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Dr. Parampreet Kaur
 perineurium -Several layers of flattened cells
with elongated nuclei
 Schwann cell cytoplasm
well-preserved & has
eosinophilic staining properties
67. peripheral nerves
 Traumatic neuroma
 Morton neuroma
 Benign tumors
◦ Schwannoma
◦ Neurofibroma
◦ Perineuriomas
 Collectively designated as
Malignant
peripheral nerve
Sheath tumors
(MPNSTs).
 TRAUMATIC NEUROMAS.
 Peripheral nerve severed or crushed
 distal end - wallerian degeneration=proximal end-
regenerates.
 Failure to meet-tangled mass of nerve fibers
 Microscopy-All the elements of a nerve recognized: axons,
Schwann cells, perineurial cells, and fibroblasts
 Morton neuroma (Morton metatarsalgia)
 Repeated mild trauma -b/w 3rd and 4th toes. F>M
 Microscopy-affected nerve markedly distorted.
 Extensive perineurial fibrosis, -In a concentric
fashion.
 Arterioles thickened and sometimes occluded by
thrombi
 Palisaded encapsulated neuroma
 proliferation of Schwann cells & numerous axons
within a capsule derived from perineurium
 IHC- Schwann cells -S-100 protein, axons –
neurofilaments
 capsule for EMA, the latter indicates perineurial
cells
 Few truly encapsulated neoplasms
 Any age; typically in adulthood
 Common sites -intracranial sites (cerebellopontine
angle), posterior mediastinum, retroperitoneum, flexor
surface of extremities, and head & neck
 Slow-growing, usually painless tumors
 Often sporadic; <5% occur in patients with NF-2
 Ovoid or fusiform mass, usually <5 cm
 Well-defined
 Typically encapsulated
 Pink to tan, firm C/S
 Focal areas of cystic degeneration
 Hypercellular Antoni A areas
 Hypocellular, myxoid Antoni B
 Hyalinized vessels
 Focal areas of hemorrhage,
hemosiderin deposition, and
xanthomatous change
 Rarely -glandular structures or pure
epithelioid morphology-
 benign epithelioid
 Ancient schwannoma
◦ cytologic atypia/hyalinization
 Cellular schwannoma
◦ Antoni A areas
◦ nuclear atypia, mitotic activity & focal
necrosis
 psammomatous melanotic schwannoma
◦ from the spinal nerve roots
◦ low grade malignancy = local recurrence
 Plexiform
schwannomas
 Epithelioid
schwannoma
67. peripheral nerves
67. peripheral nerves
 S-100 protein-strongly
+ve
 Calretinin, calcineurin
 CD57, CD56, GFAP +ve
 Collagen IV: surround
individual tumor cells
 Neurofibroma
 Leiomyoma
 Malignant peripheral nerve sheath tumor
 Well-defined fusiform lesion -in a/w nerve trunk
 Dermal or S/C tissues throughout the body
 Any age -most commonly in young adults
 Lesions-localized, diffuse, or plexiform, the
latter two having a strong association with NF1
 NF1, von Recklinghausen disease
◦ Autosomal dominant
◦ chromosome 17
◦ Positive family history
◦ Multiple neurofibromas
◦ Café-au-lait spots
(hyperpigmented skin lesions)
◦ Lisch nodules
(pigmented iris hamartomas)
 Not encapsulated, softer than schwannoma
 Firm, gray-white cut surface
 Diffuse lesions -ill-defined, plaque like
thickening of S/C tissues
 Plexiform lesions-Multinodular conglomerate of
lesions -“bag of worms”
 Combined proliferation of all the
elements of a peripheral nerve: axons,
Schwann cells, fibroblasts, and (in the
plexiform type) perineurial cells
 Stroma -mucoid material- occasionally
hyalinized- Wisps of collagen
 Mild nuclear atypia- common
 stroma -numerous mast cells
 Distorted organoid structures
resembling Wagner–Meissner or Pacini
corpuscles sometimes
 May contain melanin pigment -
pigmented neurofibroma
 Epithelioid morphology- Epithelioid
neurofibroma
67. peripheral nerves
 Plexiform neurofibroma
◦ Almost exclusively a/w NF1
◦ Irregularly expanded nerve bundles giving a multinodular
appearance
◦ hypocellular with a prominent myxoid matrix
◦ Variable degree nuclear pleomorphism
◦ Infrequent mitotic activity
 Diffuse neurofibroma-Neoplastic cells expand the
dermal and S/C tissues & envelop S/C & adnexal strs
◦ Wagner meissner bodies
 Pigmented NF
 Schwannoma
 Myxoma
 Malignant peripheral nerve sheath tumor
 Special Stains and IHC
 ● S-100 protein positive
 Exclusively of perineurial cells
Microscopy
 Extremely elongated cells arranged in parallel bundles
 Some hv storiform pattern-storiform perineurial
Fibroma
 Diagnosis- Myxoid lesions of soft ts in which a
storiform or fascicular pattern of growth evident
Variants - sclerosing perineurioma
-reticular (retiform) perineurioma
-plexiform perineurioma
-rare granular cell perineurioma
 Hybrid forms of schwannoma & perineurioma
 malignant forms–Representing subtype of MPNST
 IHC- positive for EMA, Glut-1
& CD34
 negative for S-100 protein
 SITE- skin, soft tissues, intraspinal location
 Gross & microscopy-reminiscent of myxoma, except
for prominent multinodularity with a fibrous border
& presence of plumper, epithelial-like cells &
distinct fascicular or plexiform arrangement-
plexiform myxoma
 D/D
◦ Perineurioma show prominent myxoid features and a
fascicular or storiform pattern of growth
◦ Myxoid neurofibroma
*IHC some of the cells stain for
S-100 protein,
very few for EMA,
and still others for CD34.
