TUMORS OF PERIPHERAL

NERVES
Presented by
Anjum
Baker
III MDS Postgraduate
Dept of Oral Pathology & Microbiology
VIDS & RC
CONTENTS
■ Introduction
■ Classification
■ Traumatic Neuroma
■ Palisaded encapsulated neuroma
■ Mucosal neuroma
■ Neurofibroma/ Neurofibromatosis
■ Schwannoma
■ Granular cell tumor
■ Nerve Sheath Myxoma
■ Pigmented Neuroectodermal Tumor of Infancy
■ Malignant peripheral nerve sheath tumor
■ Olfactory Neuroblastoma
■ Peripheral neuroectodermal tumour
■ References
 Introduction
■ Peripheral nerve tumors are a heterogeneous and complex
group of
lesions and reflects the intricate structure of peripheral nerves

■ The range of structure and cell types from which the nerve sheath
tumors may either arise, or differentiate toward, is quite broad.
■ Majority of tumors of PNS are derived from Schwann cells and
their peripheral nerve elements.

■ In the oral region, neural tumors occur both in the soft tissues
and in jaw bones.

■ They occur as painless, smooth surface swelling in the

soft tissues, with tongue being the most common site.

■ Within the jaw bones they exhibit a slow rate of growth and mild
expansion of the cortical plates.

■ Majority are benign and the malignant neoplasms generally have

a propensity for local invasion rather than regional or distant
metastasis.
Benign Tumours:
1. Traumatic Neuroma
2. Palisaded encapsulated neuroma
3. Hamartomas – i. Mucosal neuroma
ii.Neuromuscular hamartoma
4. Neurofibroma/ Neurofibromatosis
5. Schwannoma
6. Granular cell tumor
7. Nerve Sheath Myxoma
8. Pigmented Neuroectodermal Tumor of Infancy

Malignant Tumours:
9. Malignant peripheral nerve sheath tumor
10. Olfactory Neuroblastoma
11. Malignant granular cell tumor
12. Peripheral neuroectodermal tumour

WHO CLASSIFICATION OF SOFT TISSUE

 Traumatic Neuroma
■ Occurs in response to injury(lacerating/penetrating)

■ Benign non neoplastic overgrowth of nerve fibres & schwann cells.

■ Traumatic injury  swelling,fragmentation,disintegration of nerves at

distal endsdebris cleared by macrophages,shrinkage of neural
sheathsproliferation of axis cylinders, schwann cells,endoneurium
from proximal endneural reinnervation

■ If proliferating proximal end meets scar tissue/malaligned

bonecontinuous proliferationNeuroma
C/F

■ small, firm, slow growing often painful nodules

■ Oral lesions-near mental foramen, alveolar ridge, lips, tongue

■ May occur centrally in association with nerve trunk

■ Pain on digital pressure/reflex neuralgia

■ Pathologic Features.
■ Haphazard arrangement of small nerve fascicles, containing axons,
Schwann cells, and perineural cells, with surrounding fibrosis
■ No distinctive myelin sheath
■ Reactive changes, such as capillary and myofibroblastic
proliferation.
IHC- CD68+

Differential Diagnosis.
■ Neurofibroma
(does not grow in distinct nerve twigs and consists of wavy spindled
cells dispersed among randomly arranged collagen bundles)
■ Mucosal neuromas
( submucosal nodular lesions without surrounding fibrosis occurring in
patients with MEN 2B)

Treatment
■ Complete excision
Solitary Circumscribed/ Palisaded Encapsulated Neuroma
■ Benign neoplasm/primary hyperplasia of nerve fibres, axons &
Schwann cells

■ 90% are facial lesions.

C/F

■ 5th-7th decade

■ Limited to areas bordering mucocutaneous junctions of face

■ Intra oral – hard palate

■ Solitary sessile well circumscribed papule on the nose, cheek, and

perioral skin less than 1cm diameter

■ Rubbery feel on palpation

Pathologic Features.
■ Lacks a distinct capsule
■ Overlying epithelium- acanthosis/ pseudoepitheliomatous hyperplasia
■ composed of bland eosinophilic spindle cells with poorly defined cell
margins and small wavy hyperchromatic nuclei
■ Cells arranged in fascicles set in collagenous stroma and often
separated by artefactual clefts
IHC- strongly S-100
+,EMA+

