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Surgical Pathology > SOFT TISSUE > Flashcards

SOFT TISSUE Flashcards

1
Q

Features of calcifying aponeurotic fibroma (page 1814 Rosai)

A
  • Hands/wrists, child or adolescent.
  • Infiltrative lesion with spotty calcification.
  • Bland spindle to round cells, indistinct cytoplasm in dense fibrous stroma.
  • Cartwheel, linear or whorled pattern. Cells radiate out from calcification.
  • Scarce mitosis.
  • Can have MN giant cells.
  • IHC: SMA +/-, CD68 +/-, S100 +/-, CD99 +/-.
  • DD: Rheumatoid nodule
    Schwannoma
    Soft tissue chondroma
    Fibromatosis
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2
Q

Features of fibroma of Tendon Sheath (page 1815 Rosai)

A
  • Attached to tendon or tendon sheath, lobulated.
  • Hypocellular, ++ collagenous.
  • Spindle to stellate cells, +/- bizarre cells.
  • Dilated, slit like channels at the periphery
  • IHC: SMA +, Desmin -.
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3
Q

Features of Collagenous fibroma/ desmoplastic fibroblastoma (page 1815 Rosai)

A
  • Adults. In subcutis or intramuscular. Anywhere in the body.
  • Hypocellular. Stellate & spindled fibroblasts in collagenous matrix +/- myxoid areas.
  • IHC: SMA+, Desmin -.
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4
Q

Features of Nuchal type fibroma and Gardner fibroma (page 1816 Rosai)

A

NUCHAL TYPE FIBROMA:
- Posterior neck.
- Hypocellular bundles of thick collagen.
- Entrapped adipose tissue & traumatic neuroma-like structures.
GARDNER FIBROMA:
- Same as above but in back/paraspinal region and no entrapped nerves.
- First decade of life, associated with FAP/Gardner syndrome.
- IHC: CD34 +, CD99 +, B-catenin + (nuclear).

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5
Q

Features of Superficial Acral Fibromyxoma (page 1816 Rosai)

A
  • All ages.
  • Fingers and toes.
  • Spindle to stellate cells in fibromyxoid/myxoid stroma.
  • IHC: CD34+.
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6
Q

Features of Fibrous Hamartoma of Infancy (page 1817 Rosai)

A
  • First 2 decades of life, M>F.
  • Axilla, shoulder, upper arm.
  • Organoid pattern with 3 different tissues:
    #Spindle cells: Fibroblasts/myofibroblasts in a collagenous background.
    #Mature adipose tissue.
    #Inmature cells in a whirling pattern resembling primitive mesenchyme
  • IHC: SMA+ in spidle cell area, Desmin -.
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7
Q

Features of Angiolipoma (page 1850 Rosai)

A
  • After puberty, painful lesion.
  • Well circumscribed in subcutis, can be multiple.
  • Trunk and extremities.
  • Admixture of blood vessels and mature adipose tissue.
  • Hyaline thrombi important diagnostic sign.
  • Cellular angiolipoma confused with Kaposi sarcoma or angiosarcoma.
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8
Q

Features of Desmoid-type Fibromatosis (page 1823 Rosai)

A
  • Lesions are intimately associated with skeletal muscles and their aponeurosis. Common in abdominal wall in women during or following pregnancy but also in men & other locations: shoulder girdle, chest wall, H &N, mesentery, thigh.
  • Proliferation of well differentiated fibroblasts/myofibroblasts arranged in fascicles.
  • Abundant collagen b/w cells. +/- keloid-like collagen.
  • Infiltrative pattern of growth.
  • Lack of cytologic features of malignancy.
  • Thick walled +/- ectatic vessels sharply outlined from the surrounding tissue.
  • Low or absent mitotic activity.
  • Perivascular lymphocytic infiltrate at the advancing edge of the lesion.
  • Aggressive clinical behaviour: recurrences (no mets).
  • IHC: B-catenin +(nuclear), SMA+, Desmin + Calponin+/-
    CD34 -
    MOLECULAR: Activating mutations of CTNNB1 (encoding B-catenin), leading to nuclear expression of B-catenin. In cases assoc w Gardner sdme, there is a germline mutation of the APC gene (5q22). Same end result as both APC and B-catenin are components of the Wnt signaling pathway.
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9
Q

