Mastcellsarcoma: new cases and literature review Mastcellsarcoma (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
Pediatric mastcellsarcoma of temporal bone with novel L799F (2395 C>T) KIT mutation, mimicking histiocytic neoplasm. Mastcellsarcoma (MCS) is an extremely rare neoplasm with a clinically aggressive course. Because of its rarity, its morphologic and molecular characteristics are still not well defined. We report a case of a 15-year-old girl with MCS of the temporal bone extending
Malignant transformation of mastocytoma developed on skin mastocytosis into cutaneous mastcellsarcoma. Mastocytosis is a group of disorders characterized by abnormal mast cell proliferation, involving the skin in 80% of cases. Cutaneous mastocytosis, which appears in childhood in 60% of cases, usually has a benign course with a gradually regressive evolution before puberty. Mastcellsarcomas , part of the systemic forms of mastocytosis, are very rare tumors characterized by a destructive growth of highly atypical mast cells, with secondary spread, poor prognosis, and low survival rates. We report the first known case of primary cutaneous mastcellsarcoma due to the transformation of a benign solitary mastocytoma in an adult suffering from an unregressive localized cutaneous mastocytosis
basically be divided into cutaneous mastocytosis (CM), systemic mastocytosis (SM) and mastcellsarcoma, which is a more aggressive and rarer form. Systemic mastocytosis is usually associated with somatic mutations of the KIT gene, one of which is the KIT D816V mutation. The signs and symptoms of systemic mastocytosis are known for their potential to be diffuse, which makes it more difficult to understand
NCCN Guidelines® Insights: Systemic Mastocytosis, Version 3.2024. Mastocytosis is a heterogeneous group of disorders comprising cutaneous mastocytosis, systemic mastocytosis, and mastcellsarcoma. It is associated with a variety of symptoms related to the release of mast cell mediators and mast cell tissue infiltration. Referral to specialized centers with expertise in the management
Comprehensive mastocytosis data analysis from a single center. Mastocytosis is a very rare disorder and is divided into three prognostically distinct variants by World Health Organization: Cutaneous mastocytosis (CM), systemic mastocytosis (SM), and mastcellsarcoma or localized mast cell (MC) tumors. The wide range of complaints may cause patients to consult various clinics, with resulting mis
systemic mastocytosis. J Clin Oncol. 2014 Oct 1032(29):3264-74. doi: 10.1200/JCO.2014.55.2018. Epub 2014 Aug 25.Ryan RJ, Akin C, Castells M, et al; Mastcellsarcoma: a rare and potentially under-recognized diagnostic entity with specific therapeutic implications. Mod Pathol. 2013 Apr26(4):533-43. doi: 10.1038/modpathol.2012.199. Epub 2012 Nov 30.Heide R, Beishuizen A, De Groot H, et al; Mastocytosis cellsarcoma, surgical excision with consecutive radiation and/or high-dose chemotherapy has been used[33]. More latterly, stem cell therapy has been used[29].ChildrenGuidelines for management in children are available[34, 35].Prognosis[2, 14]Cutaneous mastocytosisChildhood cases of urticaria pigmentosa and mastocytoma often resolve spontaneously. Adults are more likely to develop the systemic form
. Sometimes, extra-medullary organ invasion shows a metastasis-like or even sarcoma-like destructive growth of neoplastic cells in local tissue sites. Examples are myeloid sarcoma, mastcellsarcoma and localized blast phase of chronic myeloid leukemia. So far, little is known about mechanisms underlying re-distribution and extramedullary dissemination of LSC in myeloid neoplasms. In this article, we
mastocytosis (ASM), mast cell leukemia (MCL), and/or mastcellsarcoma (MCS).There is increasing evidence of CD30 expression in neoplastic MCs of the bone marrow. This expression has been described almost exclusively in aggressive forms of systemic mastocytosis (SM).The aim of the present study is to evaluate CD30 expression both in cutaneous and systemic forms of mastocytosis. Forty-two mastocytosis cases