Differential Diagnosis.
Schwannomas ( hyalinized, thick-walled vessels or myxoid
change, presence of central axons)

Treatment
No treatment required
Surgical excision for esthetic correction
MUCOSAL NEUROMAS
Multiple Endocrine Neoplasia Syndrome (MEN
IIB/III)
■ Also called multiple mucosal neuroma syndrome

■ Features-

• Adrenal pheochromocytoma

• C cell hyperplasia of thyroid

• Medullary thyroid carcinoma

• Diffuse ganglioneuromatosis of alimentary tract

• Submucosal neuromas of upper aerodigestive tract

■ AD disorder

■ Mutation of RET proto oncogene

C/F
■ Tall lanky marfanoid build with narrow face, muscle wasting
■ Oral mucosal neuromas- 1st decade of life
■ Also on mucosal surfaces of eyelids, GIT
■ Multiple mucosal nodules-2-7mm yellowish white sessile painless
nodules on lips, anterior tongue,commisures
■ 2-8 neuromas usually- Bumpy lip appearance
■ Thyroid Ca, pheochromocytoma- manifest after puberty
■ Increased calcitonin levels, altered epinephrine: norepinephrine ratio
Pathologic Features.
■ Partially encapsulated aggregate of nerves with thickened perineurium,
intertwined in plexiform pattern seen in loose endoneurium like fibrous
stroma
■ Individual nerves flow in fascicles of two to three fibers and are
histologically normal except for occasional hyperplasias and bulbous
expansions.
■ Myxoid change may be prominent
■ IHC- S-100+,collagen
IV+,vimentin+,NSE+,neurofilament+,
■ Special stains- Early lesions- Alcian blue +
■ Luxol fast blue- myelinated nerve fibres
Differential Diagnosis.
■ Traumatic neuromas
(single lesions, history of trauma)
Rx
■ Excision for esthetic reasons
■ Self limiting/asymptomatic
 Neurofibroma/Neurofibromatosis
■ May be solitary or associated with NF
■ Benign tumor derived from endoneural fibroblasts
■ Multiple lesions- Neurofibromatosis/Von Recklinghausen’s disease
■ Autosomal Dominant/spontaneous mutations
■ 2 subtypes
■ NF1- mutation of genes coding for neurofibromin on chromosome 17
(1 in 2500-3300 live births)
■ NF2- mutation of genes coding for Schwannomin on chromosome 22
( 1 in 33,000-40,000 live births)
■ Syndrome associated neurofibromas- malignant transformation rate –
15%, NF2>NF1
Diagnostic Criteria for NF1 (3 or more)
(1) six or more café au lait macules,
(2) two or more neurofibromas of any type,
(3) One plexiform neurofibroma,
(4) axillary or inguinal freckling,
(5) optic glioma,
(6) two or more iris hamartomas (Lisch nodules),
(7) sphenoid dysplasia or thinning of long bone cortex, or
(8) a first-degree relative with known NF1.
Diagnostic criteria for NF2 (any 1)

1. Bilateral acoustic neuroma/family history of NF2+ unilateral

acoustic neuroma/any 2 of meningioma, neurofibroma,
schwannoma,corneal opacities

2. Unilateral acoustic neuroma+any 2 of meningioma, neurofibroma,

schwannoma,corneal opacities

3. 2 or more meningiomas + unilateral acoustic neuroma/any 2 of

glioma,schwannoma or cataract

4. 1st degree relative with NF2 and any 1 of the above criteria
■ Clinical Features.
■ Neurofibroma- Present in young adults as a relatively small, soft,
dermal or subcutaneous nodule.
■ Discrete non ulcerated nodules with normal overlying skin
■ Oral lesions in 7-20% patients, site- buccal mucosa, alv. ridge,
palate
■ Multiple conventional neurofibromas, prompt consideration of
possible NF1
■ Diffuse neurofibromas, associated with NF1 in approximately
10%
of cases, are usually deeply situated.
■ Pain/paresthesia may present
Gross description
■ Not encapsulated, softer (more gelatinous) than schwannoma
■ Superficial tumors are small, pedunculated nodules
■ Deeper tumors are larger, may cause tortuous enlargement of
peripheral nerves- ‘tuber root’ app
■ Plexiform neurofibromas, grossly visible, large masses, with a
characteristic “bag of worms” appearance.
Pathologic Features.

