Department of Orthopaedics and Traumatology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC; Department of Orthopaedics, Therapeutical and Research Center of Musculoskeletal Tumor, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
Department of Orthopaedics and Traumatology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC; Department of Orthopaedics, Therapeutical and Research Center of Musculoskeletal Tumor, Taipei Veterans General Hospital, Taipei, Taiwan, ROC; Institute of Clinical Medicine, School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC; Department of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
J Chin Med Assoc. 2018 Aug;81(8):735-741. doi: 10.1016/j.jcma.2018.01.006. Epub 2018 Apr 3.
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.
We performed a retrospective study to evaluate the clinical and pathological features and oncological 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.
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).
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.
腺泡状软组织肉瘤（ASPS）是一种罕见的软组织肿瘤，主要影响年轻患者。与其他软组织肉瘤一样，它具有较高的肺转移能力，但与其他软组织肉瘤相比，它具有较高的脑转移能力。由于该疾病的罕见性，大多数 ASPS 的研究都是病例报告和小系列研究。
我们对 1994 年至 2014 年间在我院治疗的局限性或转移性 ASPS 连续患者进行回顾性研究，评估其临床病理特征和肿瘤学结果。评估了人口统计学、位置、疾病严重程度、治疗方法、无进展生存率和总生存率。
共调查了 13 名患者。原发性肿瘤最常见的部位是大腿（n=6，47%），其次是侧腹（n=3，23%）、前臂（n=2，15%）和小腿（n=2，15%）。有 3 名患者最初在其他地方被诊断为患有血管瘤。这些患者接受了非计划的肿瘤内切除术。所有患者均在我院接受了广泛的肿瘤切除术。在平均 80.5 个月（36-133 个月）的随访期内，5 年总生存率为 67.5%。4 名患者无疾病持续存在（31%），6 名患者带瘤生存（46%），3 名患者死于疾病（23%）。在 9 名出现远处肺转移的患者中，有 2 名患者有骨和脑转移。接受化疗和未接受化疗的患者 5 年生存率分别为 66.7%和 66.7%（p=0.941）。
ASPS 的治疗策略是广泛切除，术后化疗可能对长期生存至关重要。此外，这种类型的肿瘤在诊断时具有较高的远处转移率，特别是在肺部和脑部。
