Ramirez-Villaescusa José, Canosa-Fernández Adriana, Martin-Benlloch Antonio, Ruiz-Picazo David, López-Torres Hidalgo Jesús
Complejo Hospitalario Universitario de Albacete, Department of Orthopedics, Spine Unit, Albacete, Spain.
Complejo Hospitalario Universitario de Albacete, Department of Pathology, Albacete, Spain.
Int J Surg Case Rep. 2017;39:332-338. doi: 10.1016/j.ijscr.2017.08.045. Epub 2017 Sep 1.
To describe an unusual primary vertebral leiomyosarcoma in thoracic spine.
An isolated lesion of the T11 vertebra in a 62-year-old woman with no neurologic deficit is reported. Imaging findings indicated a nonspecific high-grade malignant lesion. TC-guided biopsy failed thus open incisional biopsy was needed. A diagnosis of low-intermediate mesenchymal sarcoma was made. A total en bloc spondylectomy of T11 was performed with three-column reconstruction. The histology and immunostaining showed the appearance of leiomyosarcoma. After diagnosis, post-operative radiation therapy was performed. Metastatic lesion was ruled out by CT scans of the chest, abdomen and pelvis, in addition to total body radionuclide scanning and 18-F-FDG-PET. After five years of follow-up, no signs of local recurrence, metastasis or distant lesions suggesting a primary lesion were observed.
Vertebral primary leiomyosarcoma is exceedingly rare. Primary vertebral leiomyosarcoma diagnosis must be performed when the metastatic origin is excluded. For the treatment of primary tumors, total en bloc spondylectomy (TES) is the technique of choice to achieve marginal or wide tumor resection, decrease the risk of local recurrence and remote lesions and increase survival.
A well-planned pre-operative study and a wide surgical excision can result in local tumor control and long-term survival. This case presents the longest disease-free survival period of a primary leiomyosarcoma in spinal location after total en bloc spondylectomy.
描述一例罕见的胸椎原发性平滑肌肉瘤。
报告一名62岁女性，T11椎体孤立性病变，无神经功能缺损。影像学检查结果显示为非特异性高级别恶性病变。CT引导下活检失败，因此需要进行切开活检。诊断为低中级间叶性肉瘤。对T11进行了全椎体整块切除术并进行了三柱重建。组织学和免疫染色显示为平滑肌肉瘤表现。确诊后，进行了术后放疗。除全身放射性核素扫描和18F-FDG-PET外，胸部、腹部和骨盆的CT扫描排除了转移病变。经过五年的随访，未观察到局部复发、转移或远处病变的迹象，提示为原发性病变。
椎体原发性平滑肌肉瘤极为罕见。必须在排除转移来源后才能诊断原发性椎体平滑肌肉瘤。对于原发性肿瘤的治疗，全椎体整块切除术（TES）是实现肿瘤边缘或广泛切除、降低局部复发和远处病变风险以及提高生存率的首选技术。
精心规划的术前研究和广泛的手术切除可实现局部肿瘤控制和长期生存。该病例是全椎体整块切除术后脊柱原发性平滑肌肉瘤最长的无病生存期病例。
