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Soft tissue tumors
Perivascular epithelioid cell family of tumors (PEComas) of soft tissue
PEComa - falciform ligament / ligamentum teres
Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 9 March 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.
General
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Epidemiology
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- Usually females, median age 11 years, range 3-21 years
Case reports
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Gross description
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- Median 8 cm, range 5-20 cm
Micro description
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- Fascicular or nested groups of spindle cells (usually no epithelioid cells) with lightly eosinophilic, fibrillar cytoplasm with cytoplasmic clearing and small but distinct nucleoli in delicate capillary network similar to renal cell carcinoma
- Rare mitotic figures
- No necrosis, no angiolymphatic invasion
Positive stains
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- HMB45 (100%), MelanA (50%), microphthalmic transcription factor (50%), smooth muscle actin (50%), myosin (50%)
Negative stains
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Differential diagnosis
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- Angiomyolipoma: thick walled blood vessels, lipid distended tumor cells, spindled cells
- Cellular schwannoma: true capsule, thick-walled hyalinized blood vessels, strong S100 staining
- Clear cell sarcoma of tendons and aponeuroses: epithelioid and spindled areas with tumor giant cells, S100+, positive for melanocytic markers but negative for smooth muscle actin and myosin; t(12,22) present
- Leiomyoma: distinctly eosinophilic, cigar-shaped nuclei with blunt ends and perinuclear vacuoles; thick walled blood vessels
- Leiomyosarcoma: large deep seated mass with obvious nuclear pleomorphism and mitotic activity, often with necrosis; negative for HMB45, MelanA, microphthalmic transcription factor, positive for desmin
End of Soft tissue tumors > Perivascular epithelioid cell family of tumors (PEComas) of soft tissue > PEComa - falciform ligament / ligamentum teres
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