Racem Trigui, Mehdi Debaibi, Ahmed Guermazi, Faten Souai, Amen Dhaoui, Adnen Chouchen
Departement of General Surgery, Internal Security Forces Hospital, Marsa, Tunisia.
Departement of General Surgery, Internal Security Forces Hospital, Marsa, Tunisia; Faculty of Medicine of Tunis, University of Tunis el Manar, Tunisia.
Int J Surg Case Rep. 2023 Aug;109:108596. doi: 10.1016/j.ijscr.2023.108596. Epub 2023 Jul 29.
Synovial Sarcoma is an intriguing disease, it represents a distinctive subtype of soft tissue sarcoma that does not exceed 10 % of all STS. This tumor can arise from the abdominal wall in very rare cases. Due to its unique presentation (occurring at a young age, various anatomical locations, and slow evolutionary kinetics), diagnosis can be challenging. The mainstay of treatment remains wide surgical excision with negative margins.
We herein report a challenging diagnosis of synovial sarcoma with exceptional location, presented as a slowly evolving abdominal mass of the right iliac fossa. Soft tissue MRI confirmed the presence of a sub cutaneous mass without signs of local invasion. Surgical management as indicated. Anatomopathological findings were in favor of a synovial sarcoma of the abdominal wall. The patient was discharged. No complication was observed after 3 months follow up.
Patients with synovial sarcoma of the abdominal wall is a very rare entity, therefor positive pre operative diagnosis is hard to achieve, because of the lack of specific clinical and radiological signs. No standard treatment is advised, beside surgical management wish is the main course of management.
Synovial sarcoma is an infrequent pathology, with no specific signs in both clinical and radiological findings. The main course of management is surgery with healthy resection margins. Long term follow up is advised because of the high risk of recurrence.
滑膜肉瘤是一种引人关注的疾病，它是软组织肉瘤的一种独特亚型，在所有软组织肉瘤中占比不超过10%。这种肿瘤在极罕见的情况下可起源于腹壁。因其独特的表现（发病年龄轻、解剖位置多样且生长动力学缓慢），诊断颇具挑战性。治疗的主要方法仍是进行切缘阴性的广泛手术切除。
我们在此报告一例具有特殊位置的滑膜肉瘤的挑战性诊断病例，该病例表现为右髂窝处缓慢生长的腹部肿块。软组织MRI证实存在皮下肿块，无局部侵袭迹象。按指示进行手术治疗。解剖病理学检查结果支持腹壁滑膜肉瘤的诊断。患者出院。随访3个月后未观察到并发症。
腹壁滑膜肉瘤患者是一种非常罕见的情况，因此术前很难做出阳性诊断，因为缺乏特异性的临床和放射学征象。除手术治疗这一主要治疗方法外，尚无标准治疗方案。
滑膜肉瘤是一种罕见的病理类型，临床和放射学检查均无特异性征象。主要治疗方法是进行切缘健康的手术切除。鉴于复发风险高，建议进行长期随访。
