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WHO > Ch 1 - Sinonasal Papilloas through Sarcomas > Flashcards

Ch 1 - Sinonasal Papilloas through Sarcomas Flashcards

1
Q

Which sinonasal papilloma type is the most common?

A

Inverted

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2
Q

What are two featured cells in inverted sinonasal papillomas?

A

mucocytes and transmigrating neutrophils

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3
Q

If you get an inverted sinonasal papilloma on boards, look for malignancy…up to what % transform? Also what are the gender demographics?

A

up to 27% and 2.5-3x more in males

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4
Q

What percent of inverted sinonasal papillomas are HPV positive, which strains?

A

38.5% HPV positive, low risk 2.8x more freq than high

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5
Q

INTERESTING! p16 is accepted as a serrogate marker for HPV, but which sinonasal papilloma has NO correllation?

A

inverted

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6
Q

2 most common locations for inverted sinonasal papillomas

A

nasal cavity and MEDIAL WALL of the maxillary sinus

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7
Q

What is the MRI description of an inverted sinonasal papilloma?

A

septate striated appearance

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8
Q

What is the gross description buzzword for an inverted sinonasal papilloma?

A

gray, undulating surface resembling a MULBERRY

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9
Q

Which CKs are positive for the squamous/columnar cells in an inverted sinonasal papilloma? (3)

A

CK10, CK10/13, CK1/2/10/11

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10
Q

Which gene has been linked to activating mutations in inverted sinonasal papillomas and concurrent SCCs arising from ISNPs?

A

EGFR

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11
Q

What tumor am I? A papilloma composed of both exophytic fronds and endophytic invaginations lined by multiple layers of columnar cells with oncocytic features, intraepithelial microcysts containing mucin and neutrophils

A

Oncocytic sinonasal papilloma

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12
Q

Gender, age, location for oncocytic sinonasal papilloma

A

Even male to female, >50 years, LATERAL nasal wall

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13
Q

Give the HPV association with each of the three sinonasal papillomas

A

Inverted: 38.5%, most 6/11, some 16/18

Oncocytic: no association

Exophytic: 63.5% HPV, mostly 6/11HIGHEST association

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14
Q

Which sinonasal papilloma displays both exophytic and endophytic growth?

A

Oncocytic

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15
Q

What ultrastructural component of the cell causes the oncocytic appearance in an oncocytic sinonasal papillomas?

A

high cytochrome c oxidase content and numerous mitochondria

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16
Q

What histo feature helps distinguish an oncocytic sinonasal papilloma from rhinosporidosis?

A

Oncocytic papilloma: small cysts filled with mucin or neutrophils in the EPITHELIUM

Rhino: microcysts, but in the STROMA

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17
Q

What can oncocytic sinonasal papillomas be confused with histologically? What features help distinguish them?

A

low-grade papillary adenocarcinoma (wtf is this?)…intact basement membranes and absence of infiltrative growth pattern = benign

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18
Q

Up to what % of oncocytic SN papillomas can harbor a carcinoma (SCC, MEC, NE, SNUC have all been seen with these)

A

up to 17 %

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19
Q

Age and gender for EXOPHYTIC SN papillomas?

A

males (up to 10x) and 20-50 yrs

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20
Q

About 64% of Exophytic SN papillomas have been shown to harbor HPV…are they high risk or low risk?

A

low. 6 & 11…malignant transformation is rare for these

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21
Q

Most common location for exophytic SN papillomas

A

lower anterior nasal septum

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22
Q

What does REAH stand for? What other entity is it on a spectrum with?

A

Respiratory Epithelial Adenomatoid Hamartoma….SH (Seromucinous Hamartoma)

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23
Q

Age and gender for REAH and SH

A

Male 6th decade (56 years)

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24
Q

Location for REAH and SH

A

Posterior nasal septum

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25
Q

How do you rule out a met when evaluating REAH?

A

REAH = CK20 & CDX2 negative

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26
Q

What components DEFINE a pleomorphic adenoma?

A
  1. Epithelial
  2. Myoepithelial/Mesenchymal

are essential for the diagnosis of a PA

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27
Q

gender and location of a sinonasal PA?

A

female (slight), nasal septal mucosa

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28
Q

what is unique about the histology of a sinonasal PA when compared to a PA elsewhere?

A

sinonasal PAs have a more EPITHELIAL predominant population compared to myoepithelial/stromal

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29
Q

Which has a higher recurrence: sinonasal PA or parotid PA?

A

parotid PA higher recurrence

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30
Q

WHat % of sinonasal PAs have malignant transformation? How does this compare to a parotid PA?