 An enlarging mass arising in a/w a major
nerve trunk
 Proximal extremities
 3% -10% of patients with NF1 develop MPNST
 50% are found in patients with NF1;
 often develops after 10 to 20 years
 Sporadic cases typically in adults M=F
 Cases A/W NF at a younger age and show
4:1 male-to-female
 Origin- ? Schwann cells
 fusiform, deep-seated mass
 often within a major nerve
 Typically poorly defined
 Frequently infiltrate along
adjacent nerve or into
adjacent soft tissue
 Tan-white, fleshy C/S
 focal areas of hemorrhage &
necrosis
 Cellular spindle cell tumor
 Fascicular growth pattern
 Alternating Hyper/Hypocellular
 Areas of myxoid stroma
 Nuclear palisading
 whorled nodules of spindle cells
 Perivascular tumor cell condensation
and growth along nerve twigs
 hyperchromatic wavy or buckled
nuclei-Punched out nuclei
 Minimal to marked pleomorphism
 High mitotic activity
 Geographic
necrosis
 Benign or
malignant
heterologous
elements such as
bone, cartilage,
and skeletal
muscle may be
seen
 Glands or
squamous islands
 Epithelioid
malignant MPNST
 Low grade MPNST –Curlicue arrangement of
cells
 Hyalinized cords
& nodules
 Presence of rhabdomyoblastic differentiation
& MPNST
 Desmin +ve
67. peripheral nerves
 S-100 protein focally and weakly positive
 CD56 and CD57 variably positive
 Collagen IV positive around individual tumor
cells
 Cellular schwannoma
 Leiomyosarcoma
 Fibrosarcoma
 Synovial sarcoma (monophasic)
 Clear cell sarcoma (melanoma of soft parts)
 High likelihood of local recurrence & distant
metastasis
 Metastases -lungs, liver,
 Bone; lymph node involvement is rare
 Propensity to spread for considerable
distances along the nerve sheath
 the plexiform variant of MPNST occurring in a
superficial location in children-A/W a better
prognosis
67. peripheral nerves
 flat polygonal cells
 connected to each other by junctional complexes
 numerous pinocytotic vesicles
 membrane-bound organelles-Weibel-Palade
bodies -store Von Willebrand factor.
 Many metabolic functions
 Concerned with the fine control of blood
coagulation, thrombosis & local control of b/v
constriction/dilatation & vessel wall permeability
Capillary hemangioma
(infantile & juvenile hemangioma)
Cavernous hemangioma
 Most common vascular
tumor of infancy,
 first few weeks of life
 Site- head and neck; may
involve the S/C tissue or
occasionally the viscera
 diffuse soft tissue growth
-hemangiomatosis
 Typically -crimson skin
lesion -becomes raised
over time (strawberry
hemangioma)
 grows thru 1st year of life
and regresses over time
 Commonly in children
 Site- skin of the head and
neck (port-wine nevus)
 Typically involves deeper
tissue than capillary
hemangiomas do
 May occur in abdominal
viscera-liver and spleen
 Less likely to regress over
time
 Occasionally a/w Maffucci
syndrome (multiple
enchondromas and vascular
proliferations)
 a spongy, dark-red cut
surface
67. peripheral nerves
Capillary hemangioma
 Lobular architecture -
arborizing, small vascular
channels
 lined by plump to flattened
endothelial cells,
 separated by scant connective
tissue stroma
 Cellular lesions show
inconspicuous capillary lumina;
solid growth pattern and
variable mitotic activity-
(cellular hemangioma)
 Involutional changes- vascular
ectasia and replacement by fat
or fibrous tissue
 Mast cells may be numerous
Cavernous hemangioma
 Typically in subcutaneous tissue
 Dilated, blood-filled, medium- to large-caliber vascular spaces,
 lined by flat endothelial cells
 Large, deep cavernous hemangiomas may undergo thrombosis,
ulceration, and infection .
 Thrombi seen in various stages of organization and
recanalization, the latter including papillary endothelial
hyperplasia (Masson lesion).
Epithelioid hemangioma
 Angiolymphoid hyperplasia with eosinophilia
 head and neck - pruritic red lesion
 may be multifocal -May recur
 Well-circumscribed dermal lesion - small-medium-sized b/v
 Vascular channels lined by plump endothelial cells with abundant
eosinophilic cytoplasm and vesicular oval nuclei
 B/g typically- a lymphocytic infiltrate, with germinal centers;
variable numbers -mast cells, eosinophils, & plasma cells
Pyogenic granuloma (lobular capillary hemangioma ) [LCH])
 Typically a polypoid growth in the skin or oral mucosa
 Commonly a/w pregnancy or 0CP use
 Well circumscribed - lobular architecture
 small, arborizing vascular channels & bland endothelium
Intramuscular angioma
 Capillary or cavernous channels admixed with mature skeletal
muscle and a variable amount of fat- MAY RECUR
Spindle cell hemangioma
 Subcutaneous nodule on distal extremity
 Poorly circumscribed lesion, any age ,m>f
 Biphasic population of solid masses of spindle cells
combined with cavernous vascular channels;
 may contain thrombi
 described as a low-grade angiosarcoma
 A non-neoplastic lesion related to a vascular malformation
67. peripheral nerves
 Thrombomodulin, CD34, CD31, and Fli-1
highlight endothelial cells
 GLUT1 positive in capillary hemangioma of
juvenile type only
 Hemangiopericytoma
 Angiosarcoma
 Kaposi sarcoma
 First described by Masson in hemorrhoidal
vessels as ‘vegetant intravascular
hemangioendothelioma’
 Exuberant organization & recanalization of a
thrombus
 can occur in previously normal vessels or in
varices, hemorrhoids, hematomas, pyogenic
granulomas, hemangiomas, and
angiosarcomas
 papillary formations, anastomosing vascular
channels, and plump endothelial cells.
