Case report of a clinically indolent but morphologically high-grade cutaneous mast cell tumor in an adult: Atypical cutaneous mastocytoma or mast cell sarcoma?

Autor: Wardle CLW; Department of Pathology and Medical Biology, University Medical Centre Groningen, Groningen, The Netherlands., Oldhoff JM; Department of Dermatology, University Medical Centre Groningen, Groningen, The Netherlands., Diepstra A; Department of Pathology and Medical Biology, University Medical Centre Groningen, Groningen, The Netherlands., Valent P; Ludwig Boltzmann Institute for Hematology and Oncology, Medical University of Vienna, Vienna, Austria.; Department of Internal Medicine I, Division of Hematology and Hemostaseology, Medical University of Vienna, Vienna, Austria., Horny HP; Institute for Pathology, Ludwig-Maximilians University, Munich, Germany., Oude Elberink HNG; Department of Allergy and Immunology, University Medical Centre Groningen, Groningen, The Netherlands., Kluin PM; Department of Pathology and Medical Biology, University Medical Centre Groningen, Groningen, The Netherlands., Diercks GFH; Department of Pathology and Medical Biology, University Medical Centre Groningen, Groningen, The Netherlands.
Jazyk: angličtina
Zdroj: Journal of cutaneous pathology [J Cutan Pathol] 2021 Nov; Vol. 48 (11), pp. 1404-1409. Date of Electronic Publication: 2021 Jul 02.
DOI: 10.1111/cup.14088
Abstrakt: We present a case of an adult male with a solitary mast cell tumor of the skin with unusual nuclear pleomorphism and mitotic activity. The tumor was excised, recurred within 2 years, was reexcised after 4 years and did not recur >6 years after diagnosis. The tumor showed progressive cytonuclear atypia and a high mitotic and proliferation rate by Ki67-staining from the onset. No KIT mutations were identified in the tumor and bone marrow. Serum tryptase levels and a bone marrow aspirate and trephine biopsy were normal. Although the histomorphology of the skin tumor was consistent with mast cell sarcoma, the clinical behavior without systemic progression argued against this diagnosis. The tumor was finally considered as atypical mastocytoma, borderline to mast cell sarcoma. Currently, the patient is in close follow-up and still in complete remission.
(© 2021 The Authors. Journal of Cutaneous Pathology published by John Wiley & Sons Ltd.)
Databáze: MEDLINE
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