Soft tissue pathology the sort of thing you get in exams
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Soft Tissue Pathology The Sort of Thing You Get in Exams!. Dr. Petra Dildey Royal Victoria Infirmary Newcastle upon Tyne. Case 38551/03:. 50y old male patient, soft tissue mass left popliteal fossa. Case 38551/03. Case 38551/03. Case 38551/03. Myxoid Liposarcoma.

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Soft Tissue Pathology The Sort of Thing You Get in Exams!

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Soft Tissue PathologyThe Sort of Thing You Get in Exams!

Dr. Petra Dildey

Royal Victoria InfirmaryNewcastle upon Tyne


Case 38551/03:

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


Case 38551/03


Case 38551/03


Case 38551/03


Myxoid Liposarcoma

  • Myxoid and round cell same category

  • Adults; deep-seated in extremities (thigh)

  • Histology:

    • multinodular with increased cellularity at periphery of nodules

    • myxoid matrix, occ. with mucin pools

    • typical delicate branching vessels

    • bland round to oval mesenchymal cells and univacuolated lipoblasts

    • progression to round cell LPS histological continuum

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


Case 18319/05:

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


Case 18319/05


Case 18319/05


Case 18319/05


Case 18319/05


Low Grade Fibromyxoid Sarcoma

  • Rare soft tissue sarcoma

  • Young to middle-aged adults; extremities and trunk; deep

  • Histology:

    • circumscribed, low to moderate cellularity

    • alternating fibrous and myxoid stroma

    • bland spindle cells, whorled pattern

    • arcades of blood vessels

    • occ. giant collagen rosettes

  • Genetics: t(7;16)(q33;p11)


Case 35841/03:

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


Case 35841/03


Case 35841/03


Intramuscular Myxoma

  • Important DD for myxoid soft tissue tumours

  • Middle-aged to older adults; large muscles of limb girdles

  • Histology:

    • macro circumscribed, but micro infiltrative

    • extensive myxoid matrix, hypocellular

    • bland stellate- and spindle-shaped cells

    • NO mitoses, pleomorphism, necrosis


Case 34761/09:

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


Case 34761/09


Case 34761/09


Case 34761/09


Myxofibrosarcoma

  • Rel. common fibroblastic sarcoma; myxoid MFH

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

  • Histology:

    • multinodular with fibrous septa

    • myxoid stroma

    • atypical spindle-/stellate-shaped cells, occ. pseudolipoblasts

    • curvilinear vessels


Case 19222/04:

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


Case 19222/04


Case 19222/04


Case 19222/04


Extraskeletal Myxoid Chondrosarcoma

  • Rare soft tissue sarcoma

  • Middle-aged to older adults; extremities, limb girdles and other sites; often haemorrhagic

  • Histology:

    • multinodular

    • chondromyxoid matrix

    • cords and networks of cells

    • eosinophilic cytoplasm, uniform nuclei, few mitoses

    • focal S100, occ. cytokeratin and EMA

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


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 30750/03


Case 30750/03


Case 30750/03MyoD1


Alveolar Rhabdomyosarcoma

  • Small round blue cell tumour

  • 10-25 years; often extremities, all other sites possible

  • Histology:

    • 3 subtypes: typical, solid, mixed

    • nests separated by fibrovascular septa

    • small round nuclei, scant cytoplasm

    • horse-shoe giant cells common

    • Myogenin, MyoD1, desmin positive

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


Case 7739/06:

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


Case 7739/06


Case 7739/06


Case 7739/06


Case 7739/06


Case 7739/06CK


Case 7739/06 Desmin


Desmoplastic Small Round Cell Tumour

  • Small round blue cell tumour showing divergent differentiation

  • Children and adolescents, esp. male; abdominal cavity, retroperitoneum, pelvis

  • Histology:

    • nests of variable size surrounded by desmoplastic stroma

    • small uniform cells with round nuclei,

    • occ. rhabdoid inclusions

    • epithelial, smooth muscle and neural markers positive, esp. CK, EMA and desmin (dot-like), WT1

  • Genetics: t(11;22)(p13;q12)


Case 24640/04:

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


Case 24640/04


Case 24640/04


Case 24640/04


Nodular Fasciitis

  • Small fibroblastic proliferation

  • All age groups, mostly young adults; subcutis!, anywhere in body; rapid growth

  • Histology:

    • partly loose/feathery, partly cellular

    • tissue-culture fibroblasts

    • mitotically active

    • collagen bundles, hyalinisation, giant cells

    • SMA positive, desmin negative


NEQAS Case 261:

50y old female patient, small subcutaneous tumour forearm.