 Exclusively intravascular
 lack of necrosis, bizarre cells & atypical mitoses
 characteristically fibrinous and/or hyaline (deeply
eosinophilic) appearance of the papillary stalks;
 Frequent finding -residual organizing thrombi
 Tumor differentiates toward modified smooth muscle of
the glomus body-Neuromyoarterial glomus- a normal
arteriovenous shunt abundantly supplied with nerve
fibers and fulfilling a temperature-regulating function
 Age- young adults
 Site- distal extremities,esp fingers and toes
 deep or visceral tumors -rare
 Red and blue subcutaneous nodule -painful
 may be multifocal
 Subungual lesions supplied by numerous nerve fibers
=exquisitely painful,
 Gross
 Typically smaller than 1 cm;
 well-circumscribed dermal or subcutaneous nodule
 Sheets or nests of uniform, round epithelioid cells
with oval nuclei and pale eosinophilic cytoplasm
 Groups of glomus cells may surround dilated
vessels (glomangioma)
 Minimal mitotic activity
 May show focal degenerative nuclear atypia
 solid type-confused with sweat gland tumor, melanocytic
nevus, or metastatic carcinoma.
-particularly when tumor cells very epithelioid and/or grow
in an Indian-file fashion.
 angiomatous type -diffuse quality, looking like an
angiomatosis with an increased number of glomus cells in the
vessel wall
 oncocytic variant -cytoplasm of the glomus cells is packed with
mitochondria,. Mast cells are common
SMA and h-caldesmon positive
CD34 variably +
CD31 and thrombomodulin -ve in tumor cells
Prognosis
 Benign and typically treated by excision; 5% to 10% recur
 Malignant (glomangiosarcoma)- exceedingly rare
 -defined as glomus tumors with a deep location and a size
of more than 2 cm, or atypical mitotic figures, or moderate
to high nuclear grade and five or more mitoses per 50
high-power fields.
 Cellular or cavernous hemangioma
 Paraganglioma
Hemangiopericytoma
 Borderline malignant potential
 Shows differentiation toward modified pericytes
 Adults;
 Usually deep, mostly in legs, pelvis, retroperitoneum
 Often large on clinical detection
GROSS
 Solitary, well-circumscribed, often lobulated
 Gray-white to red-brown cut surface
 Focal hemorrhage or cystic degeneration seen
 Hemangiopericytoma
 Thin-walled staghorn vascular channels
 lined by a single layer of flat endothelium,
 surrounded by perivascular and intervascular proliferation of
uniform-appearing oval to spindle-shaped pericytes
 ill-defined cytoplasmic borders
 Reticulin-rich network surrounding individual pericytes
 Focal hyalinization & myxoid change
 Mitotic rate typically <4 mitotic figures/10 hpf
 variable admixed adipose tissue “lipomatous hemangiopericytoma”
67. peripheral nerves
 Criteria not clear
 Aggressive behavior
 Brisk mitotic activity, nuclear atypia, necrosis,
and hemorrhage;
 All hemangiopericytomas have at least borderline
malignant potential
 Same as hemangiopericytoma, except with
plump, spindled to round myoid cells that
surround vessels with a hemangiopericytoid
pattern
 Subcutaneous lesion
 Distal extremities
 Special Stains and Immunohistochemistry
 CD34: equivocal positivity
 SMA positive in myopericytoma but negative in
conventional hemangiopericytoma
 Myopericytomas that occur in the setting of
immunodeficiency are associated with EBV.
 Solitary fibrous tumor
 Fibrous histiocytoma with hemangiopericytoma-
like areas
 Glomangioma
 Synovial sarcoma (monophasic)
 A diagnosis of exclusion because the
hemangiopericytoma-like pattern may be seen in a
variety of other tumors
 Metastases may appear 10 to 20 years later and
usually involve lung or bone
Hemangiopericytoma-like pattern of growth
 Synovial sarcoma
 MPNST
 Mesenchymal chondrosarcoma
◦ (Contains areas of relatively mature cartilage)
 Phosphaturic mesenchymal tumor
◦ hemangiopericytoma-like areas a/w osteoclast-like
cells, cartilage, and other patterns
 infantile fibrosarcoma
 thymoma
◦ Exhibit epithelial markers
Epithelioid
hemangioendothelioma
Retiform hemangioendothelioma
and papillary intralymphatic
angioendothelioma
(Dabska tumor)
 Any age-rare in children
 Site-dermal or S/C tissues
of extremities
 Rarely multicentric
 Bone/visceral involvement
possible
 Often surrounds a
preexisting blood vessel,
most commonly a vein
 May be a/w edema or
thrombophlebitis
 Metastasis in 33%
 All ages; more
common in adults
 Dabska tumor more
common in children
 Limbs -most
commonly affected
 ability to recur but rare
metastasis
 Violaceous plaques or subcutaneous nodules
 Often multinodular
 Infiltrative borders
 Gray to white variegated cut surfaces
 Epithelioid hemangioendotheliomas a/w
large vessels may resemble organizing
thrombi
Epithelioid hemangioendothelioma
 Cords and nests of polygonal endothelial cells
 Intracellular cytoplasmic lumens with intraluminal RBC’s
 Intramural growth into preexisting vessels
 perivascular extension of tumor
 Bland, round to oval nuclei + eosinophilic cytoplasm
 Background stroma- myxoid or hyalinized
 Low mitotic rate
Retiform hemangioendothelioma &
papillary intralymphatic angioendothelioma
(Dabska tumor)
 Both tumors- Plump cells lining lymphatic-like vascular
channels, with or without intraluminal papillary projections
 Retiform hemangioendothelioma- narrow, arborizing vascular
spaces separated by sclerotic stroma, a lymphocytic infiltrate
 Dabska tumor = Intraluminal tufts of plump endothelium
 Resembles deep lymphangioma;
