Soft Tissue PathologySoft Tissue PathologyThe Sort of Thing You Get in Exams!The Sort of Thing You Get in Exams!

Dr. Petra DildeyDr. Petra DildeyRoyal Victoria InfirmaryRoyal Victoria InfirmaryNewcastle upon TyneNewcastle upon Tyne

Case 38551/03:Case 38551/03:

50y old male patient, soft tissue mass left popliteal fossa.

Case 38551/03Case 38551/03

Case 38551/03Case 38551/03

Case 38551/03Case 38551/03

Myxoid LiposarcomaMyxoid Liposarcoma

Myxoid and round cell same categoryMyxoid and round cell same categoryAdults; deep-seated in extremities (thigh)Adults; deep-seated in extremities (thigh)Histology:Histology:– multinodular with increased cellularity at periphery of multinodular with increased cellularity at periphery of

nodulesnodules– myxoid matrix, occ. with mucin poolsmyxoid matrix, occ. with mucin pools– typical delicate branching vesselstypical delicate branching vessels– bland round to oval mesenchymal cells and bland round to oval mesenchymal cells and

univacuolated lipoblastsunivacuolated lipoblasts– progression to round cell LPS histological continuumprogression to round cell LPS histological continuum

Genetics: t(12;16)(q13;p11), t(12;22)(q13;q12)Genetics: t(12;16)(q13;p11), t(12;22)(q13;q12)

Case 18319/05:Case 18319/05:

33y old male patient, 4cm deep soft tissue tumour right forearm.

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Case 18319/05Case 18319/05

Low Grade Fibromyxoid SarcomaLow Grade Fibromyxoid Sarcoma

Rare soft tissue sarcomaRare soft tissue sarcomaYoung to middle-aged adults; extremities and Young to middle-aged adults; extremities and trunk; deeptrunk; deepHistology:Histology:– circumscribed, low to moderate cellularitycircumscribed, low to moderate cellularity– alternating fibrous and myxoid stromaalternating fibrous and myxoid stroma– bland spindle cells, whorled patternbland spindle cells, whorled pattern– arcades of blood vesselsarcades of blood vessels– occ. giant collagen rosettesocc. giant collagen rosettes

Genetics: t(7;16)(q33;p11)Genetics: t(7;16)(q33;p11)

Case 35841/03:Case 35841/03:

48y old female patient, 6.5cm intramuscular tumour left buttock.

Case 35841/03Case 35841/03

Case 35841/03Case 35841/03

Intramuscular MyxomaIntramuscular Myxoma

Important DD for myxoid soft tissue tumoursImportant DD for myxoid soft tissue tumoursMiddle-aged to older adults; large muscles of Middle-aged to older adults; large muscles of limb girdleslimb girdlesHistology:Histology:– macro circumscribed, but micro infiltrativemacro circumscribed, but micro infiltrative– extensive myxoid matrix, hypocellularextensive myxoid matrix, hypocellular– bland stellate- and spindle-shaped cellsbland stellate- and spindle-shaped cells– NO mitoses, pleomorphism, necrosisNO mitoses, pleomorphism, necrosis

Case 34761/09:Case 34761/09:

77y old male patient, 4cm superficial mass right upper arm.

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MyxofibrosarcomaMyxofibrosarcoma

Rel. common fibroblastic sarcoma; myxoid MFHRel. common fibroblastic sarcoma; myxoid MFH

Elderly patients; limbs and limb girdles; Elderly patients; limbs and limb girdles; subcutaneous and deepsubcutaneous and deep

Histology:Histology:– multinodular with fibrous septamultinodular with fibrous septa– myxoid stromamyxoid stroma– atypical spindle-/stellate-shaped cells, occ. atypical spindle-/stellate-shaped cells, occ.

pseudolipoblastspseudolipoblasts– curvilinear vesselscurvilinear vessels

Case 19222/04:Case 19222/04:

66y old male patient, haemorrhagic soft tissue tumour right calf.