■ Well circumscribed, unencapsulated

■ Elongated spindle cells with poorly defined pale eosinophilic

cytoplasm and tapering wavy or buckled nuclei admixed with
intermediate short spindle cells, numerous small nerve fibres and mast
cells.
■ often show zonation with a more cellular central region containing
residual nerve twigs and more myxoid areas at the periphery.

■ Sometimes spindle cells b/w bundles of collagen arrayed in a

characteristic “shredded carrot” pattern
Subtypes
■ Plexiform neurofibroma: irregularly expanded nerve bundles with nodular
appearance, prominent myxoid matrix; associated with NF1
■ Diffuse cutaneous: traps adnexa, infiltrates into fat
■ Intraneural
■ Epitheloid
■ Granular cell
■ Pigmented
■ Dendritic cell
Positive stains
■ S100, CD34+ (focal), Factor 13a (focal)
■ axons (highlight with silver or acetylcholinesterase stain, NSE,
neurofilament)
■ EMA- in plexiform neurofibromas
Differential diagnosis
■ Palisaded encapsulated neuroma (parallel fascicles)
Rx
■ Asymptomatic/self limiting
■ Periodic monitoring in case of NF1/2
Patients
■ Genetic counselling
 Schwannoma
■ Also known as neurilemoma/ perineural fibroblastoma/neurinoma.

■ Benign tumor of Schwann sheath

■ Defined as - Encapsulated biphasic nerve sheath tumor derived from

Schwann cells with highly ordered cellular component (Antoni A)
that palisades (Verocay bodies) and a myxoid component
(Antoni B)

■ Small tumors may be all Antoni A

■ Schwannomatosis – familial, inactivating mutations of Schwannomin-

multiple peripheral + vestibular schwannomas

■ Schwannomatosis – predisposition for malignant change

■ Clinical features

■ Ages 20-50; M=F

■ Head, neck, flexor upper and lower extremities, retroperitoneum,

■ Intra oral region- slowly enlarging, painless submucosal nodule that is

somewhat movable beneath the surface and rarely becomes larger than 2 cm
in greatest diameter

■ Pain or rapid enlargement of preexisting lesion are suggestive of malignant

change

■ may wax and wane in size

■ Gross description
■ Usually solitary, large tumors may be cystic
■ Gelatinous appearance, periphery may show fibrosis
■ Nerve of origin present in periphery - does penetrate
not
substance of tumor
■ Pathologic Features
■ Encapsulated Biphasic tumor with compact hypercellular Antoni A
areas
and myxoid hypocellular Antoni B areas
■ Antoni A type tissue-
■ Fascicles of monomorphic spindle-shaped Schwann cells with poorly
defined eosinophilic cytoplasm and pointed basophilic nuclei in variably
collagenous stroma
■ These cells show nuclear palisading, and parallel arrays of such
palisades with intervening eosinophilic cytoplasm (cell processes) are
known as Verocay bodies- ‘Soldiers across battle lines’ appearance
■ Antoni B type tissue-
■ Antoni B areas are also composed of Schwann cells, but their cytoplasm is
inconspicuous, and the nuclei appear suspended in a copious myxoid,
often microcystic, matrix. These areas are probably degenerative in
nature.
■ A common feature, most prominent in Antoni B areas, is the presence
of blood vessels with thick hyaline walls and gaping tortuous lumina
with occasional thrombi
■ Normal mitotic figures are a common finding in benign
schwannoma, especially in Antoni A areas, but it is exceptional for
these to exceed 5 per 10 high-power fields (hpf) in number.

■ May have foamy macrophages

■ Often displays degenerative nuclear atypia (ancient change)

■ collagenous spherules: large nodular masses of collagen

with
radiating edges
■ Variants
Ancient schwannoma
■ Degenerative change to tumors, usually large and of long duration,
commonly deep within retroperitoneum
■ Cyst formation, calcification, hemorrhage (stromal hemosiderin),
hyalinization, histiocytic infiltration, severe nuclear atypia (nuclear
hyperchromasia, irregular nuclear shapes)
■ No mitotic figures
Cellular schwannoma
■ Primarily Antoni A areas without Verocay bodies
■ May have nuclear atypia and focal necrosis
■ 0-3 mitotic figures/10HPF
■ 5% recur, no metastases

