Mast cell sarcoma mimicking metastatic colon carcinoma

Schwaab, Juliana; Horny, Hans Peter; Jonescheit, J.; Metzgeroth, Georgia; Schafhausen, Philippe; Gaiser, Timo; Marx, Alexander; Kienle, Peter; Hofmann, Wolf Karsten; Reiter, Andreas

doi:10.1007/s00277-013-1931-x

Cited by 14 publications

(8 citation statements)

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“…Thus, whereas patients with ISM have a normal or near normal life-expectancy, the prognosis (survival) is poor in advanced SM. With the exception of skin mastocytomas, localized MC neoplasms, including MC sarcomas (MCS), are extremely rare (38–41). …”

Section: Who Criteria and Classification Of Mastocytosis 2001–2016mentioning

confidence: 99%

Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future

et al. 2017

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Mastocytosis is a term used to denote a heterogeneous group of conditions defined by expansion and accumulation of clonal (neoplastic) tissue mast cells in various organs. The classification of the World Health Organization (WHO) divides the disease into cutaneous mastocytosis (CM), systemic mastocytosis (SM), and localized mast cell tumors. Based on histomorphologic criteria, clinical parameters, and organ involvement, SM is further divided into indolent SM (ISM) and advanced SM variants, including aggressive SM (ASM) and mast cell leukemia (MCL). The clinical impact and prognostic value of this classification has been confirmed in numerous studies, and its basic concept remains valid. However, refinements have recently been proposed by the consensus group, the WHO, and the European Competence Network on Mastocytosis (ECNM). In addition, new treatment options are available for patients with advanced SM, including allogeneic hematopoietic stem cell transplantation and multi-kinase inhibitors directed against KIT D816V and other key signalling molecules. Our current article provides an overview of recent advances in the field of mastocytosis, with emphasis on classification, prognostication, and emerging new treatment options in advanced SM.

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Section: Who Criteria and Classification Of Mastocytosis 2001–2016mentioning

confidence: 99%

Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future

et al. 2017

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“…2-CdA as a single agent does not appear to be efficacious against MCS. Of the 3 patients with MCS who received this treatment, none of them achieved remission [ 3 , 12 , 15 ]. Our preliminary experience confirms these reports with solo administration of Cladribine ® at a dose of 0.14 mg/kg/day for either 5 or 7 consecutive days [O. Hermine, personal communication].…”

Section: Management Of Mast Cell Burdenmentioning

confidence: 99%

Mast cell sarcoma: new cases and literature review

et al. 2016

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Mast cell sarcoma (MCS) is a rare form of mastocytosis characterized by the presence of solid tumor(s) comprising malignant mast cells that harbor destructive infiltration capability and metastatic potential. Here, we present an extensive literature review and report on 23 cases of MCS, including 3 new cases from the French National Reference Center for Mastocytosis. From our analysis, it appears that MCS can occur at any age. It can manifest de novo or, to a lesser extent, may evolve from a previously established mastocytosis. Bone tumor is a frequent manifestation, and symptoms of mast cell activation are rare. Histological diagnosis can be difficult because MCS is frequently composed of highly atypical neoplastic mast cells and can thus mimic other tumors. Unexpectedly, the canonical KIT D816V mutation is found in only 21% of MCS; therefore, complete KIT gene sequencing is required. The prognosis of patients with MCS is poor, with a median survival time of less than 18 months, and progression to mast cell leukemia is not unusual. Because conventional chemotherapies usually fail, the role of targeted therapies and bone marrow transplantation warrants further investigation in such aggressive neoplasms.

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“…28,[34][35][36][37] All MCS cases showed extensive local destructive growth, "metastases" and/or secondary systemic involvement, mostly in form of an MCL. [24][25][26][27][28][29][30][31][32][33][34][35][36][37][38] F I G U R E 2 Skin excision in 2014. A, Overview of the lesion of diffuse sheets of epithelioid cells in the dermis (H&E, magnification Â10).…”

Section: Discussionmentioning

confidence: 99%

“…[24][25][26][27][28][29][30][31][32][33] Additionally, there are five reported cases of systemic mastocytosis with sarcomatous growth of mast cells, all in adults and extracutaneous. 28,[34][35][36][37] All MCS cases showed extensive local destructive growth, "metastases" and/or secondary systemic involvement, mostly in form of an MCL. [24][25][26][27][28][29][30][31][32][33][34][35][36][37][38] F I G U R E 2 Skin excision in 2014.…”

Section: Discussionmentioning

confidence: 99%

“… 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 Additionally, there are five reported cases of systemic mastocytosis with sarcomatous growth of mast cells, all in adults and extracutaneous. 28 , 34 , 35 , 36 , 37 All MCS cases showed extensive local destructive growth, “metastases” and/or secondary systemic involvement, mostly in form of an MCL. 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 …”

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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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.

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Mast cell sarcoma mimicking metastatic colon carcinoma

Cited by 14 publications

References 1 publication

Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future

Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future

Mast cell sarcoma: new cases and literature review

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

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