Berking C, Brady M S
Ludwig-Maximilians-University, Munich, Germany.
Cancer. 1997 Feb 15;79(4):843-8. doi: 10.1002/(sici)1097-0142(19970215)79:4<843::aid-cncr22>3.0.co;2-z.
The authors became interested in an association between cutaneous melanoma and sarcoma when they reviewed their experience with other malignancies occurring in patients with a diagnosis of sarcoma.
The authors identified 48 patients with both melanoma and bone or soft tissue sarcoma (STS) by a computer search of all sarcoma patients entered into their institution's cancer registry between 1943 and 1996 who had an additional diagnosis of melanoma. The medical records were reviewed and clinical and pathologic data collected.
The median age at diagnosis was 46 years for patients with melanoma and 50 years for patients with sarcoma, which was consistent with population-based data. Among patients with STS (n = 41), malignant peripheral nerve sheath tumors (MPNT) were more common in patients with both diagnoses (5 of 41; 13%) when compared with all adults with STS admitted to the study center between 1982 to date (125 of 2901; 4%; P < 0.05). Liposarcoma occurred in only 1 patient with both melanoma and STS (1 of 41; 2%), despite the fact that it was the most common histologic diagnosis in all adults with STS (625 of 2901; 22%; P < 0.001). The anatomic site of STS was more commonly visceral (11 of 41; 27%) when compared with all adults with STS (424 of 2901; 15%; P < 0.05). A positive family history of cancer was noted in 50% of the patients, and 25% of patients had a third primary tumor.
Although a distinct "melanoma/sarcoma" syndrome was not identified, MPNT as well as visceral sarcomas were more common than expected in this study. The authors also noted strong family histories of cancer as well as additional primary malignancies in patients with melanoma and sarcoma, suggesting a predisposition toward cancer.
作者在回顾其诊断为肉瘤的患者中发生的其他恶性肿瘤的经验时，对皮肤黑色素瘤与肉瘤之间的关联产生了兴趣。
作者通过计算机检索1943年至1996年间录入其机构癌症登记处的所有肉瘤患者，确定了48例同时患有黑色素瘤和骨或软组织肉瘤（STS）且另有黑色素瘤诊断的患者。对病历进行了回顾，并收集了临床和病理数据。
黑色素瘤患者的诊断时中位年龄为46岁，肉瘤患者为50岁，这与基于人群的数据一致。在STS患者（n = 41）中，与1982年至今进入研究中心的所有成年STS患者相比，同时患有两种疾病的患者中恶性外周神经鞘瘤（MPNT）更为常见（41例中有5例；13%）（2901例中有125例；4%；P < 0.05）。脂肪肉瘤仅发生在1例同时患有黑色素瘤和STS的患者中（41例中有1例；2%），尽管它是所有成年STS患者中最常见的组织学诊断（2901例中有625例；22%；P < 0.001）。与所有成年STS患者相比，STS的解剖部位更常见于内脏（41例中有11例；27%）（2901例中有424例；15%；P < 0.05）。50%的患者有癌症家族史阳性，25%的患者有第三种原发性肿瘤。
尽管未发现明显的“黑色素瘤/肉瘤”综合征，但在本研究中MPNT以及内脏肉瘤比预期更为常见。作者还注意到黑色素瘤和肉瘤患者有强烈的癌症家族史以及额外的原发性恶性肿瘤，提示存在癌症易感性。