Pigmented schwannoma
■ Pigmented tumor cells have widely scattered, coarse pigment,
reactive with Fontana Masson stain ,nonreactive with Prussian blue
■ Laminated psammoma bodies also seen
■ Some are clinically aggressive, metastasizing
■ Positive stains-S100 (strong), vimentin,
■ Negative stain-Pussian blue, tyrosinase, HMB45
Plexiform schwannoma
•5% of schwannomas
•Pattern not associated with neurofibromatosis 1 or 2
•Plexiform architecture with nuclear palisading
•Biphasic pattern may not be prominent
•Often cellular with hyperchromatic nuclei and mitotic activity
•No necrosis, no myxoid change

Positive stain- S100 (strong staining of nodules but not intervening

stroma)
Differential diagnosis
•MPNST
•Plexiform neurofibroma
Epitheloid Schwannoma
■ Nests or trabeculae of epitheloid cells in a myxoidstroma
■ Closely resemble myoepithelioma
■ S-100 positive
■ EMA, Keratin – negative
Hybrid Schwannoma/Perineurioma
■ predominantly Antoni A areas; markedly whorled growth
pattern
■ Mixed positivity S-100 & EMA
Glandular Schwannoma
■ Glandular differentiation rare
■ Usually entrapped glandular structures
Microcystic-reticular variant
 Not associated with neurofibromatosis types 1 or 2
 Microcystic and reticular growth pattern with anastomosing and
intersecting spindle cells, distributed around islands of myxoid or
collagenous/hyalinized stroma
 Positive stains- S100, variable GFAP
 Negative stains-Muscle markers, keratin, p63
Angiosarcoma in schwannoma

■ Benign schwannoma with transition to angiosarcoma (irregular

vasoformative structures lined by multilayered cells with nuclear atypia
or atypical endothelial cells with a solid growth pattern,)

■ Cytoplasmic staining for CD31, CD34 or vWF

Epithelioid malignant change

■ Large epithelioid cells with abundant eosinophilic cytoplasm,

vesicular chromatin, prominent nucleoli, resembles epithelioid
MPNST

■ May recur locally, may be a precursor lesion to MPNST since they

present at younger age than MPNST

■ Strongly S100+
IHC
Positive stains
■ EMA (capsule), S100 (Schwann cells),calcinurin, laminin, type 4
collagen, vimentin, CD68, GFAP
Negative stains
■ Keratin, neurofilament, desmin

■ Malignant transformation
■ Transforms to
MPNST, angiosarcoma or epithelioid malignant
change (EMC),common in tumors with epitheloid cells.
■ Most common sites are limb, limb girdles, head/neck
■ Schwannoma with MPNST: benign schwannoma with no other
primary tumor, histologically malignant cells resembling epithelioid
MPNST; 5 year survival < 20%
Nerve sheath Myxoma/
Clinical Features
Neurothekeoma
■ Solitary painless swelling
■ Upper limb/ HN areas
■ adolescents, young adults
■ Female prediliction
■ <3cm size
Pathological Features
■ Multinodular growth pattern and copious myxoid matrix
■ Tumor cells-predominantly spindle shaped, maybe epitheloid OR
multinucleate
■ fascicular or plexiform arrangement in myxoid stroma
■ Rare foci of cartilaginous metaplasia
■ IHC -S100+ (sporadic)
Differential diagnosis- Myxoid neurofibroma: S100+ (strong)
Granular Cell Tumor
■ Usually benign, Neuroectodermal origin

■ C/F

■ Classic location is tongue.

■ Rare congenital tumors occur in gingiva.

■ Female predilection

■ Slow growing, rarely tender.

■ Usually <3cm diameter

Pathological Features
■ Maybe well defined or poorly defined with infiltrative margins

■ Nests or trabeculae of tumor cells seen which are large, rounded, or

polygonal and have copious, finely granular, eosinophilic
cytoplasm.
■ Larger eosinophilic droplets or granules maybe seen.

