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Pleomorphic hyalinizing angiectatic tumor revisited: all tumors manifest typical morphologic features of myxoinflammatory fibroblastic sarcoma, further suggesting 2 morphologic variants of a single entity

M. Michal, DV. Kazakov, L. Hadravský, A. Agaimy, M. Švajdler, N. Kuroda, M. Michal,

. 2016 ; 20 (-) : 40-3. [pub] 20151029

Language English Country United States

Document type Journal Article

Persistent link   https://www.medvik.cz/link/bmc17000827

We describe 9 cases of pleomorphic hyalinizing angiectatic tumor (PHAT). Recently described TGFBR3 and MGEA5 gene rearrangements in these tumors have confirmed the long-hypothesized link between PHAT and another soft tissue entity, the myxoinflammatory fibroblastic sarcoma (MIFS). Myxoinflammatory fibroblastic sarcoma and PHAT share the same translocation and in addition have a very similar clinical presentation. However, to our best knowledge, no study has ever addressed the striking morphologic similarities between MIFS and PHAT. Our findings based on histological criteria suggest that most, if not all, tumors diagnosed as PHAT might, in fact, represent examples of MIFS that, in addition to a conventional MIFS morphology, manifest aberrant angiectatic hyalinized vessels.

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$a We describe 9 cases of pleomorphic hyalinizing angiectatic tumor (PHAT). Recently described TGFBR3 and MGEA5 gene rearrangements in these tumors have confirmed the long-hypothesized link between PHAT and another soft tissue entity, the myxoinflammatory fibroblastic sarcoma (MIFS). Myxoinflammatory fibroblastic sarcoma and PHAT share the same translocation and in addition have a very similar clinical presentation. However, to our best knowledge, no study has ever addressed the striking morphologic similarities between MIFS and PHAT. Our findings based on histological criteria suggest that most, if not all, tumors diagnosed as PHAT might, in fact, represent examples of MIFS that, in addition to a conventional MIFS morphology, manifest aberrant angiectatic hyalinized vessels.
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$a Kazakov, Dmitry V $u Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic.
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$a Švajdler, Marián $u Department of Pathology, Charles University, Medical Faculty and Charles University Hospital Plzen, Czech Republic; Department of Pathology, Louis Pasteur University Hospital, Kosice, Slovakia.
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$a Kuroda, Naoto $u Red Cross Hospital Kochi, Kochi, Japan.
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