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WHO > Ch 1 - SCC through Teratocarcinosarcoma > Flashcards

Ch 1 - SCC through Teratocarcinosarcoma Flashcards

1
Q

Age and gender for KSCC and NKSCC of the sinonasal tract?

A

KSCC:6-7th decades, men 2x women

NKSCC: 6-7 decades, men more frequent than women

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2
Q

What % of sinonasal papillomas undergo malignant transformation and which type do they transform into?

A

2-10% into keratinizing SCC most often

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3
Q

What is the most frequent site for sinonasal KSCC?

A

Maxillary sinus

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4
Q

Which has a better prognosis: KSCC in the nasal cavity or KSCC in the paranasal sinuses?

A

Nasal cavity (maybe easier to access?)

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5
Q

What is the distinct histo characteristic of sinonasal NON-keratinizing SCC?

A

“Ribbon-like” growth pattern (and absent to limited maturation)

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6
Q

What % of sinonasal NKSCC harbour transcriptionally active high-risk HPV?

A

30-50%

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7
Q

Most common locations (2) for sinonasal NKSCC?

A

Maxillary sinus or nasal cavity

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8
Q

How are NKSCCs graded?

A

Trick question -theyre not

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9
Q

What is a common feature of the basal/superficial cells of a NKSCC tumor nest/ribbon?

A

Peripheral palisading

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10
Q

DDx for NKSCC (5)

A

SNUC, NE Ca, AdCyCa (Solid Variant), SMARCB-1 deficient carcinomas, NUT carcinoma (if abrupt keratinization)

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11
Q

What should be considered if abrupt keratinization is seen in a NKSCC?

A

NUT carcinoma

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12
Q

NKSCC positive stains (5ish)

A

CKs (including high-molecular weight CK5/6), p63, p40, p16 (diffuse), HPV ISH or PCR

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13
Q

NKSCC pert neg stains (4ish)

A

Nuclear retention of SMARCB-1 (INI1), NE markers, S100, NUT1

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14
Q

How does prognosis of NKSCC compare to KSCC in the sinonasal tract?

A

NKSCC possibly improved prognosis, but not as clear as Oropharynx

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15
Q

What are 2 factors that can improve prognosis in sinonasal NKSCC?

A

high-risk HPV or EGFR over-expression (probably cause their susceptible to EGFR inhibitors (TKIs and monoclonals)

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16
Q

What is the gender ratio for the newly discovered NKSCC with AdCyCa-like features? What is the suspected etiology?

A

Female to male 7:2…high-risk HPV

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17
Q

What tumor can be mimicked when an NKSCC with AdCyCa-like features has true ductal cells surrounded by basaloid to clear myoepithelal cells?

A

epithelial-myoepithelial carcinoma

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18
Q

Positive stains for NKSCC with AdCyCa-like features (8)…Pertainant negatives (1)

A

Myoepi: S100, calponin, p63, actin Ductal: KIT, CKs. BOTh cell types: p16 and High-risk HPV ISH

NO MYB translocations (as seen in 50% of true AdCyCa)

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19
Q

Age and gender for sinonasal spindle cell (sarcomatoid) SCC…what are the 2 primary risk factors?

A

elderly men…smoking and radiation exposure…HPV negative in the few cases tested

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20
Q

Sinonasal lymphoepithelial carcinoma most closely resembles the histo of which other carcinoma?

A

Non-keratinizing nasopharyngeal carcinoma, undifferentiated subtype

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21
Q

What are the demographics for most cases of sinonasal LEC?

A

Men in 5-7th decades from Asia, where EBV is endemic

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22
Q

What is the etiology of sinonasal LEC?

A

> 90% of cases harbor EBV

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23
Q

What is the most common location for sinonasal LEC? What must be ruled out to determine that the LEC is truly sinonasal primary?

A

Nasal cavity… invasion of a nasopharyngeal carcinoma from a nearby structure must be excluded (clinically, radiographically, pathologically)

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24
Q

Positive stains for sinonasal LEC (5)…Pertinent negatives (2 general categories)

A

Positive: PanCK, CK5/6 (vs SNUC which is CK5/6neg), p63, p40, EBER

Neg: lymphoid and melanocytic markers

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25
Q

Differential for sinonasal LEC (3)

A

lymphoma, melanoma, SNUC

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26
Q

Differentiate sinonasal LEC from SNUC (4 features)

A

SNUC lacks the syncytial growth pattern (lack of cytopasmic borders, etc) of LEC, is consistently EBER negative, and lacks CK5/6 with limited to absent p63

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27
Q

LETS GET IT: DDx for SNUC (10)

A
  1. Lymphoma
  2. NKSCC
  3. Basaloid SCC
  4. NE Carcinoma
  5. Olfactory Neuroblastoma
  6. NUT carcinoma
  7. Alveolar Rhabdomyosarcoma
  8. Ewing Sarcoma
  9. Adenoid Cystic Carcinoma, solid type
  10. Melanoma
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28
Q

What’s the small round blue cell ddx again? (7)

A 
Melanoma
Rhabdomyosarcoma
SNUC
Lymphoma
Esthesioneuroblastoma (Olfactory Neuroblastoma)
Ewing sarcoma
Pitutiary adenoma
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29
Q

What are the age and ethnicity demographics for SNUC?

