Brcić Luka, Vuletić Lovorka Batelja, Stepan Jasminka, Bonevski Aleksandra, Jakovljević Gordana, Gasparov Slavko, Marjanović Ksenija, Seiwerth Sven
Institute of Pathology, Medical School, University of Zagreb, Zagreb, Croatia.
J Clin Pathol. 2007 Apr;60(4):424-5. doi: 10.1136/jcp.2006.040857.
The mast-cell sarcoma of a bone is described here for the first time. The tumour presented in a 4-year-old boy, with pain, oedema and deformation of his right lower leg. Radiological findings revealed a destructive tumourous mass. Histopathological examination showed the tumour to be composed of large, atypical cells, with hyperchromatic oval and polygonal nuclei. The cytoplasm around them was eosinophilic with many basophilic and toluidine-blue-positive granules. These atypical mast cells were positive for chloroacetate esterase, c-kit, tryptase and negative for myeloperoxidase. The primary disease quickly progressed to mast-cell leukaemia, and despite intensive chemotherapy the patient died 18 months after first symptoms.
本文首次描述了一例骨肥大细胞肉瘤。该肿瘤发生于一名4岁男孩,表现为右小腿疼痛、水肿和畸形。放射学检查发现一个破坏性肿瘤肿块。组织病理学检查显示肿瘤由大的非典型细胞组成,细胞核呈深染的椭圆形和多边形。其周围的细胞质呈嗜酸性,有许多嗜碱性和甲苯胺蓝阳性颗粒。这些非典型肥大细胞氯乙酸酯酶、c-kit、类胰蛋白酶呈阳性,髓过氧化物酶呈阴性。原发疾病迅速进展为肥大细胞白血病,尽管进行了强化化疗,患者在出现首发症状18个月后死亡。