Tanaka Y, Sasaki Y, Nishihira H, Izawa T, Nishi T
Department of Pathology, Kanagawa Children's Medical Center, Yokohama, Japan.
Am J Clin Pathol. 1992 Apr;97(4):523-7. doi: 10.1093/ajcp/97.4.523.
A 15-year-old girl developed a juvenile granulosa cell tumor associated with Maffucci's syndrome (enchondromatosis + hemangiomas). Clinical manifestations of the disease included an abdominal mass and progressive anemia. She underwent the removal of a Stage Ic juvenile granulosa cell tumor and subsequent adjuvant chemotherapy. On follow-up examination 4 years later, no recurrence of the ovarian tumor was noted. A review of the literature showed 10 previous cases of juvenile granulosa cell tumor associated with enchondromatosis, two associated with Maffucci's syndrome, and the rest with Ollier's disease (enchondromatosis). Ovarian juvenile granulosa cell tumor may occur not infrequently in female patients with enchondromatosis in the first or second decades, in contrast to the widely recognized sarcomatous changes of enchondromas that usually occur after the second decade. Data provided from these cases also emphasize the concept of a generalized mesodermal dysplasia.
一名15岁女孩患了与马富西综合征（内生软骨瘤病 + 血管瘤）相关的青少年颗粒细胞瘤。该疾病的临床表现包括腹部肿块和进行性贫血。她接受了Ic期青少年颗粒细胞瘤的切除及后续辅助化疗。4年后的随访检查未发现卵巢肿瘤复发。文献回顾显示，之前有10例青少年颗粒细胞瘤与内生软骨瘤病相关，其中2例与马富西综合征相关，其余与奥利尔病（内生软骨瘤病）相关。与通常在第二个十年后发生的内生软骨瘤广泛认可的肉瘤样改变不同，卵巢青少年颗粒细胞瘤在患有内生软骨瘤病的女性患者的第一个或第二个十年中可能并不少见。这些病例提供的数据也强调了广义中胚层发育异常的概念。
