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Case Reports
. 2021 Nov;48(11):1404-1409.
doi: 10.1111/cup.14088. Epub 2021 Jul 2.

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  1 , J Marja Oldhoff  2 , Arjan Diepstra  1 , Peter Valent  3   4 , Hans-Peter Horny  5 , Hanneke N G Oude Elberink  6 , Philip M Kluin  1 , Gilles F H Diercks  1
Affiliations
Case Reports

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 et al. J Cutan Pathol. 2021 Nov.

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 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.

Keywords: adult; case reports; mast-cell sarcoma; mastocytoma; mastocytosis.

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Figures

FIGURE 1
FIGURE 1
A, Clinical picture of the lesion in 2014, and B, the recurrent lesion in 2018
FIGURE 2
FIGURE 2
Skin excision in 2014. A, Overview of the lesion of diffuse sheets of epithelioid cells in the dermis (H&E, magnification ×10). B, Positive tryptase staining (magnification ×400). C, Details of the cells with some pleomorphism and scant mitotic figures (H&E, magnification ×400). D, Small clusters of mast cells near the resection margin (CD117, magnification ×200)
FIGURE 3
FIGURE 3
Skin excision of the recurrent lesion in 2018. A, Detail with progressive nuclear polymorphism and increasing mitotic rate (H&E, magnification ×400). B, Focus with a high Ki‐67 proliferation index (magnification ×200)
See this image and copyright information in PMC

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