Features of DF/ Benign fibrous histiocytoma

A
  • Extremities most common.
  • Spindle cell lesion centred in the upper dermis, but it can involve deep dermis and even extend to subcutis.
  • Cellular fibrohistiocytic proliferation with collagen deposition. Histiocytes can be foamy or hemosiderin laden or they can acquire the features of Touton giant cells.
  • Cells arranged in fascicles, can have storiform pattern (less so than DFSP).
  • Fine vascular network.
  • Collagen entrapment at lateral edge of lesion.
  • Hyperplasia of overlying epidermis: +/- proliferation of hair germ like structures in the basal layer (follicular induction), rarely a BCC develops.
  • IHC: Factor XIIIa +, CD34 - (reverse for DFSP).
    Also SMA, Desmin, CD68 usually +.
  • Variants:
  • Haemorrhagic/aneurysmal: Large cystic spaces full of blood.
  • Prominent palisading (simil peripheral nerve).
  • Myxoid change.
  • Epithelioid cell DF (large, angulated cells).
  • Granular/Lipidized/clear/signet ring/balloon cells.
  • Marked focal atypia ‘Monster cells’.
  • Diffuse eosinophilic infiltrate.
  • Lichenoid, erosive, and ulcerated features.
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10
Q

Features of Dermal Nerve Sheath Myxoma

A
  • Distal extremities, particularly the fingers.
  • Multinodular lesion with a fibrous border, distinct fascicular or plexiform arrangement.
  • Reminiscent of myxoma but plumper, epithelial-like cells.
  • IHC: S100+
    (Different to neurothekeoma, S100-)
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11
Q

Features of kaposiform Haemangioendothelioma

A
  • Retroperitoneum or deep soft tissue of the extremities in children (50% during the 1st year of life).
  • Can be associated with consumption coagulopathy and thrombocytopenia. Also with lymphangiomatosis.
  • Micro:
  • Tumor nodules surrounded by a desmoplastic stroma. The nodules show areas that resemble capillary hemangioma, whereas other areas resemble Kaposi sarcoma.
  • Glomeruloid structures are a characteristic feature.
  • Thin-walled lymphatic vessels are often seen at the periphery of the tumour nodules.
  • IHC:
    CD31/CD34 + (in glomeruloid like areas), D2-40+ (in Kaposi-like areas)
    HHV8 -, GLUT 1 -.
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12
Q

Reactive vascular proliferations (4)

A
  • Papillary endothelial hyperplasia
  • Pyogenic granuloma/lobular capillary haemangioma
  • Bacillary Angiomatosis
  • Glomeruloid Haemangioma
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13
Q

Features of Papillary endothelial hyperplasia (PEH)

A
  • Can be a reactive proliferation as a result of trauma or superimposed on a pre-existing benign vascular lesion.
  • Micro:
  • Usually begins within a thrombosed blood vessel with ingrowth of endothelial cells into the thrombus, forming pseudopapilla. These are lined by a single layer of plump, normochromatic endothelial cells.
  • Endothelial proliferation may give the appearance of interanastomosing vascular channels, simulating angiosarcoma.
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14
Q

Features of Pyogenic granuloma (lobular capillary haemangioma)

A
  • Occur in mucosa (mouth) and skin. Rapid growth followed by stabilization +/- regression.
  • Micro:
  • Circumscribed, small polypoid lesion often surrounded by an epidermal collarette.
  • Lobular architecture with central larger vessels and peripheral aggregates of well formed capillaries.
  • Mixed acute and chronic inflammatory infiltrate.
  • Mitosis++
  • Note: Areas similar to PG can be found at the surface of any ulcerated lesion (eg SCC).
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15
Q