A

teenagers to elderly, avg pt age: 50-60…60-70% caucasian males

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30
Q

SNUC should be negative for which two viruses?

A

EBV and HPV

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31
Q

2 most common sites for SNUC

A

nasal cavity and ethmoid sinuses

32
Q

Name that sinonasal carcioma based on the histo:

  1. Small nests of neoplastic cells with numerous squamous pearls in a desmoplastic stroma
  2. Interconnecting squamous RIBBONS invading the stroma with a broad pushing border, peripheral palisading of tumor nuclei
  3. Solid and cribriform nodules of basaloid tumor cells, inconspicuous eosinophilic ducts
  4. loosely arranged, moderately pleomorphic spindle cells with brisk mitotic activity
  5. Sheets of malignant cells, vesicular chromatin, prom nucleoli, indistinct cytoplasmic borders imparting a SYNCYTIAL quality. Every cell EBER positive
  6. Lobules and ribbons of malignant epithelial cells, one may say undifferentiated with tumor necrosis, vesicular chromatin, and prominent nucleoli
  7. Epithelioid nests of large, undifferentiated cells with abundant eccentrically located eosinophilic cytoplasm imparting rhabdoid appearance
A
  1. KSCC
  2. NKSCC
  3. HPV-related carcinoma with AdCyCa-like features
  4. spindle cell SCC
  5. LEC
  6. SNUC
  7. SMARCB1-deficient sinonasal carcinoma
33
Q

What is unique about the spread of SNUC?

A

60% spread to adjacent structures, but only 10-15% met to LNs

34
Q

SNUC IHC: pos (6ish), neg (6)

A

Pos: PanCK (AE1/AE3), “simple CKs” (CK7,CK8,CK18), Neuron-specific enolase (NSE), +/- p63, focal/patchy Synapto/Chromo, p16 (regardless of HPV status)

Neg: CK5/6, p40 (which is more SQUAMOUS specific), carcinoembryonic antigen (?), S100, CD45, calretinin

35
Q

Differentiate SNUC from KSCC

A

KSCC histo squamous diff CK5/6 pos, p63 pos, p40 pos

36
Q

Differentiate SNUC from NE carcinoma

A

NE has speckled chromatin, rosette formation, palisading and positive for NE markers

37
Q

Differentiate SNUC from NUT carcinoma

A

NUT has squamous diff (focal), pos for p63, p40, NUT protein positive on IHC

38
Q

While NO specific genetic alteratons have been ID’d in SNUC, which gene is amplified in 1/3 of tumors? Which protein is frequently strongly expressed?

A

SOX2….KIT (CD117)

39
Q

What is the gene that is rearranged in NUT carcnioma?

A

NUTM1

40
Q

Median age and gender of NUT carcinoma?

A

21 years, slight female

41
Q

65 % of NUT carcinoma present in which 2 areas?

A

nasal cavity and paranasal sinuses (generally midline, hence the alternate name of NUT midline carcinoma)

42
Q

Name that tumor: poorly diff epitheliod cells with pale to clear glycogenated cytoplasm with abrupt keratinization possible

A

NUT carcinoma (dx is made on FISH of NUT rearrangement, not histology though)

43
Q

What is the NUT monoclonal antibody?

A

C52

44
Q

Stains for NUT carcinoma: 9 positive

A

Pos: NUT monoclonal C52, P63, p40, CK, CD34, Syn, Chromo, p16, TTF1

45
Q

Whats a good ddx for NUT carcinoma? (6)

A

SCC (poorly diff), Ewing, SNUC, Leukemia, Germ Cell Tumor, Olfactory Neuroblastoma

46
Q

How do you differentiate between NUT carcinoma and SMARCB1-deficient sinonasal carinoma?

A

NUT = focal keratinization

SMARCB1-def = basaloid cells with rhabdoid or plasmacytoid features AND loss of SMARCB1 (INI1) on IHC

47
Q

What is the fusion 70% of the time for NUT carcinoma?

A

NUTM1-BRD4 t(15;19)

48
Q

Which has a better prognosis: NUT-BRD4 or NUT-variant?

A

NUT-variant may have a longer survival than NUT-BRD

49
Q

Age and gender for sinonasal NE carcinomas?

A

middle aged to older men

50
Q

Most common location for sinonasal NE ca?

A

ETHMOID sinus

51
Q

Stains for sinonasal NE carcinoma: 9 positive (which one is unique for small cell NE ca?)