 Deposits of basement membrane
seen in cellular tufts
 Factor VIIIa, CD31, CD34, thrombomodulin,
 GLUT1 negative
 Keratin -focally +ve in epithelioid
hemangioendothelioma
 Reticulin highlights constituent vessels
 Epithelioid sarcoma
 Epithelioid angiosarcoma
 Kaposi sarcoma
 Rare tumor ; <1% of sarcomas
 Usually seen in adults
 Predilection for skin and superficial soft tissue,
breast, bone, liver, spleen
 Rare in deep soft tissue
 May be a/w chronic lymphedema (typically
postmastectomy), previous therapeutic radiation,
or AV fistulas in renal transplant recipients
 Angiosarcomas of liver are a/w prior exposure to
polyvinyl chloride and thorium dioxide
(Thorotrast)
 Cutaneous angiosarcoma- as an ill-defined,
bruiselike lesion or ulcerated hemorrhagic
nodules, or plaques simulating erysipelas
 Commonly large hemorrhagic, ill-defined masses
with spongy quality and blood-filled spaces and
deeply invasive
 •Primarily constituted by epithelioid or fusiform cells
 Rudimentary vascular differentiation;
 pleomorphism, mitoses, and widespread tissue
infiltration
 •Tumor cells in vascular spaces may be attenuated or
plump with hyperchromatic nuclei
 •Spindle cell areas may resemble fibrosarcoma or other
spindle cell tumors
67. peripheral nerves
 C
 Y
 T
 O
 L
 O
 G
 Y
 •Thrombomodulin, CD31, CD34, and Fli-1
positive
 •Cytokeratin may be +ve in epithelioid variant
Other Techniques for Diagnosis
 •Electron microscopy may demonstrate
cytoplasmic Weibel-Palade bodies in roughly
25% of cases
 Hemangioma
 Papillary endothelial hyperplasia
(intravascular hemangioendothelioma of
Masson)
 Epithelioid hemangioendothelioma
 Metastatic renal cell carcinoma, because of its
high degree of vascularity
(no clear cells in angioSA)
 Treatment
◦ radical surgery and radiation therapy
 recurrence and distant metastasis
◦ most commonly to lungs, lymph nodes, and bone
 Prognosis
◦ Related to size, multifocality, and ability to achieve
a complete excision
 Slowly progressive multifocal vasoproliferative
lesion
 low-grade malignancy-in the AIDS population
 In persons with other forms of
immunosuppression
◦ organ transplant recipients
◦ An A/W the systemic form of Castleman disease
◦ Angioimmunoblastic lymphadenopathy (two disorders of
the immune system)
◦ AIDS, Kaposi sarcoma- predominantly a feature of
homosexual population
◦ Also seen in heterosexual drug users
 Multiple blue dermal plaques or nodules
 Starting on the feet and legs
 Ocasionally a pedunculated appearance
reminiscent of pyogenic granuloma
 Temporary control by irradiation, chemotherapy,
or, if the lesion is sharply delimited, excision.
 Course – variable
◦ Increased incidence of malignancy- lymphoid type
◦ Lymph nodes and the GIT most commonly involved.
◦ Indicators of poor outcome-immunosuppression &
age>50 yrs
Classic Kaposi sarcoma
 Affects -males of Eastern European /Mediterranean
 Slowly developing nodules/plaques affecting lower
extremities
Endemic Kaposi sarcoma
 Native blacks in Central Africa
 Younger patients and children
Epidemic Kaposi sarcoma
 In immunocompromised states
 A/W HIV infection
 Typically involves trunk and mucosal surfaces
Kaposi sarcoma a/w iatrogenic immunosuppression
 Immunosuppressed states, a/w Rx for transplant
rejection,
 Typical feature-spindle cells
forming slits containing RBC’s
 Mitotic activity –moderate
 Peomorphism -usually absent.
 Admixed-Lymphocytes,
hemosiderin-laden
macrophages, & other
inflammatory cells.
 Variously sized PAS +ve
hyaline bodies, seen in the
cytoplasm of the proliferating
cells and sometimes
extracellularly =Represent the
result of ingestion and
degradation of erythrocytes
and – -not specific
 Early lesions-Spindle proliferation limited to
the papillary dermis and to the vascular
plexus surrounding the secretory coil of
sweat glands
Early patch stage
 Slitlike spaces b/w collagen bundles that often follow adnexal
structures & preexisting blood vessels that appear to protrude into
newly formed blood vessels (promontory sign)
 Extravasated RBC’s & plasma cells may be present
Plaque stage
 spindle-shaped cells proliferation,arranged as short fascicles
 Diffuse proliferation of blood vessels
 Intracytoplasmic hyaline globules
Nodular stage
 Well-defined nodules of vascular spaces
 Spindle-shaped cells replace the dermis
 Hemosiderin-laden macrophages in the vicinity
 Intracellular & extracellular hyaline globules
Late aggressive lesions of Kaposi sarcoma
 Features of an aggressive sarcoma
 Greater degree of cytologic atypia and high mitotic rate
 Special Stains and Immunohistochemistry
 Hyaline globules-PAS +ve & diastase resistant
 Vascular nature-confirmed by immunostains
CD31 and CD34
 Other Techniques for Diagnosis
 Demonstration of
◦ HHV-8
 Early lesions -from benign vascular
proliferations
◦ Targetoid hemosiderotic hemangioma
◦ Fibrous histiocytoma
◦ Kaposiform haemangioendothelioma
 Late aggressive forms-from other aggressive
sarcomas and require IHC
◦ Angiosarcoma

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67. peripheral nerves

  • 2.  perineurium -Several layers of flattened cells with elongated nuclei  Schwann cell cytoplasm well-preserved & has eosinophilic staining properties
  • 4.  Traumatic neuroma  Morton neuroma  Benign tumors ◦ Schwannoma ◦ Neurofibroma ◦ Perineuriomas  Collectively designated as Malignant peripheral nerve Sheath tumors (MPNSTs).
