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

Claire L W Wardle, J Marja Oldhoff, Arjan Diepstra, Peter Valent, Hans-Peter Horny, Hanneke N G Oude Elberink, Philip M Kluin, Gilles F H Diercks*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

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 two years, was re-excised after four years and did not recur >six year 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. This article is protected by copyright. All rights reserved.

Original languageEnglish
Pages (from-to)1404-1409
Number of pages6
JournalJournal of Cutaneous Pathology
Volume48
Issue number11
Early online date2-Jul-2021
DOIs
Publication statusPublished - Nov-2021

Keywords

  • adult
  • case reports
  • mast-cell sarcoma
  • mastocytoma
  • mastocytosis
  • SOLITARY MASTOCYTOMA
  • CLASSIFICATION
  • PATIENT

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