A

Sinonasal: up to 2.4-10% vs Parotid: 6.2%..so pretty similar

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31
Q

What are the two most common sinonasal sarcomas?

A
  1. Rhabdomyosarcoma

2. Fibrosarcoma

32
Q

What is the most common location for a sinonasal fibrosarcoma?

A

maxillary sinus

33
Q

Name that sarcoma: herringbone or chevron pattern lacking profound pleomorphism

A

fibrosarcoma

34
Q

What is a good histo differentiator between fibrosarcoma and undifferentiated pleomorphic sarcoma?

A

Undif pleo sarc = significant pleomorphism and storiform areas

35
Q

Sweet. Since Fibrosarcoma is a diagnosis of exclusion….what 10 entities should you exclude before giving up?

Also, what is a good stain, molecular, or histo feature to differentiate them?

A
  1. Sarcomatoid carcinoma (CK positive)
  2. Synovial sarcoma SSX-SS18 / t(x;18)
  3. leiomyosarcoma (SMA, MSA, h-caldesmon pos)
  4. spindle cell rhabdomyosarcoma (myogenin, myod1 pos)
  5. spindle cell melanoma (s100, HMB45, sox10 pos)
  6. MPNST (s100 pos)
  7. Biphenotypic sinonasal sarcoma (s100,SMA, t(2;4) PAX3-MAML3 fusion)
  8. glomangiopericytoma (D240 pos)
  9. Desmoid fibromatosis (B-catenin, CyclinD1, Calret pos)
  10. fibroblastic osteosarcoma (bone formation, CDK2, MDM2 over exp)
36
Q

What two stains are fibrosarcomas usually positive for? (still not very helpful)

A

vimentin and actins

37
Q

Name that sarcoma: pleomorphism, numerous mitoses, areas of tumor necrosis, histiocyte-like cells, foamy cells, giant tumor cells with large poly-lobulated nuclei

A

undifferentiated pleomorphic sarcoma

38
Q

While many stains are negative, what can be focally positive in UPS?

A

SMA…histiocytic antigens are usually negative (eventhough the alternate name is malignant fibrous histicytoma lol)

39
Q

UPS is a dx of exclusion, what are the 8 entities you should exlude?

A
  1. Carcinomas
  2. Melanoma
  3. lymphoma
  4. rhabdomyosarc
  5. leiomyosarcoma
  6. MPSNT
  7. Dediff/Pleomorph liposarcoma
  8. High grade myxofibroma
40
Q

Name that sarcoma: spindle cells in interlacing fascicles, nuclei are oval or elongate and frequently blunt-ended. Variable atypia. Eosinophilic cytoplasm perinuclear vacuoles. Epitheliod cells rare. Giant cells possible. Psammomatous calcification

A

Leiomyosarcoma

41
Q

What are 4 positive stains for leiomyosarc? How many should be positive for the diagnosis?

A
  1. Desmin
  2. h-caldesmin
  3. SMA
  4. MSA

2 of them should be positive

42
Q

What are the 4 categories of rhabdomyosarcoma?

A
  1. Embryonal
  2. Alveolar
  3. Pleomorphic
  4. Spindle-cell
43
Q

Age and gender of sinonasal rhabdomyosarcoma? What is the most common variant among the most common age?

A

0-10 years old, no sex predilection

Embryonal variant most common in young patients

44
Q

What is the most common location for a rhabdomyosarcoma?

A

paranasal sinuses

45
Q

Which type of rhabdomyosarc is more common in children? Which one for adults?

A

Children: embryonal
Adults: Alveolar

46
Q

Stains for rhabdomyosarcoma (6)

A

MOST USEFUL:

  1. Desmin
  2. Myogenin

Also:
Alveolar: myogenin 100%
Embryonal: myogenin +/-

Less specific:

  1. MyoD1
  2. fast myosin
  3. myoglobin
  4. MSA
47
Q

What unique, non-muscle stains might an alveolar rhabdomyosarc also express (7)?

A
  1. CKs
  2. EMA
  3. CD56
  4. Chromo
  5. Synapto
  6. CD20
  7. CD99
48
Q

Which rhabdomyosarcs harbor the gene fusion? What is the fusion?

A

Alveolar: PAX3-FOX01 (less commonly PAX7-FOX01)

49
Q

Genetic profile of embryonal rhabdomyosarc?

A

allelic losses in various chromosome 11 loci

50
Q

Rearrangement for pediatric spindle cell rhabdomyosarc?

A

NCOA2

51
Q

Name # syndromes that are associated with rhabdomyosarcoma?