  • 5.  TRAUMATIC NEUROMAS.  Peripheral nerve severed or crushed  distal end - wallerian degeneration=proximal end- regenerates.  Failure to meet-tangled mass of nerve fibers  Microscopy-All the elements of a nerve recognized: axons, Schwann cells, perineurial cells, and fibroblasts
  • 6.  Morton neuroma (Morton metatarsalgia)  Repeated mild trauma -b/w 3rd and 4th toes. F>M  Microscopy-affected nerve markedly distorted.  Extensive perineurial fibrosis, -In a concentric fashion.  Arterioles thickened and sometimes occluded by thrombi  Palisaded encapsulated neuroma  proliferation of Schwann cells & numerous axons within a capsule derived from perineurium  IHC- Schwann cells -S-100 protein, axons – neurofilaments  capsule for EMA, the latter indicates perineurial cells
  • 7.  Few truly encapsulated neoplasms  Any age; typically in adulthood  Common sites -intracranial sites (cerebellopontine angle), posterior mediastinum, retroperitoneum, flexor surface of extremities, and head & neck  Slow-growing, usually painless tumors  Often sporadic; <5% occur in patients with NF-2
  • 8.  Ovoid or fusiform mass, usually <5 cm  Well-defined  Typically encapsulated  Pink to tan, firm C/S  Focal areas of cystic degeneration
  • 9.  Hypercellular Antoni A areas  Hypocellular, myxoid Antoni B  Hyalinized vessels  Focal areas of hemorrhage, hemosiderin deposition, and xanthomatous change  Rarely -glandular structures or pure epithelioid morphology-  benign epithelioid
  • 10.  Ancient schwannoma ◦ cytologic atypia/hyalinization  Cellular schwannoma ◦ Antoni A areas ◦ nuclear atypia, mitotic activity & focal necrosis  psammomatous melanotic schwannoma ◦ from the spinal nerve roots ◦ low grade malignancy = local recurrence
  • 14.  S-100 protein-strongly +ve  Calretinin, calcineurin  CD57, CD56, GFAP +ve  Collagen IV: surround individual tumor cells
  • 15.  Neurofibroma  Leiomyoma  Malignant peripheral nerve sheath tumor
  • 16.  Well-defined fusiform lesion -in a/w nerve trunk  Dermal or S/C tissues throughout the body  Any age -most commonly in young adults  Lesions-localized, diffuse, or plexiform, the latter two having a strong association with NF1  NF1, von Recklinghausen disease ◦ Autosomal dominant ◦ chromosome 17 ◦ Positive family history ◦ Multiple neurofibromas ◦ Café-au-lait spots (hyperpigmented skin lesions) ◦ Lisch nodules (pigmented iris hamartomas)
  • 17.  Not encapsulated, softer than schwannoma  Firm, gray-white cut surface  Diffuse lesions -ill-defined, plaque like thickening of S/C tissues  Plexiform lesions-Multinodular conglomerate of lesions -“bag of worms”
  • 18.  Combined proliferation of all the elements of a peripheral nerve: axons, Schwann cells, fibroblasts, and (in the plexiform type) perineurial cells  Stroma -mucoid material- occasionally hyalinized- Wisps of collagen  Mild nuclear atypia- common  stroma -numerous mast cells  Distorted organoid structures resembling Wagner–Meissner or Pacini corpuscles sometimes  May contain melanin pigment - pigmented neurofibroma  Epithelioid morphology- Epithelioid neurofibroma
  • 20.  Plexiform neurofibroma ◦ Almost exclusively a/w NF1 ◦ Irregularly expanded nerve bundles giving a multinodular appearance ◦ hypocellular with a prominent myxoid matrix ◦ Variable degree nuclear pleomorphism ◦ Infrequent mitotic activity
  • 21.  Diffuse neurofibroma-Neoplastic cells expand the dermal and S/C tissues & envelop S/C & adnexal strs ◦ Wagner meissner bodies  Pigmented NF
  • 22.  Schwannoma  Myxoma  Malignant peripheral nerve sheath tumor  Special Stains and IHC  ● S-100 protein positive
  • 23.  Exclusively of perineurial cells Microscopy  Extremely elongated cells arranged in parallel bundles  Some hv storiform pattern-storiform perineurial Fibroma  Diagnosis- Myxoid lesions of soft ts in which a storiform or fascicular pattern of growth evident
  • 24. Variants - sclerosing perineurioma -reticular (retiform) perineurioma -plexiform perineurioma -rare granular cell perineurioma  Hybrid forms of schwannoma & perineurioma  malignant forms–Representing subtype of MPNST  IHC- positive for EMA, Glut-1 & CD34  negative for S-100 protein
  • 25.  SITE- skin, soft tissues, intraspinal location  Gross & microscopy-reminiscent of myxoma, except for prominent multinodularity with a fibrous border & presence of plumper, epithelial-like cells & distinct fascicular or plexiform arrangement- plexiform myxoma  D/D ◦ Perineurioma show prominent myxoid features and a fascicular or storiform pattern of growth ◦ Myxoid neurofibroma *IHC some of the cells stain for S-100 protein, very few for EMA, and still others for CD34.