NEQAS Case 261


NEQAS Case 261


NEQAS Case 261


Proliferative Fasciitis

  • A small fibroblastic proliferation similar to nodular fasciitis, but with large ganglion-like cells

  • Middle-aged and older adults; subcutis, esp. extremities; rapid growth

  • Histology:

    • ill-defined

    • tissue-culture fibroblasts

    • myxoid/collagenous stroma

    • large, ganglion-like cells with prominent nucleoli


Case 3821/03:

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


Case 3821/03


Case 3821/03


Case 3821/03


Myositis Ossificans

  • Localized, reparative lesion

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

  • Histology:

    • zonation!

    • centre resembling nodular fasciitis

    • then immature unmineralized bone

    • periphery mature bone


Case 17062/04:

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


Case 17062/04


Case 17062/04


Case 17062/04CD117 (c-kit)


GIST

  • KIT-positive mesenchymal tumours primarily of the GI-tract

  • Middle-aged and older adults; stomach, small bowel, rectum & colon in that order (rare oesophagus and elsewhere)

  • Histology:

    • fascicular architecture

    • spindle cells or epithelioid cells, rarely pleomorphic

    • CD117 (c-kit), DOG1, CD34, occ. SMA, desmin, S100

    • behaviour depending on site, max. diameter, mitoses


Case 5365/04:

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


Case 5365/04


Case 5365/04


Case 5365/04

SMA

Desmin


Leiomyosarcoma

  • Soft tissue type

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

  • Histology:

    • typical fascicular pattern

    • eosinophilic cytoplasm and blunt-ended nuclei

    • hyalinization, myxoid change

    • SMA, desmin and caldesmon

    • occ. poorly differentiated, pleomorphic areas (“dedifferentiated”)


Case 14986/04:

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


Case 14986/04


Case 14986/04


Case 14986/04


Fibromatosis

  • Superficial and deep fibroblastic proliferations

  • Age depending on type; superficial: palmar, plantar etc., deep: extra-/intraabdominal

  • Histology:

    • think of it!

    • infiltrative margin

    • cellularity variable, fascicular architecture

    • bland spindle cells

    • collagen occ. hyalinized, prominent small arteries

    • SMA positive, desmin negative, beta-catenin


Case 28715/05:

9y old female patient, 13 cm tumour caecum.


Case 28715/05


Case 28715/05


Case 28715/05


Case 28715/05ALK


Inflammatory Myofibroblastic Tumour

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

  • Lung, abdomen, bladder most common sites

  • Histology:

    • myofibroblasts in fascicular or storiform pattern

    • matrix myxoid to collagenized

    • inflam. infiltrate of lymphocytes, plasma cells, eosinophils

    • SMA, occ. desmin and CK, ALK in 50%


Case 3356/04:

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


Case 3356/04


Case 3356/04


Case 3356/04


Schwannoma

  • Benign nerve sheath tumour

  • All ages; almost any nerve

  • Histology:

    • capsule!

    • thick-walled, hyalinized vessels!

    • Antoni A and B areas, Verocay bodies

    • cystic degeneration, hyalinization, nuclear atypia, calcification, foam cells, haemorrhage

    • cellular, ancient and plexiform variants


Case 7859/04:

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


Case 7859/04


Case 7859/04


Case 7859/04


Case 7859/04CD34


Solitary Fibrous Tumour

  • Cellular SFT syn. to haemangiopericytoma

  • Middle-aged adults; extrapleural variant at any site

  • Histology:

    • circumscribed, patternless

    • alternating hypo- and hypercellular areas

    • hyalinized collagen

    • haemangiopericytomatous vascular pattern

    • small bland cells, few mitoses

    • CD34 90%, CD99 70%, BCL2 30%, occ. EMA & SMA


Case 14005/03:

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


Case 14005/03


Case 14005/03


Case 14005/03


Case 14005/03


Dedifferentiated Liposarcoma

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

  • Adults; retroperitoneum, spermatic cord and other sites

  • Histology:

    • often abrupt transition

    • well-differentiated component

    • dedifferentiated component, low or high grade

    • mostly “MFH”-type, but also heterologous differentiation

    • IHC: CDK4, MDM2


Case 21561/07:

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


Case 21561/07


Case 21561/07


Case 21561/07


Case 21561/07


Case 21561/07HMB45 SMA


Angiomyolipoma

  • Part of PEComas, can be associated with tuberous sclerosis

  • Adults, women>men; mostly kidneys

  • Histology:

    • mature fat / smooth muscle / thick-walled blood vessels

    • smooth muscle often focally epithelioid

    • IHC: HMB45, MelanA, SMA, occ. desmin

    • monotypic epithelioid variant!