A

Positive: CKs (CAM5.2, AE1/AE3), EMA (perinuclear dot formation), NE markers: synaptophysin (most sensitive and specific),, chromogranin, NSE (neuron-specific enolase - less common in large cell NEca), CD56…S100 is diffusely positive in small cell NEca), p16

52
Q

What is a key stain to differentiate sinonasal NEca from SNUC? What separates these two if both stain positive for neuroendocrine markers?

A

p16 is positive in LCNEC and SmCC and negative in SNUC…if both positive for NE markers, morphology will separate them

53
Q

Negative stains for sinonasal NEca (3)

A

CK5/6, EBER, CK20

54
Q

Which gene expression is higher in sinonasal NE ca compared to Olfactory neuroblastoma and rhabdomyosarcoma?

A

ASCL1 (hASH1)

55
Q

DDx for sinonasal Neuroendocrine Carcinoma (3)

A

Olfactory neuroblastoma, SNUC, and NUT carcinoma

56
Q

Which has a better prognosis: LCNECs or SmCCs?

A

LCNECs

57
Q

Name that tumor: Small cells with nuclear molding, even chromatin distribution

A

Small cell NEca

58
Q

Name that tumor: large cells with high N:C ratio, small nucleoli, and salt-and-pepper chromatin distribution

A

large cell NEca

59
Q

Which stain shows a cytoplasmic dot-like (perinuclear) pattern in small cell neuroendocrine carcinoma?

A

pancytokeratin

60
Q

What are the demographics for intestional type sinonasal adenocarcinoma?

A

WOOD or LEATHER-WORKERS…MALES 3-4x more likely, 6th-7th decades

61
Q

Besides leather or wood-workers, what are two other occupational exposures that can increase the risk for ITAC?

A

Formaldehyde or dust

62
Q

Most common site for ITAC?

A

LATERAL nasal wall, middle turbinate

63
Q

Name that sinonasal tumor: papillary growth with numberous goblet and Paneth cells, possible cribriform pattern, possible signet ring cells

A

Sinonasal Intestinal Type Adenocarcinoma

64
Q

Stains for ITAC: positive: the nasty 9

A

Positive: Mucicarmine, PAS/distase resistant, PanCK, CK7+/-, Carcninoembryonic antigen +/-, CK20+, CDX2, MUC2, villin, +/- NE markers

65
Q

What is the majority of genetic mutations found in ITAC? Are they MSI?

A

KRAS…they are microsatellite-stable and do not lose expression of mismatch repair proteins

66
Q

What do you call a sinonasal adenocarcinoma that does NOT have salivary gland features or intestinal features?

A

Sinonasal non-intestinal-type adenocarcinoma (non-ITAC) lol

67
Q

Which one favors men? Low Grade non-ITACs or High Grade non-ITACs?

A

High grade non-ITAC favors men

68
Q

Where do most low grade non-ITACs show up? What about high grade?

A

LG: nasal cavity (middle turbinate)…HG: both sinuses and nasal cavity

69
Q

Name that tumor: tubules that grow back to back as they invade the stroma or papillae lined by a single layer of columar epithelium. Calcispherules are rarely seen

A

sinonasal low grade non-intestinal type adenocarcinoma

70
Q

Name that tumor: predominantly solid growth pattern with occasional glandular structures and/or individual mucocytes

A

sinonasal high grade non-intestinal type adenocarcinoma

71
Q

What substance causes the clear cells in sinonasal renal-cell like carcinoma?

A

glycogen

72
Q

Name that tumor: monomorphous cuboidal to columnar glycogen-rich clear cells in nests with possible microcysts that lack mucin production, overall impression is low-grade neoplasm

A

sinonasal renal-cell like carcinoma

73
Q

LG and HG non-ITAC stains: positive (5), (1 special set of positive markers for HG), negative (4)

A

Positive: PAS/Distase resistant, CK7, DOG1, SOX10,S100…HG non-ITAC + NE markers

Negative: CK20, p63, CDX2, MUC2

74
Q

Renal cell-like carcinoma stains: 2 positive, 2 negative

A

positive: CAIX, CD10

Negative: PAX8, RCC marker

75
Q

Age and gender for teratocarcinosarcoma…wait. location too

A

60 years STRONG male predilection..nasal cavity

76
Q

Name that tumor: admixture of epithelial, mesenchymal, and neuroepithelial components, nests of immature epithelium with clear so-called FETAL-appearing cells, , spindle cells (fibroblasts/myofibroblasts/etc), and round to oval cells in nests or neurofibrillary background +/- rosette formation

A

Teratocarcinosarcoma

77
Q

Teratocarcinosarcoma: Positive (3 groups of stains, give one example of each), Negative (4)

A

Positive: epithelial (CK), mesenchymal (vimentin), neuroepithelial (synapto/chromo, S100)

Negative: PLAP, alpha-fetoprotein, hCG, CD30

WHO (7 decks)

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