Cardiac tumors - malignant
Malignant fibrous histiocytoma

Author: Nat Pernick, M.D. (see Authors page)

Revised: 20 March 2018, last major update November 2013

Copyright: (c) 2007-2018, PathologyOutlines.com, Inc.

PubMed Search: Malignant fibrous histiocytoma [title] heart

Related Topics: Myxoid MFH of heart

Cite this page: Pernick, N. Malignant fibrous histiocytoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/hearttumormfh.html. Accessed August 16th, 2018.
Definition / general
  • See also undifferentiated sarcoma (some authors consider them the same entity)
  • Second most common cardiac sarcoma after angiosarcoma but still rare
  • Mean age 44 years, rare in children
  • 90% occur in left atrium, often on posterior wall
  • Poor prognosis; metastasizes to bone, kidney, lung, lymph nodes, serosal surfaces, skin, thyroid
Case reports
  • Excision, possibly chemotherapy or radiation therapy
Gross description
  • Lobulated polypoid mass, often multiple
  • Sessile or pedunculated
  • Soft or creamy texture
  • Up to 10 cm
Gross images

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Fig A: lobulated tumor in right ventricular outflow tract

Microscopic (histologic) description
  • Usually pleomorphic storiform or myxoid subtypes
  • Atypical fibroblasts, pleomorphic histiocytoid cells and giant cells with esosinophilic cytoplasm and fibromyxoid stroma
  • Giant cell MFH: numerous osteoclast-like giant cells; also called osteoclastoma
Microscopic (histologic) images

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Storiform pattern of fibrohistiocytic cells

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Prominent vessels and myxoid stroma

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Spindled fibroblasts and mitotic figures

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Fibrohistiocytic tumor
with giant cells (arrow)
and spindle cells (circle)
Positive stains