Case 28802/10:

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


Case 28802/10


Case 28802/10


Case 28802/10


Case 28802/10


Chondroid Lipoma

  • A unique benign fatty tumour

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

  • Histology:

    • lobulated, circumscribed, nests and cords of cells

    • epithelioid multivacuolated cells, cells with eosinophilic granular cytoplasm, mature adipocytes

    • myxoid and occ. hyaline matrix, vascular

  • Genetics: t(11;16)(q13;p12-13)


Case 158/07:

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


Case 158/07


Case 158/07


Case 158/07


Case 158/07SMA


Myofibroma

  • Solitary or multicentric (myofibromatosis)

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

  • Histology:

    • apparently biphasic

    • plump myofibroblasts in whorls/bundles

    • primitive small round or short spindle cells

    • haemangiopericytomatos vascular pattern

    • hyalinization, calcification, necrosis, haemorrhage

    • SMA positive, desmin negative


Case 4899/08:

10y old boy, 2cm nodule right groin.


Case 4899/08


Case 4899/08


Case 4899/08


Case 4899/08


Case 4899/08

EMA

Desmin


Angiomatoid Fibrous Histiocytoma

  • Peculiar small tumour in skin or subcutis

  • Children and young adults; extremities, trunk, head & neck, often at sites of normal LN

  • Histology:

    • 3 components

    • capsule with lymphoplasmacytic infiltrate

    • blood-filled cystic spaces

    • proliferation of fibrohistiocytic or myofibroblastic cells

    • desmin, EMA, CD68, CD99 in half of cases

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


Case 34141/04:

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


Case 34141/04


Case 34141/04


Case 34141/04


Myogenin

MyoD1

Desmin

Case 34141/04


Pleomorphic Rhabdomyosarcoma

  • Rare RMS subtype in adults

  • Adults >45years; deep-seated in extremities most common

  • Histology:

    • haphazardly arranged bizarre cells with severely pleomorphic nuclei and deeply eosinophilic cytoplasm

    • tadpole and strap cells, occ. cross-striations

    • desmin, MyoD1, Myogenin


Case 33584/03:

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


Case 33584/03


Case 33584/03


Case 33584/03


Case 33584/03DPAS


Alveolar Soft Part Sarcoma

  • Rare soft tissue sarcoma

  • Adolescents and young adults; extremities (adults) and head and neck (children); slow growth

  • Histology:

    • distinct nested pattern, central discohesion

    • round uniform nuclei, granular cytoplasm

    • vascular invasion!

    • DPAS-positive crystals

    • TFE3, occ. muscle markers

  • Genetics: t(X;17)(p11;q25)


Cases 17157/02 & 37608/03:

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 17157/02


Case 17157/02


Case 17157/02


Case 37608/03


Case 37608/03


Case 37608/03


Case 37608/03EMA


Synovial Sarcoma

  • Relatively frequent soft tissue sarcoma

  • Young adults; deep-seated in extremities, but any site possible

  • Histology:

    • cellular!

    • biphasic, monophasic

    • epithelial element in solid nests, glands

    • spindle cells in vague fascicles

    • hyalinisation, calcification, ossification

    • EMA, cytokeratin (7 & 19), BCL2, CD99, S100

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


Case 8960/04:

68y old female patient, recurrent tumour scalp.


Case 8960/04


Case 8960/04


Case 8960/04


Case 8960/04CD31


Angiosarcoma

  • Mostly cutaneous, rarely soft tissue

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

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

  • Histology:

    • multinodular, haemorrhagic, dissecting growth pattern

    • anything from well-formed vessels to solid sheets

    • often marked endothelial atypia and mitoses

    • CD31, CD34, Fli-1, Factor 8 variably positive


Case 7503/01:

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


Case 7503/01


Case 7503/01


Case 7503/01


Case 7503/01S100


Clear Cell Sarcoma

  • Soft tissue sarcoma with melanocytic differentiation

  • Young adults; deep-seated in extremities; slow growth

  • Histology:

    • nested and fascicular architecture

    • polygonal and spindle-shaped cells

    • wreath-like giant cells

    • low mitotic rate

    • S100 and melanocytic markers positive

  • Genetics: t(12;22)(q13;q12)


Case 24689/09:

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


Case 24689/09


Case 24689/09


CK

Case 24689/09

EMA


Case 24689/09CD34


Epithelioid Sarcoma

  • Distinctive sarcoma with epithelioid morphology

  • Classic type: 10-40 years; hands, forearms, lower legs, feet

  • Proximal type: older age; pelvis, perineum, genital tract; aggressive

  • Histology:

    • multinodular, central necrosis

    • large epithelioid cells with eosinophilic cytoplasm, vesicular nuclei, small nucleoli

    • CK-profile (8,18,19), EMA, vimentin, CD34

    • DD: carcinoma, granuloma annulare, rheum. nodule


Minimum DatasetSoft Tissue Sarcomas

  • Clinical: site, depth from surface

  • Trimming:

    • one block per 10mm tumour, max. 12 blocks

    • sample margins <30mm (exceptions)

    • sample fat in retroperitoneal/abdominal/scrotal sarcomas

  • IHC: Ki67, myogenic differentiation

  • Genetic data

  • Report: type/subtype, grade (FNCLCC), margins, tissue planes involved, maximum size


Finally – the End!


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