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Dermatology Board Prep > DERM (18) Skin Growths > Cutaneous Neural Tumors > Flashcards

Cutaneous Neural Tumors Flashcards

1
Q

What are the two major groups of neural tumors?

A

Peripheral nerve sheath tumors and neural heterotopias

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2
Q

What 4 types of neural tissue are neural heterotopias associated with?

A

meningeal (meningoceal, reudimetary menigocele, meningioma), neuroglial (nasal glioma), neuroblastic/ganglionic (neuroblastoma, primary primitive neuroectodermal tumor, ganglioneuroma), miscellaneous (pigmented neuroectodermal tumor of infancy)

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3
Q

What is a/w multiple mucosal neuromas?

A

MEN2b –> neuromas occur on all mucosal surfaces (lips, tongue, conjunctiva, nasal and laryngeal). You get pheochromocytoma, aggressive medullary thyroid cancer, marfanoid appearance, blubbery lips, CALM, circumoral lentigines.

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4
Q

What mutation is seen in multiple mucosal neuromas?

A

RET (MEN2b)

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5
Q

Ddx for cutaneous tumors that hurt?

A

BANGEL or blend an egg blue rubber bleb nevus syndrome, angiolipoma/angioleiomyoma, neurilemmoma/schwannoma, glomus, eccrine spiradenoma, leiomyoma

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6
Q

Which neurofibroma is pathognomonic for neurofibroma?

A

Plexiform neurofibroma

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7
Q

What stain is + when a neural tumor has a capsule?

A

EMA

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8
Q

What stain is positive if a neural tumor as axons?

A

NSE/Axon

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9
Q

What inflammatory cell can be seen in neurofibromas that can help you distinguish them?

A

Mast cells

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10
Q

What are the 2 types of neurothekeoma?

A

Cellular and myxoid

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11
Q

What is the difference in staining between myxoid and cellular neurothekeoma?

A

Myxoid (nerve sheath myxoma): s100 + S100A6 +; cellular neurothekeoma s100-, S100A6 +

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12
Q

What are some notable cutaneous lesions that are S100A6 + ?

A

Cellular neurothekeoma, histiocytic tumors, spitz, AFX

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13
Q

What stains help distinguish cellular neurothekeoma?

A

s100 -, desmin -, s100A6 +

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14
Q

what causes the granules in the granular cell tumor?

A

lysomal dysfunction

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15
Q

What is the staining for a perineurioma?

A

s100 -, NSE/AXON -, EMA + (becuase capsule)

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16
Q

What are the two types of perineurona?

A

Soft tissue and sclerosing

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17
Q

What entity should you suspect in rapidly growing nodule in plexiform nodule

A

Malignant peripheral nerve sheath tumor

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18
Q

What margins should be achieved on Merkel cell carcinoma?

A

2-3cm

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19
Q

What is the IHC staining for Merkel cell carcinoma?

A

TTF -; CK20+; EMA and enolase +, CK7 + (maybe), CK5/6 + (low molecular weight keratins)

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20
Q

What is the DDx for small cell blue tumors?

A

LEMONS: lymphoma, Ewing’s sarcoma, Merkel, oat cell, neuroblastoma, small cell endocrine carcinoma

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21
Q

What is the presentation of a nasal glioma?

A

Occurs on the midline of the face in babies, or the medial canthus. Can present w/ nasal cartilage or have eyes spaced far apart. (along cranial suture lines)

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22
Q

What are the 3 categories of peripheral nerve sheath tumors?

A

Hamartomas (neuromas), true nerve sheath neoplasms (schwannomas, neurofibromas, nerve sheath myxoma, cellular neurothekeoma, perineuroma, malignant peripheral nerve sheath tumor); miscellaneous (Merkel cell and granular cell tumor).

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23
Q

Where are the perineurium cells derived from and what do they stain for?

A

They are modified fibroblasts of mesodermal origin. Stain for epithelial membrane antigen, not with S100

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24
Q

Clinical presentation of traumatic/amputation neuroma?

A

Uncommon tumor, usually solitary, skin-colored firm papule or nodule, painful. Is on sites of wounds, surgical scars, and amputations

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25
Q

What is the histology of a traumatic/amputation neuroma?

