Department of Orthopaedics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
Department of Orthopaedics, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
World Neurosurg. 2019 Oct;130:459-466. doi: 10.1016/j.wneu.2019.07.117. Epub 2019 Jul 23.
Malignant peripheral nerve sheath tumor (MPNST), which refers to any malignant tumor that originates from peripheral nerve sheath cells, is rarely found in the spine. Primary intraosseous spinal MPNSTs are extremely uncommon; in fact, such an occurrence has been reported in a very limited number of cases hitherto. We report here one case of low-grade intraosseous MPNST in the lumbar vertebrae and review clinical research related to this rare tumor and modus operandi to its treatment.
A 51-year-old man suffering from lower back pain was initially diagnosed with lumbar tuberculosis. The intraoperative histopathologic examination, however, revealed that the occupying lesion was synovial sarcoma when surgeons intended to perform a debridement surgery of what was thought to be tuberculosis. The operation was therefore suspended. Two months later, the patient was referred to our institution. The pathologic sections were reexamined. Its results refuted the earlier thought of being synovial sarcoma, and a final diagnosis of MPNST was made. In addition, preoperative examination revealed that the tumor had advanced rapidly to the adjacent inferior vena cava and pedicle, which ruled out the possibility of performing a total en bloc spondylectomy. Therefore, a marginal resection for 2-level consecutive lumbar vertebrae was performed successfully via an anterior-posterior combined approach. No serious complications were detected at 18-month follow-up.
Preoperative biopsy is of great importance for the diagnosis of spinal tumors. Marginal resection of a complicated intraosseous spine MPNST via an anterior-posterior combined approach, despite being technically challenging, resulted in good survival and functional outcomes.
恶性外周神经鞘瘤（MPNST）是指起源于外周神经鞘细胞的任何恶性肿瘤，在脊柱中很少见。原发性骨内脊柱 MPNST 极为罕见；事实上，迄今为止，这种情况仅在极少数病例中报道过。我们在此报告一例腰椎低度骨内 MPNST，并回顾与这种罕见肿瘤相关的临床研究和治疗方法。
一名 51 岁男性因腰痛就诊，最初被诊断为腰椎结核。然而，当外科医生打算对被认为是结核病的病灶进行清创术时，术中组织病理学检查显示占位病变为滑膜肉瘤。因此，手术暂停。两个月后，该患者被转至我院。重新检查病理切片，结果推翻了之前认为是滑膜肉瘤的想法，最终诊断为 MPNST。此外，术前检查显示肿瘤已迅速侵犯相邻的下腔静脉和椎弓根，排除了行全椎体整块切除术的可能性。因此，成功地通过前后联合入路进行了 2 个连续腰椎的边缘性切除。18 个月随访时未发现严重并发症。
术前活检对于脊柱肿瘤的诊断非常重要。通过前后联合入路对复杂的骨内脊柱 MPNST 进行边缘性切除，尽管技术上具有挑战性，但仍能获得良好的生存和功能结果。