Case 19222/04Case 19222/04

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Extraskeletal Myxoid Extraskeletal Myxoid ChondrosarcomaChondrosarcoma

Rare soft tissue sarcomaRare soft tissue sarcomaMiddle-aged to older adults; extremities, limb Middle-aged to older adults; extremities, limb girdles and other sites; often haemorrhagicgirdles and other sites; often haemorrhagicHistology:Histology:– multinodularmultinodular– chondromyxoid matrixchondromyxoid matrix– cords and networks of cellscords and networks of cells– eosinophilic cytoplasm, uniform nuclei, few mitoseseosinophilic cytoplasm, uniform nuclei, few mitoses– focal S100, occ. cytokeratin and EMAfocal S100, occ. cytokeratin and EMA

Genetics: t(9;22(q22;12), t(9;17)(q22;q11), Genetics: t(9;22(q22;12), t(9;17)(q22;q11), t(9;15)(q22;q21)t(9;15)(q22;q21)

Case 30750/03:Case 30750/03:

15y old male patient, large pelvic mass and lymphadenopathy as well as mediastinal and lung lesions on CT. Groin node biopsied.

Case 30750/03Case 30750/03

Case 30750/03Case 30750/03

Case 30750/03Case 30750/03MyoD1MyoD1

Alveolar RhabdomyosarcomaAlveolar Rhabdomyosarcoma

Small round blue cell tumourSmall round blue cell tumour10-25 years; often extremities, all other sites 10-25 years; often extremities, all other sites possiblepossibleHistology:Histology:– 3 subtypes: typical, solid, mixed3 subtypes: typical, solid, mixed– nests separated by fibrovascular septanests separated by fibrovascular septa– small round nuclei, scant cytoplasmsmall round nuclei, scant cytoplasm– horse-shoe giant cells commonhorse-shoe giant cells common– Myogenin, MyoD1, desmin positiveMyogenin, MyoD1, desmin positive

Genetics: t(1;13)(p36;q14), t(2;13)(q35;q14)Genetics: t(1;13)(p36;q14), t(2;13)(q35;q14)

Case 7739/06:Case 7739/06:

36y old female patient, large 36y old female patient, large tumour tail of pancreas with liver tumour tail of pancreas with liver metastases.metastases.

Case 7739/06Case 7739/06

Case 7739/06Case 7739/06

Case 7739/06Case 7739/06

Case 7739/06Case 7739/06

Case 7739/06Case 7739/06CKCK

Case 7739/06 Case 7739/06 DesminDesmin

Desmoplastic Small Round Cell Desmoplastic Small Round Cell TumourTumour

Small round blue cell tumour showing divergent Small round blue cell tumour showing divergent differentiationdifferentiationChildren and adolescents, esp. male; abdominal Children and adolescents, esp. male; abdominal cavity, retroperitoneum, pelviscavity, retroperitoneum, pelvisHistology:Histology:– nests of variable size surrounded by desmoplastic stromanests of variable size surrounded by desmoplastic stroma– small uniform cells with round nuclei,small uniform cells with round nuclei,– occ. rhabdoid inclusionsocc. rhabdoid inclusions– epithelial, smooth muscle and neural markers positive, epithelial, smooth muscle and neural markers positive,

esp. CK, EMA and desmin (dot-like), WT1esp. CK, EMA and desmin (dot-like), WT1

Genetics: t(11;22)(p13;q12)Genetics: t(11;22)(p13;q12)

Case 24640/04:Case 24640/04:

42y old male patient, small nodule in the subcutis of the right buttock.

Case 24640/04 Case 24640/04

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Nodular FasciitisNodular Fasciitis

Small fibroblastic proliferationSmall fibroblastic proliferationAll age groups, mostly young adults; subcutis!, All age groups, mostly young adults; subcutis!, anywhere in body; rapid growthanywhere in body; rapid growthHistology:Histology:– partly loose/feathery, partly cellularpartly loose/feathery, partly cellular– tissue-culture fibroblaststissue-culture fibroblasts– mitotically activemitotically active– collagen bundles, hyalinisation, giant cellscollagen bundles, hyalinisation, giant cells– SMA positive, desmin negativeSMA positive, desmin negative

NEQAS Case 261:NEQAS Case 261:

50y old female patient, small 50y old female patient, small subcutaneous tumour forearm.subcutaneous tumour forearm.

