Department of Neurosurgery, Royal Care Super Specialty Hospital, Neelambur, Coimbatore, Tamilnadu, India.
Spinal Disorders Center, Fujieda Heisei Memorial Hospital, Fujieda City, Shizuoka, Japan.
World Neurosurg. 2020 Mar;135:267-272. doi: 10.1016/j.wneu.2019.12.088. Epub 2019 Dec 25.
Sacral schwannomas are rare tumors arising from nerve sheath Schwann cells. They are classified into 3 types: first type schwannoma arising from the sacrum, second type schwannoma eroding the sacrum, and third type schwannoma extending extensively, involving anterior, posterior, and retroperitoneal space. Among these type 2 sacral tumors, according to Klimo's classification, an eroding sacrum extending all over posteriorly, anteriorly, and laterally is still rarer. Only 38 cases have been reported in literature reviews. Only 6 cases of giant tumors of >10 cm in any 1 dimension have been reported.
We present such a giant sacral schwannoma, with invasiveness eroding the sacrum; compressing the ureters, aortic bifurcation, bladder, and bowel; and presenting as lower abdominal pain, hydronephrosis, dysuria, and constipation. This is the first case reported in the literature that has described a large retroperitoneal tumor compressing retroperitoneal structures-aortic bifurcation vascular compression and ureter compression causing hydronephrosis.
Though complete resection, which caused various severe postoperative complications in the reported cases, is the best option, a less morbid procedure would be appropriate; hence we underwent subtotal excision of the tumor, with complete recovery of our patient's symptoms without neurologic deficit.
骶骨神经鞘瘤是一种起源于神经鞘 Schwann 细胞的罕见肿瘤。它们分为 3 种类型:第一型起源于骶骨的神经鞘瘤,第二型侵蚀骶骨的神经鞘瘤,以及第三型广泛延伸的神经鞘瘤,涉及前、后和腹膜后间隙。在这些类型的 2 种骶骨肿瘤中,根据 Klimo 的分类,一种向后、向前和向侧面广泛侵蚀的骶骨仍然更为罕见。文献回顾中仅报道了 38 例。仅报道了 6 例任何 1 个维度>10cm 的巨大肿瘤。
我们介绍了这样一个巨大的骶骨神经鞘瘤,具有侵袭性,侵蚀骶骨;压迫输尿管、主动脉分叉、膀胱和肠道;并表现为下腹痛、肾积水、排尿困难和便秘。这是文献中首次报道的一例大腹膜后肿瘤压迫腹膜后结构-主动脉分叉血管受压和输尿管受压导致肾积水的病例。
虽然完全切除是最好的选择,但会导致各种严重的术后并发症,因此我们进行了肿瘤的大部分切除术,患者的症状完全缓解,没有神经功能缺损。