Vargas-Gonzalez Roberto, Sotelo-Avila Cirilo, Coria Araceli Solis
Department of Pathology, Hospital Para el Nino Poblano, Puebla, Mexico.
Pathol Oncol Res. 2003;9(3):193-5. doi: 10.1007/BF03033737. Epub 2003 Oct 7.
Renal medullary carcinoma (RMC), an aggressive malignant epithelial neoplasm, first emerged as a distinct clinicopathologic entity in 1995. It affects individuals 40 years of age or younger and is strongly associated with sickle cell disease or trait. The majority of patients with RMC have widely disseminated disease at the time of diagnosis and most fail to respond to both chemotherapy and radiotherapy. Mortality approaches 100%, and death usually occurs within a few months to a year of diagnosis. We report a 6-year-old African-American boy with a history of gross hematuria who died four weeks after diagnosis of disseminated metastatic disease. Autopsy showed a 4.4-cm renal mass with metastases to the contra lateral kidney, liver, lungs and multiregional lymph nodes. RMC should be included in the differential diagnosis of any patient 40 years old or younger with a history of hemoglobinopathy and gross hematuria and/or abdominal or flank pain. A brief discussion of the differential diagnosis, histogenesis and treatment is presented in this study.
肾髓质癌（RMC）是一种侵袭性恶性上皮肿瘤，于1995年首次作为一种独特的临床病理实体出现。它影响40岁及以下的个体，并且与镰状细胞病或性状密切相关。大多数肾髓质癌患者在诊断时已有广泛播散性疾病，且大多数对化疗和放疗均无反应。死亡率接近100%，死亡通常发生在诊断后的几个月至一年内。我们报告一名6岁非裔美国男孩，有肉眼血尿病史，在诊断为播散性转移性疾病四周后死亡。尸检显示一个4.4厘米的肾肿块，转移至对侧肾脏、肝脏、肺和多区域淋巴结。对于任何有血红蛋白病病史且有肉眼血尿和/或腹部或侧腹疼痛的40岁及以下患者，鉴别诊断时均应考虑肾髓质癌。本研究对鉴别诊断、组织发生和治疗进行了简要讨论。
