PDF(Pubmed)
Abstract：
UNASSIGNED: Sclerosing epitheloid fibrosarcoma is a rare variant of low grade sarcoma with specific histologic and immunohistochemical features, and often associated with a poor prognosis.
METHODS: We report a case of a 35-year old male who presented with 2 year history of lower back pain, radiating to both lower limbs, weakness to both lower limbs and weight loss more than 5kgs in the past 6 months, no past history of trauma, drenching night sweats, no TB contact history, no incontinence and no any comorbidities. He reported no history of alcohol intake or cigarette smoking, no history of exposure to radiations or similar presentation to his family and no features suggestive of metastasis. On examination - he had maximum midline tenderness L3-S1, no gibbous, upper limbs neurologically intact and power 3/5 to both lower limbs, sensation intact. Computed tomography scan and magnetic resonance imaging revealed an ill-defined osteolytic mass spanning L3 to L5, vertebral plana of L4 and involvement of the left paraspinal muscles. The tumor extended to the extradural space and was also abutting on the distal great vessels. Our intervention entailed laminectomy, tumor debulking and posterior instrumental fusion of the spine. Histopathology findings were in keeping with an invasive sclerosing epitheloid fibrosarcoma. He had adjuvant chemotherapy with good outcome.
CONCLUSIONS: This case was unique due to its lumbar column location, abutting the distal vessels and despite its low grade, it illustrates the malignant potential which responded well to adjuvant chemotherapy.
CONCLUSIONS: Invasive SEF is an aggressive tumor that requires early diagnosis. Multi-modal treatment with surgical resection, adjuvant chemotherapy can improve patient survival and quality of life.
METHODS: We report a case of a 35-year old male who presented with 2 year history of lower back pain, radiating to both lower limbs, weakness to both lower limbs and weight loss more than 5kgs in the past 6 months, no past history of trauma, drenching night sweats, no TB contact history, no incontinence and no any comorbidities. He reported no history of alcohol intake or cigarette smoking, no history of exposure to radiations or similar presentation to his family and no features suggestive of metastasis. On examination - he had maximum midline tenderness L3-S1, no gibbous, upper limbs neurologically intact and power 3/5 to both lower limbs, sensation intact. Computed tomography scan and magnetic resonance imaging revealed an ill-defined osteolytic mass spanning L3 to L5, vertebral plana of L4 and involvement of the left paraspinal muscles. The tumor extended to the extradural space and was also abutting on the distal great vessels. Our intervention entailed laminectomy, tumor debulking and posterior instrumental fusion of the spine. Histopathology findings were in keeping with an invasive sclerosing epitheloid fibrosarcoma. He had adjuvant chemotherapy with good outcome.
CONCLUSIONS: This case was unique due to its lumbar column location, abutting the distal vessels and despite its low grade, it illustrates the malignant potential which responded well to adjuvant chemotherapy.
CONCLUSIONS: Invasive SEF is an aggressive tumor that requires early diagnosis. Multi-modal treatment with surgical resection, adjuvant chemotherapy can improve patient survival and quality of life.
摘要：
■硬化性上皮样纤维肉瘤是一种罕见的低级别肉瘤，具有特定的组织学和免疫组织化学特征，通常与预后不良有关。
方法：我们报告一例35岁男性，有2年的腰痛病史，辐射到双下肢，在过去的6个月中，下肢无力和体重减轻超过5公斤，没有创伤史,湿透的盗汗，没有结核病接触历史，没有尿失禁和任何合并症。他报告没有饮酒或吸烟的历史，没有放射史或其家人的类似表现，也没有提示转移的特征。在检查中-他有最大的中线压痛L3-S1，没有隆起，上肢的神经系统完好无损，双下肢力量3/5，感觉完好无损。计算机断层扫描和磁共振成像显示，跨越L3至L5的溶骨性肿块不明确，L4的椎骨平面和左侧椎旁肌肉受累。肿瘤延伸到硬膜外腔，并且还邻接远端大血管。我们的干预需要椎板切除术，肿瘤减积和脊柱后路器械融合。组织病理学发现与侵袭性硬化性上皮样纤维肉瘤一致。他接受了辅助化疗，结果良好。
结论：由于腰柱位置，该病例是独特的，邻接远端血管，尽管它的等级低，它说明了对辅助化疗反应良好的恶性潜能。
结论：侵袭性SEF是一种侵袭性肿瘤，需要早期诊断。多模式治疗与手术切除，辅助化疗可以提高患者的生存率和生活质量。
方法：我们报告一例35岁男性，有2年的腰痛病史，辐射到双下肢，在过去的6个月中，下肢无力和体重减轻超过5公斤，没有创伤史,湿透的盗汗，没有结核病接触历史，没有尿失禁和任何合并症。他报告没有饮酒或吸烟的历史，没有放射史或其家人的类似表现，也没有提示转移的特征。在检查中-他有最大的中线压痛L3-S1，没有隆起，上肢的神经系统完好无损，双下肢力量3/5，感觉完好无损。计算机断层扫描和磁共振成像显示，跨越L3至L5的溶骨性肿块不明确，L4的椎骨平面和左侧椎旁肌肉受累。肿瘤延伸到硬膜外腔，并且还邻接远端大血管。我们的干预需要椎板切除术，肿瘤减积和脊柱后路器械融合。组织病理学发现与侵袭性硬化性上皮样纤维肉瘤一致。他接受了辅助化疗，结果良好。
结论：由于腰柱位置，该病例是独特的，邻接远端血管，尽管它的等级低，它说明了对辅助化疗反应良好的恶性潜能。
结论：侵袭性SEF是一种侵袭性肿瘤，需要早期诊断。多模式治疗与手术切除，辅助化疗可以提高患者的生存率和生活质量。
