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Pathology > NEURO 4 > Flashcards

NEURO 4 Flashcards

1
Q

What are the three common nerve sheath tumors

A

Schwannomas, neurofibromatosis, and malignant peripheral nerve sheath tumor (MPNST)

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2
Q

Schwannomas

A

Encapsulated , benign tumors that can be associated with NF2

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3
Q

Pathogenic sign for NF2

A

Bilateral acoustic schwannnomas is virtually pathogenic

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4
Q

Neurofibromatosis

A

Benign peripheral nerve sheath tumors sometimes associated with NF1

  • localized
  • cutaneous
  • plexiform
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5
Q

It is though the __ is a malignant transformation of plexiform neurofibroma

A

MPNST

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6
Q

MPNST

A

Can be de novo sporadic neoplasms of NF1 associated tumors arising through malignant transformation of a plexiform neurofibroma

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7
Q

Schwannomas

A

Benign tumors with Schwann cell differentiation that arise directly from peripheral nerves

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8
Q

Forms of schwannoma

A

NF2 and sporadic forms are associated with NF2 gene mutation on chromosome 22

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9
Q

What does NF2 gene code for

A

Merlin, which normally restricts cell surface expression of GF receptors via interactions with the actin cytoskeleton

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10
Q

Schwannoma absence of Merlin

A

Hyperproliferation of cells in response to gF

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11
Q

Schwann cell origin

A

Borne out by their uniform immureactivity for s-100

S for schwann

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12
Q

Morphology schwannoma

A

Well circumscribed, encapsulated masses that abut the nerve but do not invade it, allowing surgical excision-benign

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13
Q

Antoni A areas schwannoma

A

Admixture of dense areas containing spindle cells in intersecting fascicles and Verocay bodies

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14
Q

Verocay bodies

A

Nuclear free zones between pulsating nuclei

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15
Q

Antoni b areas

A

Admixture of loose areas (hypocellular) spindle cells are spread apart by extracellular matrix (may be associated with microcyst formation)

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16
Q

Schwann cells

A

Presence of spindled, elongated nucleus with a wavy or buckled shape

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17
Q

Presentation schwannomas

A

Signs and symptoms due to compression of the involved nerve or adjacent structures (brainstem or spinal cord

Tinnitus and hearing loss

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18
Q

Cranial vault schwannoma

A

Tumors are seen at the cerebellopontine angle (attached to vestibular branch of CNVIII vestibular branch)

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19
Q

Acoustic neuroma

A

Double misnomer. Neighbor a neuroma nor does it arise from acoustic part of nerve

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20
Q

What other nerves can schwannomas appear

A

Sensory (trigeminal and dorsal)

21
Q

Extramural schwannoma

A

Associated with large nerve trunks or as soft tissue lesions without an associated nerve

22
Q

Treatment schwannoma

A

Surgery

23
Q

Neurofibroma

A

Benign, nerve sheath tumors with a more heterogenous composition than schwannomas

24
Q

What are neufibromas derived from

A

Schwann cell lineage

25
Q

Neoplastic Schwann cells are admired with what

A

Perineurial like cells, fibroblasts, mast cells, CD34 spindle cells

26
Q

Cause of neurofibroma

A

Sporadic of NF1 associated

27
Q

Superficial cutaneous growth pattern neurofibroma

A

Pedunculated nodules (isolated-sporadic, multiple-NF1)

28
Q

Diffuse growth pattern neurofibroma

A

Large, plaque like elevation of skin NF1

29
Q

Plexiform growth of neurofibroma

A

Seen in deep or superficial locations, associated with nerve roots or large nerves NF1

Rarely transform to MPNST

30
Q

Pathogenesis neurofibroma

A

Only the schwann cells in neurofibromas show complete loss of NF1 product indicating that these are the neoplastic cells

Neurofibromin is a tumor suppressor that inhibits RAS by stimulating GTPase

NF1 Haplo-insufficient mast cells are hypersensitized to KIT ligand and respond by producing factors to stimulate schwann cell growth

