Nagpal Anish P, Chandra Somesh, Goel Shaifali
Anya Gastro Surgicentre, Ahmedabad, India.
Sterling Hospital, Ahmedabad, India.
Indian J Surg Oncol. 2016 Dec;7(4):464-466. doi: 10.1007/s13193-016-0542-0. Epub 2016 Jul 22.
Extraosseous osteogenic sarcoma is a very rare malignant neoplasm. The most common sites are the extremities, thorax, and the abdomen. Retroperitoneal osteosarcomas are rare and very few cases have been reported. They are similar in their biology to high-grade soft tissue sarcomas. R0 resection appears to be the best possible treatment for these tumors but there are no published cases on how to manage them when it involves posterior and intra-spinal regions. We report a 62-year-old male who presented with a backache, and investigations revealed a large retroperitoneal fibrosarcoma invading into the lumbar spine, but was found to be an extra osseous osteosarcoma on final histopathological examination. It is important to emphasize that due to the rarity of soft tissue sarcomas as well as the uniqueness of the multimodal treatment plan for each subtype, soft tissue sarcomas involving the spine are best managed by a multi disciplinary team. Overall, patients with soft tissue sarcomas involving the spine usually present a poor long-term prognosis. Therefore, whenever feasible, "en bloc" resection of such lesions has been shown to play a crucial role in improving the overall and recurrence-free survival rates.
骨外骨肉瘤是一种非常罕见的恶性肿瘤。最常见的部位是四肢、胸部和腹部。腹膜后骨肉瘤很罕见,仅有少数病例报道。它们在生物学特性上与高级别软组织肉瘤相似。R0切除似乎是这些肿瘤的最佳治疗方法,但对于涉及脊柱后部和椎管内区域的病例,尚无关于如何处理的公开报道。我们报告了一名62岁男性,他因背痛就诊,检查发现一个巨大的腹膜后纤维肉瘤侵犯腰椎,但最终组织病理学检查发现是骨外骨肉瘤。必须强调的是,由于软组织肉瘤罕见,且每种亚型的多模式治疗方案独特,累及脊柱的软组织肉瘤最好由多学科团队管理。总体而言,累及脊柱的软组织肉瘤患者长期预后通常较差。因此,只要可行,此类病变的“整块”切除已被证明在提高总体生存率和无复发生存率方面起着关键作用。