■ Tumor cell nuclei are small, centrally situated, and

usually pyknotic/hyperchromatic/vesicular.
■ scattered mitoses or mild nuclear atypia, which often
appears degenerative.
■ Nests of tumor cells are commonly found around, or adjacent to, small
nerves, often being located within the epineurium.
■ Superficial tumors- acanthosis or pseudoepitheliomatous hyperplasia of ep

■ Positive stains-NSE, S100 (nuclear and cytoplasmic), inhibin and

calretinin, acid phosphatase, luxol fast blue, PAS
Rx
Surgical excision is curative
Recurrence is mostly due to incomplete excision
Malignant Granular Cell Tumor
Accounts for no more than 2% to 3% of all granular cell tumors
Among the reported cases, more than 50% have pursued a metastasizing or
fatal
clinical course.
Criteria for its recognition are very hard to define because some cases
have appeared histologically to be remarkably bland and monomorphic.
Any unusually large or deep-seated lesion with infiltrative margins or necrosis
and in which cytologic pleomorphism or nucleoli are prominent and mitoses
are quite frequent should be regarded with suspicion
Pigmented neuroectodermal tumor of
infancy
 Also called melanotic neuroectoderrmal tumor of infancy
 Historically called melanotic progonoma /retinal anlage tumor
 Uncommon, rapidly growing mesenchymal tumor of jaw or skull in
infants, neural crest origin, with biphasic histology of melanin
containing cells and neuroblast-like cells.
 C/F
 90% occur in HN region
 Presents in 1st year of life
 Male predilection
 Rapidly growing jaw tumors
 Usually benign but may be locally aggressive or undergo malignant
change
■ Pathological Features
■ two principal cellular components:
■ small basophilic neuroblast-like cells, set in a fibrillary matrix, and larger
epithelioid, eosinophilic cells with vesicular nuclei and containing
variable amounts of melanin pigment (melanocyte-like cells)
■ The melanin-producing cells are often arranged in alveolar or
pseudoglandular structures set in a dense fibroblastic stroma
 Minimal mitotic activity, no atypia
 IHC
 Epitheloid cells: keratin and HMB45, Melan A, Fontana stain
for melanin pigment, rarely vimentin, Ki-67
 Neuroblast like cells: CD57/Leu7 and NSE

 Differential diagnosis
Neuroblastoma/other small round blue cell tumors or melanoma

 Treatment

Excision with negative margins. Recur in 37%, metastasis in 7%, no

prognostic factors have been identified
 MPNST
■ Formerly known as malignant schwannoma/neurofibrosarcoma
C/F
■ Sporadic or NF1 associated (in 30%-40% cases)
■ Sporadic cases associated with previous benign tumors
(Schwannoma, ganglioneuroma) or history of irradiation
■ Peak incidence- 5th decade of life
■ Earlier incidence if NF1 associated.
■ Male preponderance
■ Bulky deep-seated tumor usually arising from major nerves in
limbs, retroperitoneum & HN regions.

■ High clinical suspicion for MPNST in NF1 patient or tumor arising

within anatomic component of a major nerve or contiguous with
neurofibroma
■ most MPNSTs are greater than 10 cm in maximum diameter by the
time of presentation. (unless associated with neurological
symptoms- rare)
■ Gross description
■ Large infiltrative mass producing fusiform enlargement of major
nerve
■ those arising in a preexisting benign tumor may show a zoned
appearance
■ It was formerly believed that to make a diagnosis of MPNST one
must
demonstrate:
■ (1) origin from a nerve or preexisting benign nerve sheath tumor,
■ (2) ultrastructural evidence of Schwann cell differentiation, or
■ (3) development of a spindle cell sarcoma in a patient with NF1
■ Pathological Features
■ Spindle celled fascicular appearance
■ Distinctive features that may suggest
neural differentiation are the abrupt
alternation between cellular and more
myxoid areas and the apparent
perivascular accentuation or
whorling of tumor cells
■ Up to 10% of cases have a copious myxoid stroma throughout.
■ Diagnostic clues at the cellular level are the presence of pale, poorly
defined cytoplasm and nuclei with a narrow, tapering outline, often with a
wavy or buckled configuration in some areas (not as common as in
neurofibromas)
■ Hyperchromatic nuclei ,focally pleomorphic with inconspicuous nucleoli.
■ Nuclear palisading is infrequent
■ Frequent mitotic figures, May have bizarre cells
■ Geographic necrosis with tumor palisading at edges
■ 15% cases have
metaplastic
bone, muscle cartilage,
■ May have
differentiation glandular
for keratin, EMA,
(positive
CEA,
chromogranin)
■ May have melanin in
tumor cells (overlaps with
primary melanoma of
nerves)
Variants

