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Soft Tissue Tumors Part 3
Muscle, Vascular, Nerve, Other
Pleomorphic leiomyosarcoma
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 10 August 2009, last major update - August 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Leiomyosarcoma: general, cutaneous, epithelioid, myxoid, pleomorphic, rhabdoid features
Definition
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● Pleomorphic areas should be at least 2/3 of tumor
Epidemiology
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● 10% of all leiomyosarcomas
● Mean age 58 years, range 31-89 years
Clinical
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● Usually extremities, also retroperitoneum/abdominal cavity, chest/abdominal wall, occasionally scalp
● May be more aggressive than ordinary leiomyosarcoma, as 65% die of disease
Case reports
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● 63 year old man with tumor of adrenal gland (Archives 2003;127:e32)
● 65 year old man with beta-hcG producing retroperitoneal tumor (Sarcoma 2000;4:179)
Micro description
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● Focally fascicular with smooth muscle tumor cells
● Pleomorphic areas mimic MFH-pleomorphic
● Storiform pattern common
● Stromal hyalinization, chronic inflammatory infiltrate
● Usually high grade
● Occasionally rhabdoid features
Micro images
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Adrenal gland tumor
Positive stains
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● At least one smooth muscle marker (desmin, muscle-specific actin or alpha-smooth muscle actin) in the leiomyosarcomatous fascicular areas
Differential diagnosis
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● Atypical fibroxanthoma - superficial skin lesion with no necrosis, no vascular invasion, no infiltrative margins
● MFH-pleomorphic - negative for smooth muscle markers (i.e. no evidence of specific differentiation other than myofibroblasts / fibroblasts, Oncol Rep 1999;6:533)
● Myofibrosarcoma - low grade tumor overall, may have numerous thin walled capillaries, no prominent inflammation
References
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End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Pleomorphic leiomyosarcoma
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