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Increased risk of malignant melanoma in patients with systemic mastocytosis?
Department of Hematology, Karolinska University Hospital Huddinge.
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2014 (English)In: Acta Dermato-Venereologica, ISSN 0001-5555, E-ISSN 1651-2057, Vol. 94, no 5, 583-584 p.Article in journal (Refereed) Published
Abstract [en]

Mastocytosis, a group of rare disorders that occur in bothchildren and adults, is characterised by abnormal growthand pathological accumulation of mast cells in one or moreorgans, most commonly the skin (1). Urticaria pigmentosa(UP) is the most common cutaneous variant. In cases ofextracutaneous involvement, systemic mastocytosis (SM)can be diagnosed on the basis of the criteria formulatedby the WHO. The course of SM in most patients (90%) isindolent, with more aggressive presentation in only a few.The incidence of cutaneous melanoma is increasingand although this malignancy and mastocytosis originatefrom 2 different types of cells (melanocytes from theneural crest and mast cells from haematopoetic stem cells,respectively) they share certain similarities, includingexpression of the transcription factors MITF and STAT3,and dependence of the growth factor receptor KIT and itsligand stem cell factor for their growth and development(2, 3). We have found 5 published case reports that suggesta relationship between these 2 pathologies. In the first,published in 1979, a patient with nodular mastocytosis de-veloped both melanocytoma and mastocytoma (4). In thesecond, UP and SM preceded a metastatic melanoma (5)and the third involved combined mastocytoma-junctionalnaevus (6). In the fourth case, malignant melanoma wasdiagnosed prior to SM (7). And finally, a patient withtelangiectasia macularis eruptive perstans (TEMP), arare form of cutaneous mastocytosis, was found to havea malignant melanoma (8).Here, we describe our 4 additional cases and discusspossible associations between these 2 diseases.

Place, publisher, year, edition, pages
2014. Vol. 94, no 5, 583-584 p.
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Hematology
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URN: urn:nbn:se:uu:diva-270839DOI: 10.2340/00015555-1788PubMedID: 24473924OAI: oai:DiVA.org:uu-270839DiVA: diva2:890653
Available from: 2016-01-04 Created: 2016-01-04 Last updated: 2017-12-01Bibliographically approved

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