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Skin / Soft Tissue Tumors
Pleomorphic hyalinizing angiectatic tumor
Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.
Reviewer: David Lucas, M.D., University of Michigan Health Systems (January 2009)
Revised: 22 October 2009, last major update June 2009
Definition
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● Rare tumor (< 50 cases reported) of subcutis, with ectatic blood filled vessels surrounded by hyalinized stroma and pleomorphic spindle cells
Terminology
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● Part of WHO classification for skin tumors and soft tissue tumors
● First described in 1996 (AJSP 1996;20:21)
Epidemiology
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● Low grade malignancy of adults (median age 51 years), often in extremities or trunk
● May be derived from stromal fibroblasts
Case reports
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● 77 year old woman (West Indian Med J 2007;56:544)
● 83 year old woman with thigh tumor (Path Int 2002;52:664)
● Tumor with sarcomatous component (Pathol Int 2007;57:281)
Treatment and prognosis
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● Wide local excision
● 20% recur; may recur as sarcoma
Micro description
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● Unencapsulated with infiltrative margins
● Atypical spindle cells that contain hemosiderin and resemble MFH-pleomorphic and schwannoma, have intranuclear and cytoplasmic inclusions
● Also large ectatic spaces with blood and fibrin and perivascular hyalinization
● Abundant mast cells, occasional fat
● May have monomorphic partially myxoid spindle cell component as precursor lesion (hemosiderotic fibrohistiocytic lipomatous lesion, see Hemosiderotic fibrohistiocytic lipomatous lesion of soft tissue, AJSP 2004;28:1417)
● No/rare mitotic figures
Micro images
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Various images Various images on page
Virtual slide (USCAP):
tumor with sarcomatous areas
Positive stains
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● CD34 (strong, Archives 2000;124:423), vimentin, VEGF, CD99, Factor XIIIa (focal)
Negative stains
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● S100, CD31; Ki-67 < 2% (Chin Med J (Engl) 2007;120:876)
Differential diagnosis
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● Schwannoma (not infiltrative, strongly S100+)
Additional references
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End of Skin / Soft Tissue Tumors > Pleomorphic hyalinizing angiectatic tumor
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