  • 26.  An enlarging mass arising in a/w a major nerve trunk  Proximal extremities  3% -10% of patients with NF1 develop MPNST  50% are found in patients with NF1;  often develops after 10 to 20 years  Sporadic cases typically in adults M=F  Cases A/W NF at a younger age and show 4:1 male-to-female  Origin- ? Schwann cells
  • 27.  fusiform, deep-seated mass  often within a major nerve  Typically poorly defined  Frequently infiltrate along adjacent nerve or into adjacent soft tissue  Tan-white, fleshy C/S  focal areas of hemorrhage & necrosis
  • 28.  Cellular spindle cell tumor  Fascicular growth pattern  Alternating Hyper/Hypocellular  Areas of myxoid stroma  Nuclear palisading  whorled nodules of spindle cells  Perivascular tumor cell condensation and growth along nerve twigs  hyperchromatic wavy or buckled nuclei-Punched out nuclei  Minimal to marked pleomorphism  High mitotic activity
  • 29.  Geographic necrosis  Benign or malignant heterologous elements such as bone, cartilage, and skeletal muscle may be seen  Glands or squamous islands  Epithelioid malignant MPNST
  • 30.  Low grade MPNST –Curlicue arrangement of cells  Hyalinized cords & nodules
  • 31.  Presence of rhabdomyoblastic differentiation & MPNST  Desmin +ve
  • 33.  S-100 protein focally and weakly positive  CD56 and CD57 variably positive  Collagen IV positive around individual tumor cells
  • 34.  Cellular schwannoma  Leiomyosarcoma  Fibrosarcoma  Synovial sarcoma (monophasic)  Clear cell sarcoma (melanoma of soft parts)
  • 35.  High likelihood of local recurrence & distant metastasis  Metastases -lungs, liver,  Bone; lymph node involvement is rare  Propensity to spread for considerable distances along the nerve sheath  the plexiform variant of MPNST occurring in a superficial location in children-A/W a better prognosis
  • 37.  flat polygonal cells  connected to each other by junctional complexes  numerous pinocytotic vesicles  membrane-bound organelles-Weibel-Palade bodies -store Von Willebrand factor.  Many metabolic functions  Concerned with the fine control of blood coagulation, thrombosis & local control of b/v constriction/dilatation & vessel wall permeability
  • 38. Capillary hemangioma (infantile & juvenile hemangioma) Cavernous hemangioma  Most common vascular tumor of infancy,  first few weeks of life  Site- head and neck; may involve the S/C tissue or occasionally the viscera  diffuse soft tissue growth -hemangiomatosis  Typically -crimson skin lesion -becomes raised over time (strawberry hemangioma)  grows thru 1st year of life and regresses over time  Commonly in children  Site- skin of the head and neck (port-wine nevus)  Typically involves deeper tissue than capillary hemangiomas do  May occur in abdominal viscera-liver and spleen  Less likely to regress over time  Occasionally a/w Maffucci syndrome (multiple enchondromas and vascular proliferations)  a spongy, dark-red cut surface
  • 40. Capillary hemangioma  Lobular architecture - arborizing, small vascular channels  lined by plump to flattened endothelial cells,  separated by scant connective tissue stroma  Cellular lesions show inconspicuous capillary lumina; solid growth pattern and variable mitotic activity- (cellular hemangioma)  Involutional changes- vascular ectasia and replacement by fat or fibrous tissue  Mast cells may be numerous
  • 41. Cavernous hemangioma  Typically in subcutaneous tissue  Dilated, blood-filled, medium- to large-caliber vascular spaces,  lined by flat endothelial cells  Large, deep cavernous hemangiomas may undergo thrombosis, ulceration, and infection .  Thrombi seen in various stages of organization and recanalization, the latter including papillary endothelial hyperplasia (Masson lesion).