NEQAS Case 261NEQAS Case 261

NEQAS Case 261NEQAS Case 261

NEQAS Case 261NEQAS Case 261

Proliferative FasciitisProliferative Fasciitis

A small fibroblastic proliferation similar to A small fibroblastic proliferation similar to nodular fasciitis, but with large ganglion-like cellsnodular fasciitis, but with large ganglion-like cellsMiddle-aged and older adults; subcutis, esp. Middle-aged and older adults; subcutis, esp. extremities; rapid growthextremities; rapid growthHistology:Histology:– ill-definedill-defined– tissue-culture fibroblaststissue-culture fibroblasts– myxoid/collagenous stromamyxoid/collagenous stroma– large, ganglion-like cells with prominent nucleolilarge, ganglion-like cells with prominent nucleoli

Case 3821/03:Case 3821/03:

18y old female patient, calcified soft tissue mass right thigh, recent increase in size, vague history of trauma.

Case 3821/03Case 3821/03

Case 3821/03Case 3821/03

Case 3821/03Case 3821/03

Myositis OssificansMyositis Ossificans

Localized, reparative lesionLocalized, reparative lesion

Any age, mostly young adults; anywhere; Any age, mostly young adults; anywhere; history of trauma; rapid growthhistory of trauma; rapid growth

Histology:Histology:– zonation!zonation!– centre resembling nodular fasciitiscentre resembling nodular fasciitis– then immature unmineralized bonethen immature unmineralized bone– periphery mature boneperiphery mature bone

Case 17062/04:Case 17062/04:

61y old female patient, 4cm tumour in gastric fundus.

Case 17062/04Case 17062/04

Case 17062/04Case 17062/04

Case 17062/04Case 17062/04CD117 (c-kit)CD117 (c-kit)

GISTGIST

KIT-positive mesenchymal tumours primarily of KIT-positive mesenchymal tumours primarily of the GI-tractthe GI-tractMiddle-aged and older adults; stomach, small Middle-aged and older adults; stomach, small bowel, rectum & colon in that order (rare bowel, rectum & colon in that order (rare oesophagus and elsewhere)oesophagus and elsewhere)Histology:Histology:– fascicular architecturefascicular architecture– spindle cells or epithelioid cells, rarely pleomorphicspindle cells or epithelioid cells, rarely pleomorphic– CD117 (c-kit), DOG1, CD34, occ. SMA, desmin, S100CD117 (c-kit), DOG1, CD34, occ. SMA, desmin, S100– behaviour depending on site, max. diameter, mitosesbehaviour depending on site, max. diameter, mitoses

Case 5365/04:Case 5365/04:

60y old male patient, 10cm tumour around left common iliac vessels.

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Case 5365/04Case 5365/04

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SMASMA DesminDesmin

LeiomyosarcomaLeiomyosarcoma

Soft tissue typeSoft tissue type

Middle-aged and older adults; retroperitoneum, Middle-aged and older adults; retroperitoneum, large vessels and other soft tissue siteslarge vessels and other soft tissue sites

Histology:Histology:– typical fascicular patterntypical fascicular pattern– eosinophilic cytoplasm and blunt-ended nucleieosinophilic cytoplasm and blunt-ended nuclei– hyalinization, myxoid changehyalinization, myxoid change– SMA, desmin and caldesmonSMA, desmin and caldesmon– occ. poorly differentiated, pleomorphic areas occ. poorly differentiated, pleomorphic areas

(“dedifferentiated”)(“dedifferentiated”)

Case 14986/04:Case 14986/04:

33y old female patient, soft tissue tumour abdominal wall.