Features of Nodular fasciitis

A
  • Young adults. Rapidly growing mass trunk/UL/Neck. +/- Hx of trauma.
  • Deep dermis/ subcutis.
  • Well circumscribed (usually) & cellular.
  • ‘Tissue culture’ growth: fascicles of plump spindle cells in a loose, feathery collagenous stroma with areas of myxoid or microcystic change.
  • Mitosis++, RBC extravasation, lymphocytes.
  • IHC: SMA+
  • Molecular: t (17,22). MYH9- USP6 fusion ( FISH).
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16
Q

Spontaneously painful nodules of skin and soft tissues (5)

A 
Leiomyoma
Traumatic neuroma
Glomus tumour
Eccrine spiradenoma
Angiolipoma
17
Q

Features of Elastofibroma

A
  • Subscapular region. Elderly patients.
  • Hx of repetitive manual labour.
  • Thickened basophilic elastic fibers forming refractile cylinders amongst fibroblasts and collagen bundles (spaghetti & meatballs look).
  • Entrapped adipose tissue common.
  • IHC: Elastin stain +.
18
Q

Features of Myositis Ossificans

A
  • Young adults, extremities.
  • Hx of trauma (>50% cases).
  • Triphasic pattern/ zonation:
  • Center: Reactive fibroblasts (Nodular fasciits-like)
  • Intermediate: Osteoid
  • Periphery: Mature lamellar bone
19
Q

Features of Proliferative fasciits and Proliferative myositis

A
  • Adults, average 50 years old.
  • In subcutis, along fibrous septae (PF) or intramuscular (PM).
  • More ill defined than nodular fasciits.
  • Spindle cells & scattered ganglion-like cells (large vesicular nuclei, prominent nucleoli and eosinophilic cytoplasm).
  • IHC: ganglion-like cells are SMA -, Vimentin +.
20
Q

Features of superficial fibromatosis

A

Palmar Fibromatosis (Dupuytren’s): Only adults, bilateral in ETOH.
Plantar Fibromatosis: Children and adults. Often multinodular.
- Proliferative phase: Fascicles of bland spindle cells in a nodular pattern. +/- giant cells in plantar lesions, +/- mitosis.
- Involutional phase: Paucicellular, dense collagen.
- IHC: SMA +.

21
Q

Features of solitary fibrous tumour (SFT)

A
  • Wide age range. Slow growing painless mass.
  • Common in pleura, but any location.
  • Well circumscribed.
  • Monotonous ovoid to spindle cells in a “paternless pattern” (arranged haphazardly) associated with wire-like collagen.
  • Staghorn/HPC vessels.
  • Hyper/hypocellular areas.
  • Low mitosis (3/10 HPF).
  • IHC: CD34, STAT6, CD99, BCL2 +.
22
Q

Features of inflammatory myofibroblastic tumour (IMT)

A
  • Children and adults, any site but common in lung, mesentery and retroperitoneum.
  • Spindle cells in variable patterns, admixed with Lo and plasma cells.
  • Myxoid or collagenous background, hyper/hypocellular areas.
  • Mitosis common.
  • IHC: SMA +, ALK + (50% cases).
23
Q

Features of Dermatofibrosarcoma protuberans (DFSP)

A
  • Young adults and children.
  • Trunk and proximal extremities.
  • Diffuse, deep dermis to subcutis.
  • Uniform, bland spindle cells in a storiform pattern.
  • Tumour cells infiltrate around adnexa and into subcutis in a ‘lace-like’ pattern (‘honeycomb’ appearance of fat).
  • Few mitosis.
  • IHC: CD34+.
  • Molecular: fusion COL1A1 & PDGFB.
24
Q

Features of adult fibrosarcoma

A
  • Rare malignant tumour of fibroblasts with a herringbone architecture (long fascicles intersecting at acute angles) and variable collagen. Mitosis present.
  • No pleomorphism, no giant cells.
  • Diagnosis of exclusion.
  • DD: Fibromatosis, SS, MPNST, UPS.
  • IHC: SMA +.
25
Q

Features of low grade fibromyxoid sarcoma (LGFMS)