  • 42. Epithelioid hemangioma  Angiolymphoid hyperplasia with eosinophilia  head and neck - pruritic red lesion  may be multifocal -May recur  Well-circumscribed dermal lesion - small-medium-sized b/v  Vascular channels lined by plump endothelial cells with abundant eosinophilic cytoplasm and vesicular oval nuclei  B/g typically- a lymphocytic infiltrate, with germinal centers; variable numbers -mast cells, eosinophils, & plasma cells Pyogenic granuloma (lobular capillary hemangioma ) [LCH])  Typically a polypoid growth in the skin or oral mucosa  Commonly a/w pregnancy or 0CP use  Well circumscribed - lobular architecture  small, arborizing vascular channels & bland endothelium Intramuscular angioma  Capillary or cavernous channels admixed with mature skeletal muscle and a variable amount of fat- MAY RECUR
  • 43. Spindle cell hemangioma  Subcutaneous nodule on distal extremity  Poorly circumscribed lesion, any age ,m>f  Biphasic population of solid masses of spindle cells combined with cavernous vascular channels;  may contain thrombi  described as a low-grade angiosarcoma  A non-neoplastic lesion related to a vascular malformation
  • 45.  Thrombomodulin, CD34, CD31, and Fli-1 highlight endothelial cells  GLUT1 positive in capillary hemangioma of juvenile type only
  • 47.  First described by Masson in hemorrhoidal vessels as ‘vegetant intravascular hemangioendothelioma’  Exuberant organization & recanalization of a thrombus  can occur in previously normal vessels or in varices, hemorrhoids, hematomas, pyogenic granulomas, hemangiomas, and angiosarcomas
  • 48.  papillary formations, anastomosing vascular channels, and plump endothelial cells.  Exclusively intravascular  lack of necrosis, bizarre cells & atypical mitoses  characteristically fibrinous and/or hyaline (deeply eosinophilic) appearance of the papillary stalks;  Frequent finding -residual organizing thrombi
  • 49.  Tumor differentiates toward modified smooth muscle of the glomus body-Neuromyoarterial glomus- a normal arteriovenous shunt abundantly supplied with nerve fibers and fulfilling a temperature-regulating function  Age- young adults  Site- distal extremities,esp fingers and toes  deep or visceral tumors -rare  Red and blue subcutaneous nodule -painful  may be multifocal  Subungual lesions supplied by numerous nerve fibers =exquisitely painful,  Gross  Typically smaller than 1 cm;  well-circumscribed dermal or subcutaneous nodule
  • 50.  Sheets or nests of uniform, round epithelioid cells with oval nuclei and pale eosinophilic cytoplasm  Groups of glomus cells may surround dilated vessels (glomangioma)  Minimal mitotic activity  May show focal degenerative nuclear atypia
  • 51.  solid type-confused with sweat gland tumor, melanocytic nevus, or metastatic carcinoma. -particularly when tumor cells very epithelioid and/or grow in an Indian-file fashion.  angiomatous type -diffuse quality, looking like an angiomatosis with an increased number of glomus cells in the vessel wall  oncocytic variant -cytoplasm of the glomus cells is packed with mitochondria,. Mast cells are common
  • 52. SMA and h-caldesmon positive CD34 variably + CD31 and thrombomodulin -ve in tumor cells Prognosis  Benign and typically treated by excision; 5% to 10% recur  Malignant (glomangiosarcoma)- exceedingly rare  -defined as glomus tumors with a deep location and a size of more than 2 cm, or atypical mitotic figures, or moderate to high nuclear grade and five or more mitoses per 50 high-power fields.
  • 53.  Cellular or cavernous hemangioma  Paraganglioma
  • 54. Hemangiopericytoma  Borderline malignant potential  Shows differentiation toward modified pericytes  Adults;  Usually deep, mostly in legs, pelvis, retroperitoneum  Often large on clinical detection GROSS  Solitary, well-circumscribed, often lobulated  Gray-white to red-brown cut surface  Focal hemorrhage or cystic degeneration seen
  • 55.  Hemangiopericytoma  Thin-walled staghorn vascular channels  lined by a single layer of flat endothelium,  surrounded by perivascular and intervascular proliferation of uniform-appearing oval to spindle-shaped pericytes  ill-defined cytoplasmic borders  Reticulin-rich network surrounding individual pericytes  Focal hyalinization & myxoid change  Mitotic rate typically <4 mitotic figures/10 hpf  variable admixed adipose tissue “lipomatous hemangiopericytoma”
  • 57.  Criteria not clear  Aggressive behavior  Brisk mitotic activity, nuclear atypia, necrosis, and hemorrhage;  All hemangiopericytomas have at least borderline malignant potential
  • 58.  Same as hemangiopericytoma, except with plump, spindled to round myoid cells that surround vessels with a hemangiopericytoid pattern  Subcutaneous lesion  Distal extremities  Special Stains and Immunohistochemistry  CD34: equivocal positivity  SMA positive in myopericytoma but negative in conventional hemangiopericytoma  Myopericytomas that occur in the setting of immunodeficiency are associated with EBV.
  • 59.  Solitary fibrous tumor  Fibrous histiocytoma with hemangiopericytoma- like areas  Glomangioma  Synovial sarcoma (monophasic)  A diagnosis of exclusion because the hemangiopericytoma-like pattern may be seen in a variety of other tumors  Metastases may appear 10 to 20 years later and usually involve lung or bone
  • 60. Hemangiopericytoma-like pattern of growth  Synovial sarcoma  MPNST  Mesenchymal chondrosarcoma ◦ (Contains areas of relatively mature cartilage)  Phosphaturic mesenchymal tumor ◦ hemangiopericytoma-like areas a/w osteoclast-like cells, cartilage, and other patterns  infantile fibrosarcoma  thymoma ◦ Exhibit epithelial markers
  • 61. Epithelioid hemangioendothelioma Retiform hemangioendothelioma and papillary intralymphatic angioendothelioma (Dabska tumor)  Any age-rare in children  Site-dermal or S/C tissues of extremities  Rarely multicentric  Bone/visceral involvement possible  Often surrounds a preexisting blood vessel, most commonly a vein  May be a/w edema or thrombophlebitis  Metastasis in 33%  All ages; more common in adults  Dabska tumor more common in children  Limbs -most commonly affected  ability to recur but rare metastasis
