Fowler M R, Williams R B, Alba J M, Byrd C R
Am J Surg Pathol. 1982 Jun;6(4):363-74. doi: 10.1097/00000478-198206000-00009.
A large presacral myelolipoma associated with increasing constipation is reported in a 70-year-old obese diabetic female. The resected tumor was encapsulated and dark red to yellow. Light and electron microscopy revealed mature and immature hematopoietic tissue, lymphoid aggregates and mature adipocytes. Myelolipomas arising in extra-adrenal sites are rare. Considered in the differential diagnosis are reactive extramedullary hematopoietic tumors that usually occur in patients with severe chronic anemias. Patients with extramedullary hematopoietic tumors are characterized by anemia, frequent hepatosplenomegaly and abnormal peripheral blood smears, and may be any age. The tumors are usually multiple and usually located in the mediastinum or epidural space. In contrast, patients with extra-adrenal myelolipomas are usually older than 40 years, have normal blood studies, absent hepatosplenomegaly, and usually have chronic debilitating diseases or endocrinopathies. Extra-adrenal myelolipomas are single and usually occur within the abdomen. Extramedullary hematopoietic tumors have a predominance of the hematopoietic component, with erythroid hyperplasia of that component. Extra-adrenal myelolipomas may have a predominance of either the hematopoietic or fatty component, chiefly the latter, and generally have a more conspicuous lymphocyte population. Although the components of these tumors are the same, we consider them separable clinically, pathogenetically and, in many cases, pathologically.
报道了一名70岁肥胖糖尿病女性患者，其患有与便秘加重相关的巨大骶前髓脂肪瘤。切除的肿瘤有包膜，颜色从暗红色到黄色。光镜和电镜检查显示有成熟和不成熟的造血组织、淋巴样聚集物及成熟脂肪细胞。肾上腺外部位发生的髓脂肪瘤罕见。鉴别诊断需考虑反应性髓外造血肿瘤，这类肿瘤通常发生于严重慢性贫血患者。髓外造血肿瘤患者的特征为贫血、频繁肝脾肿大及外周血涂片异常，可发生于任何年龄。肿瘤通常为多发，常位于纵隔或硬膜外间隙。相比之下，肾上腺外髓脂肪瘤患者通常年龄大于40岁，血液检查正常，无肝脾肿大，通常患有慢性衰弱性疾病或内分泌病。肾上腺外髓脂肪瘤为单发，通常发生于腹部。髓外造血肿瘤以造血成分占优势，该成分有红系增生。肾上腺外髓脂肪瘤的造血或脂肪成分均可占优势，主要是后者，且通常有更明显的淋巴细胞群体。尽管这些肿瘤的成分相同，但我们认为它们在临床、发病机制及很多情况下在病理方面均可区分。
