors with a _____ histopathologic pattern are associated with a worse outlook than those with a _______ or _______ arrangement

solid = worse than cribriform or tubular

Adenoid cystic carcinoma: prognosis is poorest for tumors arising in what 2 locations?

maxillary sinus and submandibular gland

Adenoid cystic carcinoma: Most studies have shown that microscopic identification of _______ has LITTLE effect on the prognosis.

perineural invasion...interesting!

Tumor DNA ploidy analysis may help to predict the prognosis of adenoid cystic carcinoma; patients with ______ tumors have been shown to have a significantly better outcome than patients with _______ tumors

patients with diploid tumors have been shown to have a significantly better outcome than patients with aneuploid (either less than 23 pairs, or more than) tumors

Adenoid cystic carcinoma: Metastases occur in approximately \_\_% of patients, most frequently involving what 3 locations?

35%...lungs, bone, and brain

What are the 2 alternate names for polymorphous adenocarcinoma?

lobular carcinoma and terminal duct carcinoma

Polymorhous Adenocarcinoma: almost EXCLUSIVELY located where?

minor salivary glands (65% on palate \> upper lip \> buccal mucosa)

Polymorhous Adenocarcinoma: age, sex?

60-80 years...FEMALE (2/3 of cases)

Polymorhous Adenocarcinoma: Is it encapsulated?

No, at low power, the tumor sometimes appears well circumscribed. However, the peripheral cells are usually infiltrative, invading the adjacent tissue in a single-file fashion

Polymorhous Adenocarcinoma: when compared to Adenoid cystic carcinoma, what two IHC stains (Neville - NOT p63/p40 lol) are helpful in the distinction?

CD43 and c-kit (CD117) are weak staining in polymorphous compared to strong staining in adenoid cystic

Polymorhous Adenocarcinoma: what IHC can be helpful to distinguish from pleomorphic adenoma?

GFAP (glial fibrillary acidic protein) is strongly positive in pleomorphic adenoma and negative in polymorphous adenocarcinoma

Polymorhous Adenocarcinoma: Metastasis to regional lymph nodes is relatively uncommon, occurring in \_\_% to \_\_%

9% to 17%

Polymorhous Adenocarcinoma: The overall prognosis is relatively good. Recurrent disease has been reported in \_\_% to \_\_%. Microscopic identification of ________ does NOT appear to affect the prognosis

9% to 29%....perineural invasion

Salivary adenocarcinomas, not otherwise specified (NOS): Like most salivary tumors, they appear to be most common in the \_\_\_\_\_\_\_, followed by what 2 sites?

parotid gland \> minor glands \> submandibular gland

Salivary adenocarcinomas, not otherwise specified (NOS): The survival rate is better for tumors where? rather than where?

oral cavity.... rather than major salivary glands

Salivary adenocarcinomas, not otherwise specified (NOS): 10-year survival rate for parotid tumors ranges from \_\_% to \_\_%; compared to \_\_% for intraoral tumors

parotid tumors ranges from 26% to 55%...76% for intraoral tumors