Department of Biomorphological and Functional Science, Section of Anatomic Pathology, Federico II University, Via Pansini 5, 80131 Naples, Italy.
Pathol Res Pract. 2012 Nov 15;208(11):683-6. doi: 10.1016/j.prp.2012.06.010. Epub 2012 Sep 7.
Mastocytosis is a neoplastic disease of mast cells and their CD34+ precursors, including a heterogeneous group of disorders. It is characterized by abnormal growth and accumulation of mast cells in one or more organ systems. Mast cell sarcoma is an extremely rare and aggressive disease characterized by local proliferation of atypical mast cells, destructive growth and poor prognosis, without systemic involvement. Very few clinical cases describing this entity have been reported in the literature. In this paper, we report a case of a mast cell sarcoma, localized in the scalp of a 63-year-old woman; it appears to be the first manifestation of undisclosed systemic mastocytosis.
肥大细胞增多症是一种肥大细胞及其 CD34+前体细胞的肿瘤性疾病，包括一组异质性疾病。其特征是一个或多个器官系统中异常的肥大细胞生长和积累。肥大细胞瘤肉瘤是一种极为罕见且侵袭性的疾病，其特征是异常肥大细胞的局部增殖、破坏性生长和预后不良，而无全身受累。文献中很少有描述这种疾病的临床病例报道。本文报告了一例发生于 63 岁女性头皮的肥大细胞瘤肉瘤，它似乎是未明确的系统性肥大细胞增多症的首发表现。