lineage disease (SM-AHNMD), and three with mastcellsarcoma (MCS). FDG-PET was performed at the time of the SM diagnosis (15/19), to evaluate lymph node (LN) activity (3/19) or the efficacy of therapy (1/19). FDG uptake was observed in the bone marrow (BM) (9/19, 47%), LN (6/19, 32%), spleen (12/19, 63%), or liver (1/19, 5%). No significant FDG uptake was observed in the SSM and ASM patients
lymphadenopathy with blood or tissue eosinophilia. * * Mast cell leukemia: Patients are grouped as those with more than or those with less than 10% mast cells in the peripheral blood. * * Mastcellsarcoma * * Extracutaneous mastocytoma Patients whose conditions fall into the indolent category have a good prognosis, whereas patients with all other disorders have a relatively poor
* * Mast cell leukemia * * Mastcellsarcoma * * Extracutaneous mastocytoma [1, 2] This article focuses on cutaneous mastocytosis (CM). The single World Health Organization (WHO) major criterion is multifocal dense infiltrates of mast cells in bone marrow and/or other extracutaneous organs. One major and 1 minor criterion or 3 minor diagnostic criteria are needed to establish mastocytosis has a malignant transformation rate as high as 30%. [26] Rarely, mast cell leukemia may develop in young adults with persistent maculopapular cutaneous mastocytosis. [27] Primary cutaneous mastcellsarcoma due to the transformation of a benign solitary mastocytoma in an adult has been reported. [28] Cutaneous mastocytosis onset after age 10 years portends a poorer prognosis, because late-onset
lymphadenopathy with blood or tissue eosinophilia. * * Mast cell leukemia: Patients are grouped as those with more than or those with less than 10% mast cells in the peripheral blood. * * Mastcellsarcoma * * Extracutaneous mastocytoma Patients whose conditions fall into the indolent category have a good prognosis, whereas patients with all other disorders have a relatively poor
lymphadenopathy with blood or tissue eosinophilia. * * Mast cell leukemia: Patients are grouped as those with more than or those with less than 10% mast cells in the peripheral blood. * * Mastcellsarcoma * * Extracutaneous mastocytoma Patients whose conditions fall into the indolent category have a good prognosis, whereas patients with all other disorders have a relatively poor
. Malignant transformation of mastocytoma developed on skin mastocytosis into cutaneous mastcellsarcoma. Am J Surg Pathol. 2012 May. 36(5):779-82. [QxMD MEDLINE Link]. 29. Brockow K, Jofer C, Behrendt H, Ring J. Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients. Allergy. 2008 Feb. 63(2):226-32. [QxMD MEDLINE Link]. 30
* * Mast cell leukemia * * Mastcellsarcoma * * Extracutaneous mastocytoma [1, 2] This article focuses on cutaneous mastocytosis (CM). The single World Health Organization (WHO) major criterion is multifocal dense infiltrates of mast cells in bone marrow and/or other extracutaneous organs. One major and 1 minor criterion or 3 minor diagnostic criteria are needed to establish mastocytosis has a malignant transformation rate as high as 30%. [26] Rarely, mast cell leukemia may develop in young adults with persistent maculopapular cutaneous mastocytosis. [27] Primary cutaneous mastcellsarcoma due to the transformation of a benign solitary mastocytoma in an adult has been reported. [28] Cutaneous mastocytosis onset after age 10 years portends a poorer prognosis, because late-onset
lymphadenopathy with blood or tissue eosinophilia. * * Mast cell leukemia: Patients are grouped as those with more than or those with less than 10% mast cells in the peripheral blood. * * Mastcellsarcoma * * Extracutaneous mastocytoma Patients whose conditions fall into the indolent category have a good prognosis, whereas patients with all other disorders have a relatively poor
. Malignant transformation of mastocytoma developed on skin mastocytosis into cutaneous mastcellsarcoma. Am J Surg Pathol. 2012 May. 36(5):779-82. [QxMD MEDLINE Link]. 29. Brockow K, Jofer C, Behrendt H, Ring J. Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients. Allergy. 2008 Feb. 63(2):226-32. [QxMD MEDLINE Link]. 30