Benetos Ioannis S, Mavrogenis Andreas F, Soultanis Konstantinos Ch, Zoubos Aristides B, Papagelopoulos Panayiotis J, Soucacos Panayotis N
First Department of Orthopaedics, Athens University Medical School, Athens, Greece.
J Surg Orthop Adv. 2006 Winter;15(4):209-13.
Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma that most commonly arises in the deep soft tissues of the lower extremities of adults. Median survival of patients with metastatic ASPS has been reported to be from 3 to 3.3 years. The time between detection of metastases and death varies from 10 months to 6.2 years. In this article, a case of an 11-year-old male with primary ASPS of the right forearm is presented. Successful long-term local control of the primary tumor was achieved with wide margin surgical resection and adjuvant radiation therapy. Three years after diagnosis, the patient developed pulmonary metastases. Chemotherapy was unsuccessfully used to control the metastatic disease. Despite that, the patient survived longer than expected, and passed away 9 years after the detection of pulmonary metastases. This time to death after the development of metastases vastly exceeded the previously reported survival rates of patients with metastatic ASPS.
肺泡软组织肉瘤(ASPS)是一种罕见的软组织肉瘤,最常见于成人下肢的深部软组织。据报道,转移性ASPS患者的中位生存期为3至3.3年。从发现转移到死亡的时间从10个月到6.2年不等。本文报告了一例11岁男性右前臂原发性ASPS病例。通过广泛边缘手术切除和辅助放疗成功实现了对原发性肿瘤的长期局部控制。诊断三年后,患者出现肺转移。化疗未能有效控制转移性疾病。尽管如此,患者存活时间超过预期,在发现肺转移9年后去世。转移发生后的死亡时间大大超过了先前报道的转移性ASPS患者的生存率。