31
Q

Neurofibroma morphology

A

Small, well delineated, unencapsulated nodular lesions

Arise in dermis and SQ fat-benign nodules int he dermis and SQ fat-neurofibromas

Low cellularity and admired with bland schwann cells, stromal cells (mast cells, perineurial cells, CD34 spindle cells, fibroblasts

Stroma contains loose collagen

Dane also structures may be trapped at the edge of lesions

32
Q

Diffuse neurofibroma morphology

A

Diffuselyinfiltrates the dermis and SQ CT

Entrants fat and appendage structures->plaque like appearance 
May grow to be large
Pseudomeissner corpsules (tactile like bodies)

Focal collections of cells mimicking appearance of Meissen corpuscles

33
Q

Plexiform neurofibroma morphology

A

Plexiform neurofibroma has the potential to transform to a malignant peripheral nerve sheath tumor

Grows within and expand the nerve fascicles, entrapping associated axons

Ropy thickening of multipl fascicles->bag of wormsapperance

External perineurial layer of the nerve is preserved individual nodules appear encapsulated

ECM varies from loose/myxoid to collagenous/fibrous

Collagen may be seen in bundles (shredded carrots_

34
Q

Malignant peripheral nerve sheath tumor (MPSNT_

A

85%high grade
50% seen in NF1 patients, result of malignant transformation from plexiform neurofibromas

Can also arise de novo and are associated with larger, peripeheral nerves of the chest, abdomen, pelvis, neck or limb girdle

35
Q

Morphology malignant peripheral nerve sheath tumor

A

Poorly defined tumor masses

Infiltrate along the axis of the parent nerve, invading adjacent soft tissue

Fasciculations arrangement of spindle cells

MARBELIZed appearance due to variation in cellularity )marble like appearance also seen in multicystic encephalopathy)

Mitosis, necrosis, and nuclear anaplastic are common

36
Q

What aremalignant peripheral nerve sheath tumors hard to differentiate from

A

Undifferentiated sarcoma

Helpful clues include a diagnosis of NF1 in the affected patient an a clearly demonstrated an atomic relationship to a nerve or to a preexisting (plexiform) neurofibroma

37
Q

Triton tumor

A

Divergent differentiation

Focal areas with lines of differentiation (glandular , cartilaginous, osseous, rhabdomyoblastic)

38
Q

NF1

A

Systemic disease with non neoplastic manifestations and neurofibromas of all type MPNST, gliomas of the optic nerve, other glial tumors and hamartomatous lesions, pheochromocytoma

Had have nodular lesions distorting thing like the face and ears
Plexiform architecture

39
Q

Mutation NF1

A

AD

17q NF1 LOF (neurofibromin)

40
Q

Penetrate of NF1

A

High

41
Q

Mosaicism NF1

A

Patients exhibit only subtitle features or have disease restricted to a certain body part

42
Q

Presentation NF1

A

Mental retardation or seizures
Skeletal defects
Lisch nodules-pigmentaed nodules in retina

Cafe au last spots-macular cutaneous hyperpigmentation(virtually pathognomonic)

43
Q

NF3

A

Less common than NF1

44
Q

Presentation of NF2

A

Bilateral CNVIII schwannomas and multiple meningiomas

Gliomas (ependymomas of the spinal cord) and also occur

45
Q

NF2 non neoplastic lesions

A

Schwannomas

Meningioangiomatosis

Glial harmartia

46
Q

Schwannosis

A

Nodular ingrowth of schwann cells into the spinal cord

47
Q

Meningioangiomatosis

A

Proliferation of meningeal cells and flood vessels growing in the brain

48
Q

Glial harmartia

A

Microscopic nodular collections of glial cells at abnormal locations (superficial or dep cerebral cortex)

49
Q

Mutations NF2

A

AD
Chromosome 22q12(Merlin) loss of tumor suppressor
Commonly mutate d in sporadic meningiomas and schwannomas
NF1 gene is on chromosome 17

Nonsense and frameshift mutations cause more severe phenotypes than miss ensue mutation

Pathology (36 decks)

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