Definition
Pleomorphic hyalinizing angiectatic tumor (PHAT) is a locally aggressive soft tissue neoplasm composed of spindle to pleomorphic cells and ectatic blood vessels, surrounded by fibrin and collagen. A possible relationship with hemosiderotic fibrolipomatous tumor (HFLT) and with myxoinflammatory fibroblastic sarcoma has been suggested, although the exact relationship between these three entities has still to be determined (Dei Tos 2019).
Clinical Features
Incidence
It is a very rare tumor.
Age
It generally occurs in middle-aged patients.
Sex
Men and women are equally affected.
Site
It typically occurs in the subcutis of the lower extremity, especially in the ankle and foot; rarely it can occur in deep soft tissues (Weiss and Dei Tos 2013).
Treatment
Surgical excision is the treatment of choice; recurrences are usually controlled by re-excision.
Outcome
Local recurrences can occur in 30–50% of cases. Rare cases recurred as myxoid pleomorphic sarcoma have been reported.
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References and Further Reading
Antonescu, C. R., Zhang, L., Nielsen, G. P., Rosenberg, A. E., Dal Cin, P., & Fletcher, C. D. (2011). Consistent t(1;10) with rearrangements of TGFBR3 and MGEA5 in both myxoinflammatory fibroblastic sarcoma and hemosiderotic fibrolipomatous tumor. Genes, Chromosomes & Cancer, 50(10), 757–764.
Dei Tos, A. P. (2019). Soft tissue sarcomas. A pattern-based approach to diagnosis. Cambridge: Cambridge University Press.
Smith, M. E., Fisher, C., & Weiss, S. W. (1996). Pleomorphic hyalinizing angiectatic tumor of soft parts. A low-grade neoplasm resembling neurilemoma. The American Journal of Surgical Pathology, 20(1), 21–29.
Wei, S., Pan, Z., Siegal, G. P., Winokur, T. S., Carroll, A. J., & Jhala, D. (2012). Complex analysis of a recurrent pleomorphic hyalinizing angiectatic tumor of soft parts. Human Pathology, 43(1), 121–126.
Weiss, S. W., & Dei Tos, A. P. (2013). Pleomorphic hyalinizing angiectatic tumor of soft parts. In C. D. M. Fletcher, J. A. Bridge, P. C. W. Hogendoorn, & F. Mertens (Eds.), World Health Organization classification of tumours. Pathology and genetics of tumours of soft tissue and bone (p. 200). Lyon: IARC.
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Gambarotti, M. (2020). Pleomorphic Hyalinizing Angiectatic Tumor. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5453-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5453-1
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