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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

Soft Tissue PathologyThe Sort of Thing You Get in Exams!

Dr. Petra Dildey

Royal Victoria InfirmaryNewcastle upon Tyne


Case 38551 03

Case 38551/03:

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





Myxoid liposarcoma
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

Case 18319/05:

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






Low grade fibromyxoid sarcoma
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

Case 35841/03:

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




Intramuscular myxoma
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

Case 34761/09:

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





Myxofibrosarcoma
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

Case 19222/04:

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





Extraskeletal myxoid chondrosarcoma
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

Case 30750/03:

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





Alveolar rhabdomyosarcoma
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

Case 7739/06:

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








Desmoplastic small round cell tumour
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

Case 24640/04:

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





Nodular fasciitis
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

NEQAS Case 261:

50y old female patient, small subcutaneous tumour forearm.





Proliferative fasciitis
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

Case 3821/03:

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





Myositis ossificans
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

Case 17062/04:

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




Case 17062 04 cd117 c kit
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

Case 5365/04:

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




Case 5365 043
Case 5365/04

SMA

Desmin


Leiomyosarcoma
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

Case 14986/04:

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





Fibromatosis
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

Case 28715/05:

9y old female patient, 13 cm tumour caecum.






Inflammatory myofibroblastic tumour
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

Case 3356/04:

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





Schwannoma
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

Case 7859/04:

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






Solitary fibrous tumour
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

Case 14005/03:

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






Dedifferentiated liposarcoma
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

Case 21561/07:

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






Case 21561 07 hmb45 sma
Case 21561/07HMB45 SMA


Angiomyolipoma
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

Case 28802/10:

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






Chondroid lipoma
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

Case 158/07:

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






Myofibroma
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

Case 4899/08:

10y old boy, 2cm nodule right groin.






Case 4899 085
Case 4899/08

EMA

Desmin


Angiomatoid fibrous histiocytoma
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

Case 34141/04:

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





Case 34141 044

Myogenin

MyoD1

Desmin

Case 34141/04


Pleomorphic rhabdomyosarcoma
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

Case 33584/03:

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






Alveolar soft part sarcoma
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

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.









Synovial sarcoma
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

Case 8960/04:

68y old female patient, recurrent tumour scalp.






Angiosarcoma
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

Case 7503/01:

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






Clear cell sarcoma
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

Case 24689/09:

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




Case 24689 093

CK

Case 24689/09

EMA



Epithelioid sarcoma
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 dataset soft tissue sarcomas
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



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