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Case 14986/04Case 14986/04

Case 14986/04Case 14986/04

FibromatosisFibromatosis

Superficial and deep fibroblastic proliferationsSuperficial and deep fibroblastic proliferationsAge depending on type; superficial: palmar, Age depending on type; superficial: palmar, plantar etc., deep: extra-/intraabdominalplantar etc., deep: extra-/intraabdominalHistology:Histology:– think of it!think of it!– infiltrative margininfiltrative margin– cellularity variable, fascicular architecturecellularity variable, fascicular architecture– bland spindle cellsbland spindle cells– collagen occ. hyalinized, prominent small arteriescollagen occ. hyalinized, prominent small arteries– SMA positive, desmin negative, beta-cateninSMA positive, desmin negative, beta-catenin

Case 28715/05:Case 28715/05:

9y old female patient, 13 cm 9y old female patient, 13 cm tumour caecum.tumour caecum.

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Case 28715/05Case 28715/05

Case 28715/05Case 28715/05ALKALK

Inflammatory Myofibroblastic Inflammatory Myofibroblastic TumourTumour

Heterogenous group of tumours; primarily, a Heterogenous group of tumours; primarily, a visceral and soft tissue tumour in children and visceral and soft tissue tumour in children and adolescents often with ALK gene rearrangementadolescents often with ALK gene rearrangement

Lung, abdomen, bladder most common sitesLung, abdomen, bladder most common sites

Histology:Histology:– myofibroblasts in fascicular or storiform patternmyofibroblasts in fascicular or storiform pattern– matrix myxoid to collagenizedmatrix myxoid to collagenized– inflam. infiltrate of lymphocytes, plasma cells, inflam. infiltrate of lymphocytes, plasma cells,

eosinophilseosinophils– SMA, occ. desmin and CK, ALK in 50% SMA, occ. desmin and CK, ALK in 50%

Case 3356/04:Case 3356/04:

45y old female patient, 5cm soft tissue tumour left calf.

Case 3356/04Case 3356/04

Case 3356/04Case 3356/04

Case 3356/04Case 3356/04

SchwannomaSchwannoma

Benign nerve sheath tumourBenign nerve sheath tumourAll ages; almost any nerveAll ages; almost any nerveHistology:Histology:– capsule!capsule!– thick-walled, hyalinized vessels!thick-walled, hyalinized vessels!– Antoni A and B areas, Verocay bodiesAntoni A and B areas, Verocay bodies– cystic degeneration, hyalinization, nuclear cystic degeneration, hyalinization, nuclear

atypia, calcification, foam cells, haemorrhageatypia, calcification, foam cells, haemorrhage– cellular, ancient and plexiform variantscellular, ancient and plexiform variants

Case 7859/04:Case 7859/04:

63y old female patient, 17.5cm mass left upper quadrant of abdomen.

Case 7859/04Case 7859/04

Case 7859/04Case 7859/04

Case 7859/04Case 7859/04

Case 7859/04Case 7859/04CD34CD34

Solitary Fibrous TumourSolitary Fibrous Tumour

Cellular SFT syn. to haemangiopericytomaCellular SFT syn. to haemangiopericytomaMiddle-aged adults; extrapleural variant at any Middle-aged adults; extrapleural variant at any sitesiteHistology:Histology:– circumscribed, patternlesscircumscribed, patternless– alternating hypo- and hypercellular areasalternating hypo- and hypercellular areas– hyalinized collagenhyalinized collagen– haemangiopericytomatous vascular patternhaemangiopericytomatous vascular pattern– small bland cells, few mitosessmall bland cells, few mitoses– CD34 90%, CD99 70%, BCL2 30%, occ. EMA & SMACD34 90%, CD99 70%, BCL2 30%, occ. EMA & SMA

Case 14005/03:Case 14005/03:

69y old male patient, deep soft tissue mass left thigh.

