The Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu, Fukuoka, Japan.
Department of Pathology, Kenwakai Otemachi Hospital, Kitakyushu, Japan.
CEN Case Rep. 2021 Aug;10(3):414-421. doi: 10.1007/s13730-021-00580-3. Epub 2021 Feb 17.
Neuroblastoma is a well-known embryonal cancer; however, adult-onset neuroblastomas are rare. The systemic symptoms are related to catecholamine excretion or intraabdominal mass effects. Only two cases of neuroblastoma with nephrotic syndrome have previously been reported. We herein present the first autopsy case of neuroblastoma in an older individual associated with minimal change nephrotic syndrome.
A 63-year-old man was admitted to our hospital for investigation of general fatigue. His renal function was normal and his urine was negative for protein. A computed tomography scan showed a renal tumor and intraabdominal lymph node swelling. Approximately 4 months after admission, he suddenly developed acute renal failure and severe proteinuria, and hemodialysis was instituted. A computed tomography scan revealed an increase in the size of the renal tumor and lymph nodes. He died 1 month later and an autopsy was performed. The tumor exhibited diffuse proliferation of tumor cells with scant cytoplasm, namely small blue cell tumor with rosette formation. As a result of immunohistochemical study, a neuroblastoma was diagnosed. Despite the patient's severe renal failure, most glomeruli showed no remarkable changes. The tubular epithelium exhibited detachment and vacuolation. Electron microscopic study of the glomeruli showed diffuse effacement of the foot processes. These features indicate a diagnosis of minimal change nephrotic syndrome with acute tubular injury.
Minimal change nephrotic syndrome is the most common renal manifestation associated with lymphoproliferative malignancies. We here present an extremely rare case of adult-onset neuroblastoma with minimal change nephrotic syndrome.
神经母细胞瘤是一种众所周知的胚胎性癌症；然而，成人发病的神经母细胞瘤较为罕见。全身症状与儿茶酚胺排泄或腹腔内肿块效应有关。之前仅有两例神经母细胞瘤伴肾病综合征的病例报告。我们在此报告首例与微小病变性肾病综合征相关的老年个体发生的神经母细胞瘤尸检病例。
一名 63 岁男性因全身乏力入院检查。他的肾功能正常，尿液蛋白阴性。计算机断层扫描显示肾脏肿瘤和腹腔内淋巴结肿大。入院后约 4 个月，他突然出现急性肾衰竭和严重蛋白尿，并开始进行血液透析。计算机断层扫描显示肾脏肿瘤和淋巴结的大小增加。1 个月后，他死亡并进行了尸检。肿瘤表现为肿瘤细胞弥漫性增殖，细胞质稀少，即形成玫瑰花结的小蓝细胞肿瘤。免疫组织化学研究结果诊断为神经母细胞瘤。尽管患者的肾衰竭严重，但大多数肾小球没有明显变化。肾小管上皮细胞脱落并出现空泡。肾小球的电子显微镜研究显示足突弥漫性消失。这些特征表明存在微小病变性肾病综合征伴急性肾小管损伤。
微小病变性肾病综合征是与淋巴增生性恶性肿瘤相关的最常见的肾脏表现。我们在此报告首例成人发病的神经母细胞瘤伴微小病变性肾病综合征。
