THE MALIGNANT POTENTIAL OF ENCHONDROMATOSIS
THE MALIGNANT POTENTIAL OF ENCHONDROMATOSIS
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DOI：
10.2106/00004623-198769020-00016
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发表时间：
1987-02-01
影响因子：
5.3
通讯作者：
BONFIGLIO, M
中科院分区：
文献类型：
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作者：
SCHWARTZ, HS;ZIMMERMAN, NB;BONFIGLIO, M
在一项长期随访的三机构回顾性研究中，确定了44名患有多发性内生软骨瘤的患者。37名患者没有血管瘤（奥利耶病），7名患者有血管瘤（马富奇综合征）。在37名患有奥利耶病的患者中，有4名发展为低度软骨肉瘤，1名发展为星形细胞瘤，1名发展为卵巢颗粒细胞瘤。在7名患有Maffucci综合征的患者中，有4名患者患有6例低度恶性软骨肉瘤，1例高度恶性骨肉瘤，1例胰腺癌，1例胆管腺癌和1例星形细胞瘤。两组中均无患者死于骨骼肉瘤，但5例非骨骼恶性病变患者中有4例死亡。根据对这些患者的生命表分析，我们估计，在40岁时，奥利耶病患者继发性软骨肉瘤的发生率约为25%，而在马富奇综合征患者中，恶性变性几乎是肯定的。我们的结论是，对于内生软骨瘤病患者，定期监测脑部和腹部的隐匿性恶性病变是必要的。
In a tri-institutional, retrospective study with long-term follow-up, forty-four patients who had multiple enchondromas were identified. Thirty-seven patients did not have hemangiomas (Ollier disease) and seven did (Maffucci syndrome). Of the thirty-seven patients who had Ollier disease, a low-grade chondrosarcoma developed in four; an astrocytoma, in one; and a granulosa-cell ovarian tumor, in one. In four of the seven patients who had Maffucci syndrome, there were six low-grade chondrosarcomas, one high-grade osteosarcoma, one pancreatic adenocarcinoma, one biliary adenocarcinoma and one astrocytoma. None of the patients in either group died of the skeletal sarcoma, but four of five patients who had a non-skeletal malignant lesion died. From life-table analyses of these patients, we estimated that the incidence of secondary chondrosarcoma in patients who have Ollier disease is about 25 per cent at the age of forty years, and that malignant degeneration is almost a certainty in patients who have Maffucci syndrome. We concluded that periodic surveillance of the brain and abdomen for occult malignant lesions is indicated in patients who have enchondromatosis.