Case 14005/03Case 14005/03

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Case 14005/03Case 14005/03

Dedifferentiated LiposarcomaDedifferentiated Liposarcoma

In up to 10% of well-diff. liposarcomasIn up to 10% of well-diff. liposarcomas

Adults; retroperitoneum, spermatic cord and Adults; retroperitoneum, spermatic cord and other sitesother sites

Histology:Histology:– often abrupt transitionoften abrupt transition– well-differentiated componentwell-differentiated component– dedifferentiated component, low or high gradededifferentiated component, low or high grade– mostly “MFH”-type, but also heterologous mostly “MFH”-type, but also heterologous

differentiationdifferentiation– IHC: CDK4, MDM2IHC: CDK4, MDM2

Case 21561/07:Case 21561/07:

59y old female patient, 3.3kg / 59y old female patient, 3.3kg / 26cm tumour retroperitoneum.26cm tumour retroperitoneum.

Case 21561/07Case 21561/07

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HMB45 HMB45 SMA SMA

AngiomyolipomaAngiomyolipoma

Part of PEComas, can be associated with Part of PEComas, can be associated with tuberous sclerosistuberous sclerosisAdults, women>men; mostly kidneysAdults, women>men; mostly kidneysHistology:Histology:– mature fat / smooth muscle / thick-walled mature fat / smooth muscle / thick-walled

blood vesselsblood vessels– smooth muscle often focally epithelioidsmooth muscle often focally epithelioid– IHC: HMB45, MelanA, SMA, occ. desmin IHC: HMB45, MelanA, SMA, occ. desmin – monotypic epithelioid variant!monotypic epithelioid variant!

Case 28802/10:Case 28802/10:

21y old female patient, 4cm mass 21y old female patient, 4cm mass in left tibialis anterior muscle. in left tibialis anterior muscle.

Case 28802/10Case 28802/10

Case 28802/10Case 28802/10

Case 28802/10Case 28802/10

Case 28802/10Case 28802/10

Chondroid LipomaChondroid Lipoma

A unique benign fatty tumourA unique benign fatty tumour

Young adults, 80% women; prox. limbs & limb Young adults, 80% women; prox. limbs & limb girdles; deep & subcutaneousgirdles; deep & subcutaneous

Histology:Histology:– lobulated, circumscribed, nests and cords of cells lobulated, circumscribed, nests and cords of cells – epithelioid multivacuolated cells, cells with epithelioid multivacuolated cells, cells with

eosinophilic granular cytoplasm, mature adipocyteseosinophilic granular cytoplasm, mature adipocytes– myxoid and occ. hyaline matrix, vascularmyxoid and occ. hyaline matrix, vascular

Genetics: t(11;16)(q13;p12-13)Genetics: t(11;16)(q13;p12-13)

Case 158/07:Case 158/07:

14y old female patient, small soft tissue tumour left knee.

Case 158/07Case 158/07

Case 158/07Case 158/07

Case 158/07Case 158/07

Case 158/07Case 158/07SMASMA

MyofibromaMyofibroma

Solitary or multicentric (myofibromatosis)Solitary or multicentric (myofibromatosis)

All ages, commonly in infants & children; solitary: skin, All ages, commonly in infants & children; solitary: skin, muscle, multiple: skin, viscera, muscle, bonemuscle, multiple: skin, viscera, muscle, bone

Histology:Histology:– apparently biphasicapparently biphasic– plump myofibroblasts in whorls/bundlesplump myofibroblasts in whorls/bundles– primitive small round or short spindle cellsprimitive small round or short spindle cells– haemangiopericytomatos vascular patternhaemangiopericytomatos vascular pattern– hyalinization, calcification, necrosis, haemorrhagehyalinization, calcification, necrosis, haemorrhage– SMA positive, desmin negativeSMA positive, desmin negative

Case 4899/08:Case 4899/08:

10y old boy, 2cm nodule right groin.

