Jundt G, Baumhoer D
Knochentumor-Referenzzentrum und DÖSAK-Zentralregister am Institut für Pathologie, Universitätsspital Basel, Schoenbeinstraße 40, 4031, Basel, Schweiz.
Pathologe. 2018 Mar;39(2):132-138. doi: 10.1007/s00292-017-0397-3.
Chondroblastomas are very rare benign primary bone tumors occurring preferentially in the epiphyses or apophyses of long bones in children and adolescents. In most cases the typical histological and imaging findings lead to a correct diagnosis that may be substantiated by demonstrating the highly specific point mutation in the H3F3B gene (p.K36M), either by sequencing or immunohistochemistry. Recurrences occur in 5-15% of cases, postsurgical metastatic deposits to the lungs are very rare (<1%). Histologically "malignant" chondroblastomas have been reported as single case reports. The treatment of choice is a thorough curettage, also in the case of local relapses.
软骨母细胞瘤是非常罕见的原发性良性骨肿瘤,好发于儿童和青少年长骨的骨骺或骨突。在大多数情况下,典型的组织学和影像学表现可得出正确诊断,通过测序或免疫组织化学检测H3F3B基因(p.K36M)中的高度特异性点突变可进一步证实。5%-15%的病例会复发,术后肺转移非常罕见(<1%)。组织学上“恶性”的软骨母细胞瘤仅有个案报道。治疗的首选方法是彻底刮除术,局部复发时也是如此。