Dujardin Fanny, Beaussart Pauline, de Muret Anne, Rosset Philippe, Waynberger Eric, Mulleman Denis, de Pinieux Gonzague
Department of Pathology, Hôpital Trousseau, CHRU de Tours, 37042 Tours Cedex 09, France.
Skeletal Radiol. 2009 Aug;38(8):819-23. doi: 10.1007/s00256-009-0693-y. Epub 2009 Apr 10.
Primary carcinoid tumor (well-differentiated neuroendocrine tumor) of the bone involving the sacrum is extremely rare. We report the case of a 72-year-old man who presented with a 20-year history of intermittent low back pain and was found to have an intraosseous sacral mass on imaging. A needle biopsy revealed that this lesion was a well-differentiated neuroendocrine tumor. Workup did not show any primary tumor or other metastatic disease. There was no associated tailgut cyst or sacrococcygeal teratoma. The lesion was treated with radiation therapy because a surgical approach was rejected. The patient is free of metastatic disease after 28 years evolution of the lesion, retrospectively seen to be present on a conventional radiography performed in 1980. A review of the literature revealed 20 case reports of neuroendocrine tumors arising from the presacral region (with or without associated tailgut cyst or sacrococcygeal teratoma) and sometimes extending to the sacrum. One additional case was located within the neural canal and involved the sacrum, the presacral region, and the rectal wall. Our case is the only tumor arising primarily from the sacrum. The long evolution of this lesion without any other location makes metastatic disease very improbable and this case appears to be a unique example of primary intraosseous sacral carcinoid tumor.
累及骶骨的原发性类癌肿瘤（高分化神经内分泌肿瘤）极为罕见。我们报告一例72岁男性，有20年间歇性腰痛病史，影像学检查发现骶骨有一骨内肿块。针吸活检显示该病变为高分化神经内分泌肿瘤。检查未发现任何原发性肿瘤或其他转移性疾病。无相关尾肠囊肿或骶尾部畸胎瘤。由于拒绝手术，该病变接受了放射治疗。回顾性观察发现，该病变在1980年的传统X线片上就已存在，经过28年的病变发展，患者无转移性疾病。文献回顾显示，有20例关于起源于骶前区域（伴或不伴相关尾肠囊肿或骶尾部畸胎瘤）且有时延伸至骶骨的神经内分泌肿瘤的病例报告。另外1例位于椎管内，累及骶骨、骶前区域和直肠壁。我们的病例是唯一主要起源于骶骨的肿瘤。该病变长期存在且无其他部位受累，发生转移性疾病的可能性极小，此病例似乎是原发性骶骨骨内类癌肿瘤的独特例子。