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Case 4899/08Case 4899/08

Case 4899/08Case 4899/08

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Case 4899/08Case 4899/08

EMAEMA DesminDesmin

Angiomatoid Fibrous HistiocytomaAngiomatoid Fibrous Histiocytoma

Peculiar small tumour in skin or subcutisPeculiar small tumour in skin or subcutisChildren and young adults; extremities, trunk, Children and young adults; extremities, trunk, head & neck, often at sites of normal LNhead & neck, often at sites of normal LNHistology:Histology:– 3 components3 components– capsule with lymphoplasmacytic infiltratecapsule with lymphoplasmacytic infiltrate– blood-filled cystic spacesblood-filled cystic spaces– proliferation of fibrohistiocytic or myofibroblastic cellsproliferation of fibrohistiocytic or myofibroblastic cells– desmin, EMA, CD68, CD99 in half of casesdesmin, EMA, CD68, CD99 in half of cases

Genetics: t(12;22)(q13;q12), t(12;16)(q13;p11), Genetics: t(12;22)(q13;q12), t(12;16)(q13;p11), t(1;22)(q33;q12)t(1;22)(q33;q12)

Case 34141/04:Case 34141/04:

65y old male patient, recurrent deep soft tissue tumour left thigh.

Case 34141/04Case 34141/04

Case 34141/04Case 34141/04

Case 34141/04Case 34141/04

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Myogenin MyoD1

Desmin

Pleomorphic RhabdomyosarcomaPleomorphic Rhabdomyosarcoma

Rare RMS subtype in adultsRare RMS subtype in adultsAdults >45years; deep-seated in Adults >45years; deep-seated in extremities most commonextremities most commonHistology:Histology:– haphazardly arranged bizarre cells with haphazardly arranged bizarre cells with

severely pleomorphic nuclei and deeply severely pleomorphic nuclei and deeply eosinophilic cytoplasmeosinophilic cytoplasm

– tadpole and strap cells, occ. cross-striationstadpole and strap cells, occ. cross-striations– desmin, MyoD1, Myogenindesmin, MyoD1, Myogenin

Case 33584/03:Case 33584/03:

35y old male patient, 12cm tumour in right iliac fossa adjacent to right psoas muscle, kidney and adrenal gland.

Case 33584/03Case 33584/03

Case 33584/03Case 33584/03

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Case 33584/03Case 33584/03DPASDPAS

Alveolar Soft Part SarcomaAlveolar Soft Part Sarcoma

Rare soft tissue sarcomaRare soft tissue sarcomaAdolescents and young adults; extremities Adolescents and young adults; extremities (adults) and head and neck (children); slow (adults) and head and neck (children); slow growthgrowthHistology:Histology:– distinct nested pattern, central discohesiondistinct nested pattern, central discohesion– round uniform nuclei, granular cytoplasmround uniform nuclei, granular cytoplasm– vascular invasion!vascular invasion!– DPAS-positive crystalsDPAS-positive crystals– TFE3, occ. muscle markersTFE3, occ. muscle markers

Genetics: t(X;17)(p11;q25)Genetics: t(X;17)(p11;q25)

Cases 17157/02 & 37608/03:Cases 17157/02 & 37608/03:

17157/02: 17157/02: 61y old female patient, rapidly growing recurrent soft tissue tumour left foot.

37608/03: 32y old male patient, growing painful soft tissue mass right thigh.

Case 17157/02Case 17157/02

Case 17157/02Case 17157/02

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Case 37608/03Case 37608/03

Case 37608/03Case 37608/03

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Case 37608/03Case 37608/03EMAEMA

Synovial SarcomaSynovial Sarcoma

Relatively frequent soft tissue sarcomaRelatively frequent soft tissue sarcomaYoung adults; deep-seated in extremities, but any site Young adults; deep-seated in extremities, but any site possiblepossibleHistology:Histology:– cellular!cellular!– biphasic, monophasicbiphasic, monophasic– epithelial element in solid nests, glandsepithelial element in solid nests, glands– spindle cells in vague fasciclesspindle cells in vague fascicles– hyalinisation, calcification, ossificationhyalinisation, calcification, ossification– EMA, cytokeratin (7 & 19), BCL2, CD99, S100EMA, cytokeratin (7 & 19), BCL2, CD99, S100

Genetics: t(X;18)(p11;q11), t(X;20)(p11;q13)Genetics: t(X;18)(p11;q11), t(X;20)(p11;q13)

Case 8960/04:Case 8960/04:

68y old female patient, recurrent tumour scalp.

