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Soft Tissue Tumors Part 3
Muscle, Vascular, Nerve, Other
Leiomyosarcoma with Rhabdoid features
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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● Diffuse proliferation of rhabdoid cells in otherwise identifiable leiomyosarcoma
● Tumors with scattered rhabdoid cells may be better classified as pleomorphic leiomyosarcoma
● Distinct from rhabdoid tumor of kidney
Epidemiology
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● Often infancy or childhood, in neck or paraspinal region (Pathol Int 2006;56:287)
Case reports
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● 56 year old woman with inguinal mass with epithelioid and rhabdoid features (Wien Klin Wochenschr 2007;119:557).
Treatment and prognosis
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● Presence of rhabdoid cells in a tumor, in general, is associated with aggressive behavior
● Poorer prognosis for external soft tissue (not intra-abdominal) cases, but not for retroperitoneal cases (Mod Path 2000;13:1211)
Gross description
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● 3-22 cm
Micro description
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● Rhabdoid cells are large and polygonal with eosinophilic cytoplasm, eosinophilic globular perinuclear inclusions, eccentric nuclei and
prominent nucleoli
Micro images
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Various images Immunostains
Positive stains
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● Rhabdoid cells - vimentin, desmin, low molecular weight cytokeratin in inclusion bodies, muscle actin in cytoplasm, INI1 in nucleus (Mod Pathol 2005;18:951)
Electron microscopy
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● Paranuclear aggregates or whorls of intermediate filaments
Molecular
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● Malignant rhabdoid tumors of various sites may have abnormalities in the long arm of chromosome 22 and alteration of the hSNF5/INI1 (SMARCB1) gene (Am J Surg Pathol 1999;23:249, Am J Hum Genet 1999;65:1342), also seen in epithelioid sarcoma (Am J Dermatopathol. 2009;31:152)
Molecular images
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FISH-not necessarily from leiomyosarcoma
Differential diagnosis
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● Rhabdoid cells may be present in synovial sarcoma, extraskeletal myxoid chondrosarcoma and leiomyosarcoma
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Leiomyosarcoma with rhabdoid features
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