  • 62.  Violaceous plaques or subcutaneous nodules  Often multinodular  Infiltrative borders  Gray to white variegated cut surfaces  Epithelioid hemangioendotheliomas a/w large vessels may resemble organizing thrombi
  • 63. Epithelioid hemangioendothelioma  Cords and nests of polygonal endothelial cells  Intracellular cytoplasmic lumens with intraluminal RBC’s  Intramural growth into preexisting vessels  perivascular extension of tumor  Bland, round to oval nuclei + eosinophilic cytoplasm  Background stroma- myxoid or hyalinized  Low mitotic rate
  • 64. Retiform hemangioendothelioma & papillary intralymphatic angioendothelioma (Dabska tumor)  Both tumors- Plump cells lining lymphatic-like vascular channels, with or without intraluminal papillary projections  Retiform hemangioendothelioma- narrow, arborizing vascular spaces separated by sclerotic stroma, a lymphocytic infiltrate  Dabska tumor = Intraluminal tufts of plump endothelium  Resembles deep lymphangioma;  Deposits of basement membrane seen in cellular tufts
  • 65.  Factor VIIIa, CD31, CD34, thrombomodulin,  GLUT1 negative  Keratin -focally +ve in epithelioid hemangioendothelioma  Reticulin highlights constituent vessels
  • 66.  Epithelioid sarcoma  Epithelioid angiosarcoma  Kaposi sarcoma
  • 67.  Rare tumor ; <1% of sarcomas  Usually seen in adults  Predilection for skin and superficial soft tissue, breast, bone, liver, spleen  Rare in deep soft tissue  May be a/w chronic lymphedema (typically postmastectomy), previous therapeutic radiation, or AV fistulas in renal transplant recipients  Angiosarcomas of liver are a/w prior exposure to polyvinyl chloride and thorium dioxide (Thorotrast)
  • 68.  Cutaneous angiosarcoma- as an ill-defined, bruiselike lesion or ulcerated hemorrhagic nodules, or plaques simulating erysipelas  Commonly large hemorrhagic, ill-defined masses with spongy quality and blood-filled spaces and deeply invasive
  • 69.  •Primarily constituted by epithelioid or fusiform cells  Rudimentary vascular differentiation;  pleomorphism, mitoses, and widespread tissue infiltration  •Tumor cells in vascular spaces may be attenuated or plump with hyperchromatic nuclei  •Spindle cell areas may resemble fibrosarcoma or other spindle cell tumors
  • 71.  C  Y  T  O  L  O  G  Y
  • 72.  •Thrombomodulin, CD31, CD34, and Fli-1 positive  •Cytokeratin may be +ve in epithelioid variant Other Techniques for Diagnosis  •Electron microscopy may demonstrate cytoplasmic Weibel-Palade bodies in roughly 25% of cases
  • 73.  Hemangioma  Papillary endothelial hyperplasia (intravascular hemangioendothelioma of Masson)  Epithelioid hemangioendothelioma  Metastatic renal cell carcinoma, because of its high degree of vascularity (no clear cells in angioSA)
  • 74.  Treatment ◦ radical surgery and radiation therapy  recurrence and distant metastasis ◦ most commonly to lungs, lymph nodes, and bone  Prognosis ◦ Related to size, multifocality, and ability to achieve a complete excision
  • 75.  Slowly progressive multifocal vasoproliferative lesion  low-grade malignancy-in the AIDS population  In persons with other forms of immunosuppression ◦ organ transplant recipients ◦ An A/W the systemic form of Castleman disease ◦ Angioimmunoblastic lymphadenopathy (two disorders of the immune system) ◦ AIDS, Kaposi sarcoma- predominantly a feature of homosexual population ◦ Also seen in heterosexual drug users
  • 76.  Multiple blue dermal plaques or nodules  Starting on the feet and legs  Ocasionally a pedunculated appearance reminiscent of pyogenic granuloma  Temporary control by irradiation, chemotherapy, or, if the lesion is sharply delimited, excision.  Course – variable ◦ Increased incidence of malignancy- lymphoid type ◦ Lymph nodes and the GIT most commonly involved. ◦ Indicators of poor outcome-immunosuppression & age>50 yrs
  • 77. Classic Kaposi sarcoma  Affects -males of Eastern European /Mediterranean  Slowly developing nodules/plaques affecting lower extremities Endemic Kaposi sarcoma  Native blacks in Central Africa  Younger patients and children Epidemic Kaposi sarcoma  In immunocompromised states  A/W HIV infection  Typically involves trunk and mucosal surfaces Kaposi sarcoma a/w iatrogenic immunosuppression  Immunosuppressed states, a/w Rx for transplant rejection,
  • 78.  Typical feature-spindle cells forming slits containing RBC’s  Mitotic activity –moderate  Peomorphism -usually absent.  Admixed-Lymphocytes, hemosiderin-laden macrophages, & other inflammatory cells.  Variously sized PAS +ve hyaline bodies, seen in the cytoplasm of the proliferating cells and sometimes extracellularly =Represent the result of ingestion and degradation of erythrocytes and – -not specific
  • 79.  Early lesions-Spindle proliferation limited to the papillary dermis and to the vascular plexus surrounding the secretory coil of sweat glands
  • 80. Early patch stage  Slitlike spaces b/w collagen bundles that often follow adnexal structures & preexisting blood vessels that appear to protrude into newly formed blood vessels (promontory sign)  Extravasated RBC’s & plasma cells may be present Plaque stage  spindle-shaped cells proliferation,arranged as short fascicles  Diffuse proliferation of blood vessels  Intracytoplasmic hyaline globules Nodular stage  Well-defined nodules of vascular spaces  Spindle-shaped cells replace the dermis  Hemosiderin-laden macrophages in the vicinity  Intracellular & extracellular hyaline globules Late aggressive lesions of Kaposi sarcoma  Features of an aggressive sarcoma  Greater degree of cytologic atypia and high mitotic rate
  • 81.  Special Stains and Immunohistochemistry  Hyaline globules-PAS +ve & diastase resistant  Vascular nature-confirmed by immunostains CD31 and CD34  Other Techniques for Diagnosis  Demonstration of ◦ HHV-8
  • 82.  Early lesions -from benign vascular proliferations ◦ Targetoid hemosiderotic hemangioma ◦ Fibrous histiocytoma ◦ Kaposiform haemangioendothelioma  Late aggressive forms-from other aggressive sarcomas and require IHC ◦ Angiosarcoma