Mestiri Sarra, Elghali Mohamed Amine, Bourigua Rym, Abdessayed Nihed, Nasri Salsabil, Amine Ben Abdallah, Missaoui Nabiha, Ben Maitig Mahmoud, Hmissa Sihem, Sriha Badreddine, Mokni Moncef
Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.
Cancer Registry of the Center of Tunisia, Sousse, Tunisia.
Rare Tumors. 2019 Jan 21;11:2036361318820171. doi: 10.1177/2036361318820171. eCollection 2019.
Soft tissue leiomyosarcomas are rare, accounting for almost 5%-10% of all soft tissue sarcomas; they account for almost 1% of all sarcomas. They are aggressive tumors where location, size, and management require a multidisciplinary approach. Since there are few series published, we here analyze epidemiological pattern, clinical and pathologic features of soft tissue leiomyosarcomas.
We conducted a retrospective study of 29 consecutive cases of histologically proven soft tissue leiomyosarcoma extracted from the database of the Cancer Registry of the Center of Tunisia and the Department of Pathology of Farhat Hached University Hospital of Sousse of Tunisia, during a 10-year period (from January 1996 to December 2005). Epidemiologic details, clinico-pathological features, and treatment modalities were assessed with focus on patients' 5-year overall survival, tumor relapse, and metastases.
Soft tissue leiomyosarcoma accounted for 17.5% of all soft tissue sarcomas diagnosed at our pathology department. Most of patients were of advanced age (median: 52 years), with extremes ranging from 12 and 87 years. There was a slight male predominance (sex-ratio = 1.07). Tumors were located mostly in the lower limbs (45%). Deep sites as retroperitoneum was found only in two cases. Tumor size was more than 5 cm in 83% of cases (average size = 9.4 cm). Five cases had metastasis on initial staging. For 24 patients, the disease was locally limited at the moment of diagnosis. Palliative chemotherapy was indicated for four patients and surgery was performed for 20 patients. Local recurrence occurred in 11 patients (55% of operated patients) and metastasis in 6 patients. Overall, 5-year survival was about 24%.
Our study results highlight the scarcity of soft tissue leiomyosarcoma. Unfortunately, unusual tumor sites, disease's advanced stages, and intralesional resection made the prognosis poorer than in other series. Clinical course of soft tissue leiomyosarcoma was highly marked by local recurrence and metastasis.
软组织平滑肌肉瘤较为罕见，占所有软组织肉瘤的近5%-10%；占所有肉瘤的近1%。它们是侵袭性肿瘤，其位置、大小及治疗需要多学科方法。由于发表的系列研究较少，我们在此分析软组织平滑肌肉瘤的流行病学模式、临床及病理特征。
我们对10年间（1996年1月至2005年12月）从突尼斯中心癌症登记数据库及突尼斯苏塞法哈特·哈谢德大学医院病理科提取的29例经组织学证实的软组织平滑肌肉瘤连续病例进行了回顾性研究。评估了流行病学细节、临床病理特征及治疗方式，重点关注患者的5年总生存率、肿瘤复发及转移情况。
软组织平滑肌肉瘤占我们病理科诊断的所有软组织肉瘤的17.5%。大多数患者为高龄（中位数：52岁），年龄范围为12至87岁。男性略占优势（性别比=1.07）。肿瘤大多位于下肢（45%）。仅2例位于腹膜后等深部部位。83%的病例肿瘤大小超过5厘米（平均大小=9.4厘米）。5例初诊时已有转移。24例患者诊断时疾病局限于局部。4例患者接受姑息化疗，20例患者接受手术。11例患者发生局部复发（占手术患者的55%），6例患者发生转移。总体而言，5年生存率约为24%。
我们的研究结果凸显了软组织平滑肌肉瘤的稀缺性。不幸的是，不寻常的肿瘤部位、疾病的晚期阶段及病灶内切除使得预后比其他系列研究更差。软组织平滑肌肉瘤的临床病程以局部复发和转移为显著特征。