■ Epitheloid MPNST

■ Glandular MPNST

■ Pigmented MPNST

■ IHC -CD99/O13 (86%), S100 (62%), CD57 (55%), collagen IV, , GFAP

Leu7/CD57 in neurofibroma-like areas

P53, topoisomerase II- prognostic markers

■ Differential diagnosis
■ Pleomorphic liposarcoma

■ Amelanotic melanoma

■ Synovial sarcoma: usually CK7+,

CK19+
■ Malignant triton tumor (MTT)
■ MPNST with well developed skeletal muscle component
■ Diagnostic criteria of Woodruff (Cancer 1973;32:426):
– Tumor arises along a peripheral nerve, in a ganglioneuroma, in
NF1 patient, or is a metastasis from such a tumor
– Tumor has characteristics of Schwann cell tumor
– Rhabdomyoblasts arise from body of tumor
■ Rx and Prognosis
■ Wide surgical excision
■ Tumors are chemotherapy and radiotherapy resistant

■ Recur locally, distant metastases frequent

■ Plexiform variant in children has better prognosis, otherwise cannot

predict prognosis

■ 5 yr survival rate -30-60%

OLFACTORY NEUROBLASTOMA

■ Also called esthesioneuroblastoma

■ Rare, malignant neuroectodermal tumor thought to arise from

olfactory membrane or olfactory placode which extends from roof
of nasal cavity in fetus to mid nasal septum and superior turbinate

■ Not related to neuroblastomas elsewhere in body

■ Clinical Features

■ Median age 50 years, range 3-79 years; no gender preference

■ Painful swelling in nasal fossa

■ 20% develop distant metastases, usually to cervical lymph nodes

and lung; late recurrence (after 10 years) is common
■ Usually upper nasal vault; rarely in nasopharynx, maxillary or
ethmoid sinus. Locally invasive into paranasal sinuses, nasopharynx,
palate, orbit, base of skull, brain

■ Staging(Kadish)

A. Confined to nasal cavity

B. Confined to nasal cavity and paranasal sinuses
C. Other
■ 40-50% cases are Stage B
■ 5 year survival by stage:
■ A: 75%, B: 68%, C: 41%
■ Gross description
Red-gray, highly vascular, polypoid mass of soft tissue

■ Pathological Features

■ Nests or sheets of uniform small cells with scant cytoplasm, round

nuclei with indistinct nuclear membrane and punctuate
chromatin, no/indistinct nucleoli
■ Mild to moderate nuclear pleomorphism

■ Prominent fibrillary or reticular background in 85% of cases

■ Variable mitotic figures

■ Variable Homer Wright rosettes (cells surrounding central zones of fibrils)

■ tumor cell melanin maybe present

■ Necrosis is poor prognostic factor

■ IHC
Keratin, NSE (strong), synaptophysin (strong), chromogranin,
neurofilament, catecholamines, S100 in cells at periphery of cell
nests

Differential diagnosis
■ Ewing/PNET
■ Lymphoma
■ Rhabdomyosarcoma

Rx
Surgery/Radiotherpay
 PNET
■ Rare soft tissue tumor, morphologically indistinguishable from Ewing
sarcoma of bone.
■ Definition : a cohesive family of tumors that show varying degrees
of neuronal differentiation and almost invariable EWSR 1 gene
rearrangement, most often as a consequence of a reciprocal t(11;22)
(q24;q12) chromosomal translocation
■ C/F
■ Deep soft tissue of trunk/lower limb is common

■ Usually age 30 or less, usually male preponderance

■ Patients typically present with a rapidly enlarging, often painful mass,
■ Aggressive; common metastases to lung, bones
■ Gross Features
■ Large pale soft irregular masses with central necrosis
■ Pathological Features
■ Predominantly lobular, or sometimes trabecular, growth pattern
■ Associated with a prominent ramifying capillary network.
■ little or no stroma is seen (although rare hyaline examples do
occur)
■ confluent or “filigree” pattern of necrosis is commonly found.
■ Small round/oval cells with scanty cytoplasm containing glycogen
■ Poorly differentiated- ‘dusty chromatin pattern’/ pleomorphism
■ Usually more neuroepithelial features than similar bone tumors
■ Homer wright rosettes
IHC
■ Glycogen, vimentin, CD99 / O13 / mic2, S100, keratin
(20%)

Differential diagnosis

■ Lymphoma

■ Rhabdomyosarcoma

■ Rx & Prognosis
■ Wide surgical excisison
■ Chemotherapy/Radiotherapy resistant
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