Case 8960/04Case 8960/04

Case 8960/04Case 8960/04

Case 8960/04Case 8960/04

Case 8960/04Case 8960/04CD31CD31

AngiosarcomaAngiosarcoma

Mostly cutaneous, rarely soft tissueMostly cutaneous, rarely soft tissue

Skin: lymphoedema, post-irradiation; adults; site Skin: lymphoedema, post-irradiation; adults; site dep. on aetiologydep. on aetiology

Soft tissue: any age; any site; with syndromesSoft tissue: any age; any site; with syndromes

Histology:Histology:– multinodular, haemorrhagic, dissecting growth patternmultinodular, haemorrhagic, dissecting growth pattern– anything from well-formed vessels to solid sheetsanything from well-formed vessels to solid sheets– often marked endothelial atypia and mitosesoften marked endothelial atypia and mitoses– CD31, CD34, Fli-1, Factor 8 variably positiveCD31, CD34, Fli-1, Factor 8 variably positive

Case 7503/01:Case 7503/01:

18y old female patient, 5cm soft tissue tumour around the right ankle.

Case 7503/01Case 7503/01

Case 7503/01Case 7503/01

Case 7503/01Case 7503/01

Case 7503/01Case 7503/01S100S100

Clear Cell SarcomaClear Cell Sarcoma

Soft tissue sarcoma with melanocytic Soft tissue sarcoma with melanocytic differentiationdifferentiationYoung adults; deep-seated in extremities; slow Young adults; deep-seated in extremities; slow growthgrowthHistology:Histology:– nested and fascicular architecturenested and fascicular architecture– polygonal and spindle-shaped cellspolygonal and spindle-shaped cells– wreath-like giant cellswreath-like giant cells– low mitotic ratelow mitotic rate– S100 and melanocytic markers positiveS100 and melanocytic markers positive

Genetics: t(12;22)(q13;q12)Genetics: t(12;22)(q13;q12)

Case 24689/09:Case 24689/09:

64y old male patient, 2cm tumour medial aspect left wrist associated with tendon sheath.

Case 24689/09Case 24689/09

Case 24689/09Case 24689/09

Case 24689/09Case 24689/09

EMACK

Case 24689/09Case 24689/09CD34CD34

Epithelioid SarcomaEpithelioid Sarcoma

Distinctive sarcoma with epithelioid morphologyDistinctive sarcoma with epithelioid morphologyClassic type: 10-40 years; hands, forearms, Classic type: 10-40 years; hands, forearms, lower legs, feet lower legs, feet Proximal type: older age; pelvis, perineum, Proximal type: older age; pelvis, perineum, genital tract; aggressivegenital tract; aggressiveHistology:Histology:– multinodular, central necrosismultinodular, central necrosis– large epithelioid cells with eosinophilic cytoplasm, large epithelioid cells with eosinophilic cytoplasm,

vesicular nuclei, small nucleolivesicular nuclei, small nucleoli– CK-profile (8,18,19), EMA, vimentin, CD34CK-profile (8,18,19), EMA, vimentin, CD34– DD: carcinoma, granuloma annulare, rheum. noduleDD: carcinoma, granuloma annulare, rheum. nodule

Minimum DatasetMinimum DatasetSoft Tissue SarcomasSoft Tissue Sarcomas

Clinical: site, depth from surfaceClinical: site, depth from surfaceTrimming:Trimming:– one block per 10mm tumour, max. 12 blocksone block per 10mm tumour, max. 12 blocks– sample margins <30mm (exceptions)sample margins <30mm (exceptions)– sample fat in retroperitoneal/abdominal/scrotal sample fat in retroperitoneal/abdominal/scrotal

sarcomassarcomas

IHC: Ki67, myogenic differentiationIHC: Ki67, myogenic differentiationGenetic dataGenetic dataReport: type/subtype, grade (FNCLCC), Report: type/subtype, grade (FNCLCC), margins, tissue planes involved, maximum sizemargins, tissue planes involved, maximum size

Finally – the End!Finally – the End!