Soft tissue tumors - Leiomyosarcoma with Rhabdoid features

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Soft tissue tumors

Smooth muscle

Leiomyosarcoma with Rhabdoid features

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 23 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

Leiomyosarcoma: see general, cutaneous, epithelioid, myxoid, pleomorphic

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); also adults

Case reports
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● 38 year old woman with leiomyosarcoma of broad ligament with osteoclast-like giant cells and rhabdoid cells (Int J Gynecol Pathol 2010;29:432)
● 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 Pathol 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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Thigh #1

Thigh #2

Thigh #3

Retroperitoneum

Thigh-stains

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 description
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● Paranuclear aggregates or whorls of intermediate filaments

Molecular description
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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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Normal 22q dosages

Differential diagnosis
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● Rhabdoid cells may also be present in synovial sarcoma and extraskeletal myxoid chondrosarcoma

End of Soft tissue tumors > Smooth muscle > Leiomyosarcoma with Rhabdoid features

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