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Neville and Damm > Ch11 Salivary - Malignant > Flashcards

Ch11 Salivary - Malignant Flashcards

1
Q

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

A

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

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2
Q

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

A

low and intermediate grades of mucoep

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3
Q

MucoEp age range

A

20-70 years

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4
Q

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

A

mucoep

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5
Q

What are the three lesional cells in mucoep?

A

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

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6
Q

What are the 3 tools used to grade mucoep?

A
  1. amount of cyst formation 2. degree of cytologic atypia 3.relative # of mucous, epidermoid, and intermediate cells
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7
Q

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

A

Low-grade

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8
Q

_____-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.

A

High-grade

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9
Q

What is the cure rate for low-grade mucoep?

A

90-98%

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10
Q

What is the survival rate for high-grade mucoep?

A

30-54%

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11
Q

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

A

BETTER

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12
Q

Two theories for intraosseous MucoEp:

A

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

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13
Q

Intraosseous mucoep: age, sex, location

A

middle aged adults, female, mandible - molar/ramus

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14
Q

Intraosseous mucoep: clinical finding

A

cortical swelling

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15
Q

Intraosseous mucoep: radiographic findings

A

uni or multi-locular RL w/ well defined borders

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16
Q

Intraosseous mucoep: Conservative tx recurrence? Aggressive tx recurrence?

A

conserv: 40%, aggressive: 13%

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17
Q

Intraosseous mucoep: % of cases that met

A

12%

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18
Q

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

A

serous acinar

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19
Q

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

A

mammary analogue secretory carcinoma

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20
Q

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

A

85-90%

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21
Q

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

A

mid 40’s - early 50’s

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22
Q

Are acinic cell carcinomas encapsulated?

A

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

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23
Q

What are the 4 patterns of acinic cell carcinoma?

A
  1. solid 2. microcystic 3. papillary-cystic 4. follicular
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24
Q

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.

A

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

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25
Q

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

A

t(12;15)(p13;q25)….ETV6-NTRK3

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26
Q

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

A

Acinic Cell Carcinoma

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27
Q

What is the most common location for MASC?

A

parotid

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28
Q

MASC: age? sex?

A

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

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29
Q

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

A

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

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30
Q

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

A

low-grade…generally favorable

31
Q

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

A

epithelial

32
Q

Carcinoma ex PA: age range

A

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

33
Q

Carcinosarcoma top 3 locations

A

parotid > submandibular gland, minor salivary glands

34
Q

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

A

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

35
Q

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

A
  1. cellular pleomorphism 2. abnormal mitotic activity
36
Q

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

A

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

37
Q

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

A

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)

38
Q

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

A
  1. carcinoma in situ ex mixed tumor 2. intracapsular carcinoma ex pleomorphic adenoma
39
Q

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

A

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

40
Q

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

A

poorly diff adenocarcinoma or undifferentiated carcinoma

41
Q

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

A

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

42
Q

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

A

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

43
Q

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

44
Q

Metastasizing mixed tumor: mortality rate of ___% has been reported

45
Q

What skin adnexal tumor does adenoid cystic carcinoma resemble histologically?

A

cylindroma

46
Q

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

A

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

47
Q

What is the most common maligancy of the submandibular gland?

A

adenoid cystic carcinoma

48
Q

Adenoid cystic carcinoma: age, sex

A

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

49
Q

What is an important clinical finding for adenoid cystic carcinoma?

A

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

50
Q

Adenoid cystic carcinoma: what are the 2 cell populations?

A

myoepithelial and ductal cells

51
Q

Adenoid cystic carcinoma: 3 major histo patterns?

A
  1. cribriform 2.tubular 3.solid (usually a combo is seen and final dx is based on predominant pattern)
52
Q

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 “_______”

A

Swiss cheese

53
Q

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

A

basophilic

54
Q

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

A

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

55
Q

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 #)

A

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

56
Q

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

A

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

57
Q

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

58
Q

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

A

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

59
Q

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

A

solid = worse than cribriform or tubular

60
Q

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

A

maxillary sinus and submandibular gland

61
Q

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

A

perineural invasion…interesting!

62
Q

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

A

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

63
Q

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

A

35%…lungs, bone, and brain

64
Q

What are the 2 alternate names for polymorphous adenocarcinoma?

A

lobular carcinoma and terminal duct carcinoma

65
Q

Polymorhous Adenocarcinoma: almost EXCLUSIVELY located where?

A

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

66
Q

Polymorhous Adenocarcinoma: age, sex?

A

60-80 years…FEMALE (2/3 of cases)

67
Q

Polymorhous Adenocarcinoma: Is it encapsulated?

A

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

68
Q

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

A

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

69
Q

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

A

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

70
Q

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

71
Q

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

A

9% to 29%….perineural invasion

72
Q

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

A

parotid gland > minor glands > submandibular gland

73
Q

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

A

oral cavity…. rather than major salivary glands

74
Q

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

A

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

Neville and Damm (72 decks)

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