Soft Tissue Tumors - Malignant fibrous histiocytoma of soft tissue

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Soft Tissue Tumors

Fibrohistiocytic tumors

Malignant fibrous histiocytoma of soft tissue - giant cell type

Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 28 October 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Diagnosis of exclusion - an undifferentiated pleomorphic sarcoma with prominent osteoclast-like giant cells

Terminology
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● Also called malignant giant cell tumor of soft parts, malignant osteoclastoma, giant cell sarcoma

Epidemiology
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● 3-15% of malignant fibrous histiocytomas
● Number of cases is declining as specific sarcoma types are identified
● Rare in children

Clinical features
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● Extremities and trunk
● Osteoclast-like cells have functional features of osteoclasts (J Pathol 1989;159:53)

Case reports
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● 5 year old boy with retroperitoneal tumor (Turk J Pediatr 2007;49:307)
● 53 year old male with laryngeal tumor (Rom J Morphol Embryol 2010;51:359)
● 59 year old male with vocal cord lesion on the vocal cord (Rom J Morphol Embryol 2009;50:481)
● Disseminated tumor (Indian J Pathol Microbiol 2007;50:795)

Gross description
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● Hemorrhagic and necrotic

Gross images
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Left atrial tumor at autopsy

Micro description
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● Resembles pleomorphic malignant fibrous histiocytoma, with addition of evenly dispersed osteoclast-like giant cells with 20-100 uniform nuclei that are small and round/oval, accompanied by smaller stromal cells with similar nuclei
● 2-3 MF/10 HPF
● May have angiolymphatic invasion
● Rarely small foci of neoplastic bone or cartilage (some designate these tumors as osteosarcoma or chondrosarcoma)
● No xanthoma cells or siderophages, no necrosis

Micro images
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Osteoclast-like giant cells with uniform nuclei in a pleomorphic MFH

Tumor cells may form neoplastic bone and osteoid

Similar lesion in prostate

Left atrial tumor at autopsy (H&E and vimentin)

Cytology description
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● Hypercellular smear with cohesive, branching clusters of spindle cells with ovoid, focally hyperchromatic nuclei and inconspicuous nucleoli
● Also interspersed osteoclast-like giant cells, some associated with clusters of spindle cells
● Hemorrhagic background with cellular debris and occasional spindle cells and lymphocytes (Acta Cytol 2003;47:673)

Cytology images
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Multinucleated osteoclastic-type giant cells

Positive stains
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● Vimentin, CD68, S100 (variable) , SMA (variable)

Differential diagnosis
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● Extraskeletal osteosarcoma: prominent malignant osteoid
● Giant cell tumor of soft tissue: no pleomorphic MFH features
● Leiomyosarcoma: smooth muscle morphology and immunostains; no prominent osteoclastic giant cells
● Melanoma: giant cells, if present, are typically not osteoclast-like; positive for melanocytic markers
● Osteoclast-rich carcinoma: malignant epithelial component
● MFH-pleomorphic: no osteoclast-like cells with uniform nuclei

End of Soft Tissue Tumors > Fibrohistiocytic tumors > Malignant fibrous histiocytoma of soft tissue - giant cell type

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