Ravnik Janez, Potrc Stojan, Kavalar Rajko, Ravnik Maja, Zakotnik Branko, Bunc Goraz
Department of Neurosurgery, University Medical Centre Maribor, Maribor, Slovenia.
Spine (Phila Pa 1976). 2009 May 1;34(10):E363-6. doi: 10.1097/BRS.0b013e31819b2cab.
A case report is of a giant dumbbell-shaped synovial sarcoma of the thoracolumbar spine is presented.
To report a case of a rare dumbbell-shaped tumor treated by multimodal approach. Surgical procedures, adjuvant treatment, and outcome were discussed.
Synovial sarcomas of the spine are very rare tumors. Radical surgical resection is the goal, but is often not feasible. Dumbbell-shaped spinal synovial sarcoma with a giant extraspinal extension has not yet been reported. The rationale for 2-step surgical procedure and adjuvant therapy is discussed in light of the clinical picture, preoperative imaging and extension of the disease.
A 32-year-old male patient presented with signs of quickly progressive paraparesis. A dumbbell-shaped tumor at the level Th12-L1 was found with a giant retroperitoneal extension. Tumor was nonradically excised in a 2-step operation: first through a dorsal approach with laminectomy and removal of the intraspinal extradural part, and later through a laparotomy with removal of the retroperitoneal part. Histologic examination revealed highly malignant synovial sarcoma. Patient was treated with chemotherapy and radiotherapy after surgery.
Patient was in remission and symptom free for 1 year after surgery; he then developed a local recurrence and died soon afterwards.
A good treatment result was achieved initially. A combined approach in cases like this is warranted, with as radical surgery as possible in order to avoid local recurrence, a common cause of treatment failure in sarcomas.
本文报告一例胸腰椎巨大哑铃形滑膜肉瘤病例。
报告一例采用多模式方法治疗的罕见哑铃形肿瘤病例。讨论手术步骤、辅助治疗及结果。
脊柱滑膜肉瘤是非常罕见的肿瘤。根治性手术切除是目标,但往往不可行。尚未有关于具有巨大椎体外延伸的哑铃形脊柱滑膜肉瘤的报道。根据临床表现、术前影像学检查及疾病的延伸情况,讨论了两步手术及辅助治疗的基本原理。
一名32岁男性患者出现快速进展性截瘫症状。在胸12至腰1水平发现一个哑铃形肿瘤,伴有巨大的腹膜后延伸。通过两步手术对肿瘤进行了非根治性切除:首先通过后路椎板切除术切除椎管内硬膜外部分,随后通过剖腹手术切除腹膜后部分。组织学检查显示为高度恶性滑膜肉瘤。患者术后接受了化疗和放疗。
患者术后缓解且无症状生存1年;随后出现局部复发,不久后死亡。
最初取得了良好的治疗效果。对于此类病例,应采取联合治疗方法,尽可能进行根治性手术以避免局部复发,局部复发是肉瘤治疗失败的常见原因。