A

Composed of chaotic, poorly organized tangles of nerve fascicles embedded within a fibrous scar

There are clefts between fascicles, located in any level of the dermis or subcutis

Stains: s100+, MBP+, Bodian stain+, NSE/Axon +, EMA +/-

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26
Q

What is the histology of a rudimentary digit/polydactyly?

A

Numerous nerve fiber bundles in connective tissue in the upper dermis; oval corpuscles present (looks like Meissner corpuscles)

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27
Q

Key differences between palisaded encapsulated neuroma and and traumatic neuroma?

A

Perineural tissue capsule surrounds the tumor in traumatic neuroma and there is clefting around the bundles in PEN

Also, there is no fibrous tissue sheath around fascles in PEN which is unlike truamatic neuroma.

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28
Q

What is the mutation in Men2b?

A

RET protooncogene on chromosome 10q11.2

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29
Q

Multiple mucocutaneous neuromas with a predilection for the face and distal extremities occur in what syndromes?

A

PTEN harmartoma tumor syndromes: Cowden, Bannayan-Riley-Ruvalcaba

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30
Q

What is the histologic and clinical presentation of a schwannoma?

A

These are m/c on the flexural aspects of extremities and are often painful

Histologically these only have Schwann cells in it no axons, so there is a well-encapsulated deep dermal or subQ tumor that has both hypercellular Antoni A tissue and hypocellular myxoid type B tissue

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31
Q

What is the IHC staining pattern for schwannoma?

A

s100+, NSE/AXON -, EMA + Myelin basic protein -, s100, vimentin, EMA, type IV collagen all +

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32
Q

What is the histology of a neurofibroma?

A

Non-encapsulated, loosely textured tumor centered in the dermis; grenz zone, thin fascicles of cells w/ a wavy spindle. S-shaped or comma-shaped cells with sharp pointed ends. Mitoses rare. bubblegum stroma and mast cells

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33
Q

What is the clinical presentation of neurofibroma?

A

Clinical: Very common, usually solitary skin-colored soft or rubbery papulonodules w/ “buttonhole sign”, found on trunk/head

Can be pruritic, associated with NF-1

34
Q

What type of neurofibroma is pathognomonic for NF-1?

A

Plexiform Neurofibroma

35
Q

What is the IHC staining for neurofibroma?

A

s100+, myelin basic protein +, Bodian stain shows axons, no capsule (EMA -)! so s100 +, NSE/axon +, EMA -

36
Q

What are the key features of plexiform neurofibroma?

A

Bag of worms feel on the clinical exam. On histology, they show irregular cylindrical or fusiform enlargement of subQ or deep nerves. So large fascicles of neurofibroma surrounded by perineurium often embedded within a diffuse neurofibroma. Essentially large fascicles on a background of neurofibroma

37
Q

What is the malignant transformation potential of plexiform neurofibroma?

A

Higher than solitary neurofibroma

38
Q

What chromosome is altered/abnormal in plexiform neurofibroma?

A

chr 17

39
Q

What is the clinical and histologic presentation of nerve sheath myxoma?

A

Occurs in middle-aged women with lesions on the hand’s knees and extremities.

Histology: hypocellular myxoid stroma with rare, scattered, spindled, stellate, or dendritic cells

Scant, pale cytoplasms with indistinct cytoplasmic contours

Hyperchromatic, ovoid, or angulated nuclei

S100+, vimentin +

40
Q

Staining for neurothekeoma?

A

Think “neurofreakoma” bc it doesn’t stain with S100 like the others

S100A5 +, s100 -, NK1/C3

41
Q

What are the clinical and histologic features of cellular neurothekeoma?

A

Clinical: Occurs on young women (20’s) on the face/extremities, asymptomatic

Histology: ill-defined multilobular fascicles of mainly epithelioid cells and spindle cells with a whorled appearance; possible dystrophic calcification and ossification.

42
Q

What is the clinical presentation of a granular cell tumor?