A
  1. Li-Fraumeni
  2. Costello
  3. NF1
  4. Beckwith-Wiedemann
52
Q

Which has a poorer prognosis: alveolar or embryonal rhabdomyosarc?

A

Alveolar (later age, usually present with regional and met spread)

53
Q

Compare the mean patient age and gender for a sinonasal angiosarcoma compared to a skin/soft tissue angiosarcoma

A

Sinonasal: 47 years, male

Skin: Older, male

54
Q

Exposure to radiation, vinyl chloride, coal dust are risk factors for which sinonasal sarcoma?

A

angiosarcoma (used for PVC)

55
Q

Two most common sites for sinonasal angiosarc?

A

nasal cavity and maxillary sinus

56
Q

What is a histo buzz term for what the freely anastomosing vascular channels create in an angiosarc? WHAT IS THE PATHOGNOMONIC HISTOLOGY SIGN FOR ANGIOSARC?

A

“cleft-like” spaces

PATHOGNOMONIC: Intracytoplasmic lumina (often containing erythrocytes)

57
Q

9 stains for angiosarc

A
  1. Vimentin (lol)
  2. CD34
  3. CD31
  4. Claundin 5
  5. ERG
  6. FLI1
  7. D2-40
  8. factor VIII
  9. focal keratin (epithelioid variant)
58
Q

MPNST: what % are associated with NF1? How does NF1 affect the mean age of a patient presenting with an MPNST?

How does NF1 status affect prognosis?

A

25-30% of MPNST are with NF1 (not as many as i expected)

NF1 pts with MPNST present in their 20-30’s compared to non-NF1 in their 40’s

NF1 = WORSE prognosis

59
Q

What are the two most common nerves involved by a MPNST?

A

vestibular (VIII) and vagus (X)

60
Q

MPNSTs usually arise in either a pre-existing NF or schwannoma…which one is more common?

A

NF

61
Q

Name that sarcoma: marbled effect with alternating cellular and myxiod areas, perivascular cuffs, poorly defined nuclear palisading

A

MPNST

62
Q

Remind me of the components of a Malignant Triton Tumor

A

MPNST + Rhabdomyosarcoma

63
Q

Immunoprofile: MPNST (3), Epithelioid MPNST (2)

A

MPNST: S100 (nuc and cyto), SOX10 (nuc), Nestin

Epithelioid MPNST: strong S100, loss of SMARCB1 (INI1)

64
Q

Remind me: chromosome for NF1 gene

A

17q11

65
Q

Gene fusion and translocation for biphenotypic sinonasal sarcoma….oh what are the biphenotypes again?

A

Gene fusion: PAX3-MAML3
t(2;4)

Biphenotypes: sarcoma (spindle cell proliferation) + epithelial proliferation (entrapped glands and surface)

66
Q

Age and gender for biphenotypic sinonasal sarcoma

A

FEMALE 2:1 mean 52 years

67
Q

2 most common sites for BSNS

A

(Biphenotypic sinonasal sarcoma) SUPERIOR nasal cavity and ethmoid sinus

68
Q

Part of the histomorphology of a BSNS has a herringbone pattern spindle-cell population resembling which other sarcoma?

A

synovial sarcoma

69
Q

Since the histology of a BSNS is spindle and epithelial, which other neoplasm can the epithelial component resemble?

A

a sinonasal papilloma

70
Q

What are the 3 primary stains that are at least focally positive for BSNS?

A
  1. S100
  2. SMA
  3. MSA
71
Q

While PAX3-MAML3 is the most common gene fusion in BSNS, what is the other possibility? What other entity is it shared with?

A

PAX3-FOX01 = alveolar rhabdomyosarcoma

72
Q

Name that sarcoma: variable degree of epithelial differentiation including gland formation

A

synovial sarcoma

73
Q

Most common age range for synovial sarcoma and most common risk factor?

A

patients in their 20s-30s…prior radiation therapy

74
Q

What are 3 common histo subtypes for monophasic synovial sarcoma? What are the two most common histology features of a biphasic synovial sarcoma?

A

mono: spindle cell, calcifying/ossifying, myxoid)

Bi: spindle cells with: glandular or solid epithelial cells

75
Q

Stains for synovial sarcoma (6)

A
  1. TLE1 positive in 95%
  2. CD99
  3. BCL2
  4. CD56
  5. CK7
  6. EMA
76
Q

How might you differentiate from a BSNS and a Biphasic Synovial Sarcoma?

A

TLE1 posittive in biphasic synovial sarcoma

WHO (7 decks)

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