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9 January 2014 - Case of the Week #296

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Case of the Week #296

Clinical History:
A 28 year old woman presented with a tender, firm nodule on her right big toe measuring 2 x 2 cm, with normal colored overlying skin. An Xray showed no bony changes of the big toe. An excisional biopsy was performed, and received as two irregular grayish pieces of tissue.

Micro images:

What is your diagnosis?

Acral myxoinflammatory fibroblastic sarcoma (myxoinflammatory fibroblastic sarcoma of soft tissue)

The excisional biopsy showed a multinodular tumor with dense and loose myxoid areas, hyaline stroma and chronic inflammatory cells. Pleomorphic epithelioid cells with irregular smudgy and vesicular nuclei were also seen (images 10-13, 15). Focal necrosis was noted (image 14).

Acral myxoinflammatory fibroblastic sarcoma is an uncommon low grade sarcoma of the hands and feet, first described in 1998 by Montgomery et al and Meis-Kindblom and Kindblom. It is composed of myxoid stroma, inflammation, and virocyte like cells. It is also called inflammatory myxohyaline tumor of the distal extremities with virocyte or Reed-Sternberg-like cells. It affects men and women of all ages with a slow growing mass, usually in the hands or feet (J Cutan Pathol 2008;35:192).

Grossly, it is usually small, multinodular and poorly defined, often involving the joints and tendons. The cut surface is white and gelatinous with fibrous and myxoid zones. The distinctive histologic feature is myxoid extracellular matrix with various types of tumor cells:
• Epithelioid or spindled: occasionally large with bizarre nuclei and prominent nucleoli resembling viral inclusions or Reed-Sternberg cells
• Pseudolipoblasts: multivacuolated cells resembling pleomorphic lipoblasts due to cytoplasmic vacuoles containing myxoid material
• Giant cells with emperipolesis: minimal mitotic activity (0-1 MF/10 HPF), no atypical forms, no/rare necrosis.
In addition, there are acute or chronic inflammatory cells and areas of fibrosis or hyalinized stroma. Recently a consistent translocation - t(1;10) with rearrangements of TGFBR3 and MGEA5 was found in both myxoinflammatory fibroblastic sarcoma and hemosiderotic fibrolipomatous tumor, suggesting a shared pathogenetic relationship (Genes Chromosomes Cancer 2011;50:757).

The differential diagnosis includes:
Epithelioid sarcoma: most cells are round with dense eosinophilic cytoplasm, strongly keratin+, loss of INI1 (SMARCB)
Hodgkin lymphoma: lacks giant cells and lipoblast-like cells, tumor cells have different staining patterns (CD15+ and CD30+ in classic Hodgkin lymphoma)
Myxofibrosarcoma: (formerly called myxoid MFH); rare in soft tissues of hands and feet, more frequent mitotic figures with atypical forms, no inflammatory infiltrate
Pleomorphic liposarcoma: true pleomorphic lipoblasts, rare in soft tissues of hands and feet, more frequent mitotic figures with atypical forms, no inflammatory infiltrate
Rosai-Dorfman disease: emperipolesis, but no intranuclear viral-like inclusions, S100+, usually not myxoid
• Tenosynovitis: no enlarged atypical cells

Treatment is wide local excision with or without radiation therapy. Acral myxoinflammatory fibroblastic sarcoma frequently recurs locally, but distant metastases are rare (Am J Surg Pathol 2014;38:1).

Nat Pernick, M.D., President
and Palak Thakore, Associate Medical Editor
PathologyOutlines.com, Inc.
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