A
  • Young, middle aged adults.
  • Thigh > trunk.
  • Low to moderate cellularity.
  • Deceptively bland spindle cells deposited in collagenous and myxoid background (fibroid > myxoid).
  • Prominent vessels with perivascular hypercellularity.
  • +/- prominent rosettes, whorled pattern.
  • IHC: MUC4+, EMA+.
  • Molecular: t(7,16) with FUS-CREB3L2 fusion (95%) or FUS-CREB3L1 (5%).
26
Q

Features of Myxofibrosarcoma (low grade)

A

(myxoid subtype of UPS, formerly myxoid MFH)

  • Elderly. Extremities.
  • Dermis/subcutis or deeper.
  • Multinodular tumour composed of pleomorphic spindle cells in a myxoid (>than fibroid) background.
  • Curvilinear vessels with condensation of cells around vessels.
  • Infiltrative, sends out long tentacles.
  • ‘Pseudolipoblasts’ common.
  • Higher grade: More cellular, atypical mitosis, necrosis or haemorrhage.
  • IHC: Nil specific. Vimentin, CD34 +/-.
  • Molecular: complex karyotypes.
27
Q

Features of Sclerosing Epithelioid Fibrosarcoma

A

(Variant of fibrosarcoma)

  • Small round/ovoid cells with scanty clear cytoplasm embedded in densely hialinized stroma.
  • Often single file pattern (DD with Carcinoma).
  • Like LGFMS: MUC4+ and can have FUS translocation.
28
Q

Features of Tenosynovial Giant Cell Tumour, Localized type

A
  • Adults, 40s to 60s. F>M.
  • Distal extremities (hands > toes).
  • Lobulated with capsule.
  • Small and large mononuclear cells, MNG cells, xanthoma cells, inflammatory cells & haemosiderin in a collagenous background.
  • Cleft like spaces at periphery.
  • IHC: CD45, CD68, CD123 +.
  • Molecular: Re-arrangement of CSF1 gene.
29
Q

Features of Giant Cell Tumour of Tendon Sheath

A

(same as PVNS: Pigmented Villonodular Synovitis)

  • Intra-articular location.
  • Less common than localized. Age: 50s.
  • Knee > ankle > foot.
  • Similar hstology as localized type but infiltrates surrounding tissues.
30
Q

Features of Giant Cell Tumour of the Soft Tissues

A
  • All ages, hand or arm.
  • Multiple nodules infiltrating surrounding soft tissues.
  • Bland mononuclear cells, MNG cells (osteoclasts) & short spindle cells.
  • Mitosis =/-.
  • Similar histo but different entity to GCT of bone.
31
Q

Features of Hibernoma

A
  • Interscapular area, axilla and thigh. Also mediastinum and retroperitoneum.
  • Gross: brown cut surface.
  • Organoid arrangement of large cells with centrally located nuclei and cytoplasm with multiple small vacuoles.
  • Can be hybrid with lipoma.
32
Q

Features of Lipoblastoma

A
  • Infants/children < 5yo.
  • Proximal extremities.
  • ‘Lipoblastoma’ if well circumscribed, ‘lipoblastomatosis’ if ill defined.
  • Lobulated, mature adipocytes and lipoblasts in myxoid stroma.
  • Plexiform vascular pattern.
  • Molecular: Re-arrangement of PLAG-1 (pleomorphic adenoma gene).
    (In contrast: myxoid liposarcoma has DDT3 gene translocation).
33
Q

Features of Lipoma

A
  • Usually upper half of body.
  • 5th-6th decades of life, most are subcutaneous (unlike liposarcomas).
  • Multiple lipomas: seen in NF or MEN syndrome.
  • Usually encapsulated, except in deep locations.
  • Mature adipose tissue, no cellular atypia.
  • +/- fat necrosis, infarct, calcification or metaplastic cartilage or bone.
  • Pitfall: histiocytes associated with fat necrosis resemble lipoblasts.
  • IHC: S100+, calretinin +.

Surgical Pathology (12 decks)

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