Soft Tissue Tumors - Pleomorphic hyalinizing angiectatic tumor

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Soft Tissue Tumors

Fibrohistiocytic tumors

Pleomorphic hyalinizing angiectatic tumor

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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● Rare tumor (< 50 cases reported) of subcutis, with ectatic blood filled vessels surrounded by hyalinized stroma and pleomorphic spindle cells, no mitotic activity (Stanford University)

Terminology
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● Part of WHO classification for skin tumors and soft tissue tumors
● First described in 1996 (Am J Surg Pathol 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 or primitive undifferentiated mesenchyme

Case reports
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● 55 year old male with a left forearm mass (UPOJ 2010;20:121))
● 76 year old woman with sarcomatous component (Pathol Int 2007;57:281)
● 77 year old woman (West Indian Med J 2007;56:544)
● 83 year old woman with thigh tumor (Pathol Int 2002;52:664)

Treatment and prognosis
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● Wide local excision, but 20% recur, rarely 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
● Hemosiderotic fibrohistiocytic lipomatous lesion/tumor may be precursor lesion (Am J Surg Pathol 2004;28:1417, but see Orthopedics 2010;33:516)
● No/rare mitotic figures

Micro images
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Various images

Virtual slides
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Pleomorphic hyalinizing angiectatic tumor

Cytology description
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● Both bland and larger pleomorphic cells, hyalinized vessels (Diagn Cytopathol 2011;39:214)

Positive stains
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● CD34 (strong, Arch Pathol Lab Med 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)

Electron microscopy images
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Cytoplasmic granular deposits consistent with hemosiderin and wavy intermediate filaments consistent with vimentin

Molecular description
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● Case report of 2 unbalanced translocations involving chromosomes 1 and 3 and chromosomes 1 and 10, with a karyotype of 45,XX,der(1)t(1;3)(p31;q12),-3,der(10)t(1;10)(p31;q25)[11]/46,XX[4 (Hum Pathol 2012;43:121)

Molecular / cytogenetic images
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Unbalanced translocations involving chromosomes 1 and 3 and chromosomes 1 and 10

Differential diagnosis
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● Schwannoma: not infiltrative, no atypia, strongly S100+
● Primary cutaneous myxofibrosarcoma: see Am J Dermatopathol 2005;27:322

End of Soft Tissue Tumors > Fibrohistiocytic tumors > Pleomorphic hyalinizing angiectatic tumor

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