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
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:
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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:
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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:
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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 30750/03
MyoD1
Alveolar Rhabdomyosarcoma
Small round blue cell tumour
10-25 years; often extremities, all other sites
possible
Histology:
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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 7739/06
CK
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:
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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:
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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 17062/04
CD117 (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:
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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:
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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:
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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 28715/05
ALK
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 7859/04
CD34
Solitary Fibrous Tumour
Cellular SFT syn. to haemangiopericytoma
Middle-aged adults; extrapleural variant at any
site
Histology:
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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:
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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 21561/07
HMB45
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 158/07
SMA
Myofibroma
Solitary or multicentric (myofibromatosis)
All ages, commonly in infants & children; solitary: skin,
muscle, multiple: skin, viscera, muscle, bone
Histology:
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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:
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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
Case 34141/04
Myogenin
MyoD1
Desmin
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 33584/03
DPAS
Alveolar Soft Part Sarcoma
Rare soft tissue sarcoma
Adolescents and young adults; extremities
(adults) and head and neck (children); slow
growth
Histology:
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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 37608/03
EMA
Synovial Sarcoma
Relatively frequent soft tissue sarcoma
Young adults; deep-seated in extremities, but any site
possible
Histology:
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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 8960/04
CD31
Angiosarcoma
Mostly cutaneous, rarely soft tissue
Skin: lymphoedema, post-irradiation; adults; site
dep. on aetiology
Soft tissue: any age; any site; with syndromes
Histology:
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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 7503/01
S100
Clear Cell Sarcoma
Soft tissue sarcoma with melanocytic
differentiation
Young adults; deep-seated in extremities; slow
growth
Histology:
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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
Case 24689/09
CK
EMA
Case 24689/09
CD34
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
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!