A

Rare, well-circumscribed, slow-growing asymptomatic or occasionally tender/pruritic, solitary, firm dermal or subcutaneous papulonodule, with brownish-red or flesh-colored smooth glistening surface

Most on head/neck, especially tongue (30-40%)

Adult women of color: 2/3 AA, 2/3 female

43
Q

What is the IHC staining of a granular cell tumor?

A

S100+(unlike xanthoma), CD68+, PAS/diastase resistant +, myelin basic protein +

44
Q

What is the unique IHC staining of a perineuroma?

A

Tumor of only perineural cells as a result it is

s100 -, NSE/axon negative and EMA +, vimentin +, CD34 +, enolase +

45
Q

What disease are perineuromas a/w?

A

NF-1 and NF-2

46
Q

What is the histology of a perineuroma?

A

Circumscribed, non encapsulated lesion; storiform pattern. spindle cells with elongated bipolar cytoplasmic processes. Concentric onion bulb whorls.

47
Q

What dz is malignant peripheral nerve sheath tumor often associated with?

A

NF-1 (50% of cases)

48
Q

Clinical presentation of malignant peripheral nerve sheath tumor?

A

Soft tissue mass on flexor aspects of limbs. Suspect if neurofibroma becomes enlarged and painful. 2-13% of plexiform NF’s can become this. It will be a rapidly growin nodule within the NF. May have large overlying CALM

-Prognosis is poor: 2-3 yrs.

49
Q

What is the pathology/histology of malignant peripheral nerve sheath tumor?

A

Intersecting fascicles of atypical cells in herringbone pattern w/ myxo sarcomatous areas and areas of necrosis and hemorrhagee. very hypercellular and this is more important than atypia. Mitoses are present but not frequent.

50
Q

What is the staining pattern of malignant peripheral nerve sheath tumor?

A

s100+, enolase + MBP+, neurofilament +

51
Q

What is the histologic presentation of Merkel cell carcinoma?

A

Composed of small blue cells with scant cytoplasm and tightly packed nuclei in sheets or a trabecular array. Nuclear molding, apoptotic cells, and mitoses are often present

Poorly defined dermal mass, frequently infiltrating

Various growth patterns

Sheet-like > nested > trabecular

Monotonously uniform, small, round to oval cells abut 2-3x larger than mature lymphocytes

Extensive areas of necrosis, individual cell necrosis, and characteristic crush artifact

52
Q

M/c locations for merkel cell carcinoma?

A

Older adults: head/neck region>exremities>buttocks

53
Q

What virus is Merkel cell carcinoma a/w?

A

Merkel cell polyomavirus (50% exhibit trisomy 6)

54
Q

Staining for merkel cell carcinoma?

A

CK20+, EMA, enolase+, key for CK20 is that it shows paranucelar dots or globules. s100 is negative and TTF is negative (differs from lung small-cell cancer)

also stains for CD20, CK5/6, CK7

55
Q

What is the staging of Merkel cell carcinoma?

A

stage 1 = primary lesion <2cm

stage 2= Primary lesion >2cm

Stage III= Regional lymph node dz

Stage IV= Distant meastatic dz.

56
Q

3 main tumor-like conditions of ectopic and heterotopic neural tissue of skin?

A
  1. neuroglial
  2. meningeal
  3. neuroblastic/ganglionic abnormalities
57
Q

What is the clinical presentation of a nasal glioma?

A

Usually along cranial closure lines w/o apparent communication with underlying structures. Firm, smooth, noncompressible skin-colored to red-purple nodule, 1-3cm

Rare, benign, affects neonates, most commonly near the root of the nose but can be intranasal. This may widen nasal bone leading to hypertelorism

58
Q

Should you bx a nasal glioma?

A

NO - can be associated w/ encephalocele –> image and send to neurosurg.

59
Q

Stains for nasal glioma?

A

s100+ and GFAP + glial fibrillary acid protein

60
Q

What is the clinical appearance of a cutaneous meningioma/rudimentary meningocele?

A

Failure of neuroectoderm to properly separate from surface ectoderm in early gestation

psammoma bodies often seen histology depends on type

type I: confined to subcutis, irregular strands of meningothelial cells in a collagenous stroma

type II and III: may also involve dermis, circumscribed and more cellular than type I

61
Q

What is the clinical presentation of a cutaneous meningioma?

