Alveolar soft part sarcoma: Clinical presentation, treatment, and outcome in a series of 13 patients

作者：Lin Yu Kuan; Wu Po Kuei; Chen Cheng Fong; Chen Chao Ming; Tsai Shang Wen; Chen Paul Chih Hsueh; Chen Wei Ming^*

来源：Journal of the Chinese Medical Association, 2018, 81(8): 735-741.

DOI：10.1016/j.jcma.2018.01.006

• Alveolar soft part sarcoma
• Chemotherapy
• Metastasis
• Surgery

Background: Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor that typically affects young patients. Similar to other soft tissue sarcomas, it has high pulmonary metastasis ability, whereas compared with other soft tissue sarcomas, it has high brain metastasis ability. Because of the rarity of the disease, most studies on ASPS have been case reports and small series studies.
Method: We performed a retrospective study to evaluate the clinical and pathological features and ontological results in a consecutive series of patients with localized or metastatic ASPS treated at our institute between 1994 and 2014. Demographics, location, severity of disease, treatment provided, progression-free survival, and overall survival were evaluated.
Results: A total of 13 patients were investigated. The most common locations of primary tumor were the thigh (n = 6, 47%), followed by the flank (n = 3, 23%), forearm (n = 2, 15%), and calf (n = 2, 15%). Three patients were initially diagnosed as having hemangiomas elsewhere. These patients received unplanned intralesional excision. All the patients received wide tumor resection at our institute. Over the average follow-up period of 80.5 months (range: 36-133 months), the 5-year overall survival rate was 67.5%. Four patients were continuously disease free (31%), six were living with disease (46%), and three died of disease (23%). Of nine patients who presented with distant pulmonary metastasis, two had bony and brain metastases. The 5-year survival rate was 66.7% in patients who received chemotherapy and those who did not (p = 0.941).
Conclusion: The treatment strategy for ASPS is wide resection, and postoperative chemotherapy may be crucial for long-term survival. In addition, this type of tumor has a high distant metastasis rate at the time of diagnosis, particularly in the lungs and brain.

• 出版日期2018-8