A

Soft to firm, compressible, skin-colored nodule, cyst or plaque, that transilluminates

Rare, usually present in neonates and infants, favors the scalp, forehead, and paravertebral region

Often with partial alopecia or the “hair collar” sign

Other assoc. include brain malformation, hypertelorism, and facial clefting

Enlarge with increased intracranial pressure (crying, straining) as a result of connection to CNS

62
Q

What can cutanoeus menigioma be a/w?

A

Neurofibromatosis or other developmental anomalies

63
Q

What IHC stains can be used for malignant primitive neuroectodermal tumor/neuroblastoma?

A

CD99+, enoase+, EMA -, CK20 -

64
Q

What is a key distinctive histologic feature of neuroblastoma?

A

Small, round, blue cells in nested or infiltrative growth pattern, with the formation of Homer Wright rosettes (fibrillary material in center of rosettes characteristic)

65
Q

Clinical presentation of neuroblastoma?

A

Cutaneous mets seen more in kids; primary cutaneous tumors more often in adults

Cutaneous mets (from adrenals of ganglionic chain) presents as multiple, purple-blue dermal nodules (“blueberry muffin”)

Peripheral blanching after stroking (release of catecholamines)

“Racoon eyes” (periorbital darkening/purpura) –> orbital mets

Assoc. w/ elevated serum and urine catecholamines

66
Q

What factors affect the prognosis of neuroblastoma?

A

Clinical stage, specific genetic factors like MYCN oncogene amplification, and age

67
Q

What percentage of Merkel cell tumors are CK20 negative?

A

~20%

68
Q

What two things do you need to prove a Merkel?

A

Staining for neuroendocrine and keratin (CK20 most specific, so CK19 (second most specific), CAM5.2, other keratin stains.

KEY the keratin stains need to show a perinuclear dot.

69
Q

How do you prove the neuroendocrine origin of Merkel Cell carcinoma

A

Chromogranin-a (most sensitive), Synaptophysin, NSE

NSE is the least useful, but you can use CD56

70
Q

What is the importance of TTF-1 in Merkel cell vs small cell lung cancer, gastric, etc

A

Most Merkel’s are negative, but metastatic dz can be positive.

so once you have established a neuroendocrine tumor, then you check for the TTF-1 to see if it is metastatic

71
Q

What are the 3 main DDx for Merkel cell carcinoma on histology?

A

BCC and lymphoma, and other neuroendocrine tumors

72
Q

What type of virus is the polyomavirus?

A

DNA virus

73
Q

How does the polyomavirus work in cutaneous cells?

A

Incorporates viral DNA into the genome of the affected cells only

74
Q

What types of tumors are a/w immunosuppression due to viral origin?

A

SCC (HPV), Merkel (polyomavirus), Kaposi sarcoma (HHV-8)

75
Q

What markers are important for prognosis in Merkel Cell Carcinoma?

A

p63+, Survivin +, Ki-67+ (high Ki-67 labeling index)

76
Q

How do we use p63 in dermatopathology?

A

it is + in the tissues of origin of the tumor, lost in metastasis.

Ex: squamous cell from the lung vs primary skin? primary skin p63 + and the lung would be -

77
Q

What percent of Merkel cell carcinomas have metastasis at dx?

A

~50%

78
Q

What is the definition of satellite lesions and in-transit metastasis?

A

Satellite = these are within 2 cm of the original tumor

In-transit metastasis = between the 2cm and the basin of the sentinel lymph nodes

79
Q

What is the histology of a granular cell tumor?

A

Superficial non encapsulated irregular arrange sheets of large polyhedral cells with a central hyperchromatic nucleus and coarse granular eosinophilic cytoplasm. Induces pseudoepitheliomatous hyperplasia above the tumor. Large cytoplasmic granules are from lysosome dysfunction and are surrounded by clear halos.

80
Q

What is the histology of a nasal glioma?

A

Lobulated neural tissue (glial cells, astrocytes, rarely true neurons)

Result of embryological displacement of brain tissue

DERM (18) Skin Growths (17 decks)

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