Sandberg Jesse K, Mullen Elizabeth A, Cajaiba Mariana M, Smith Ethan A, Servaes Sabah, Perlman Elizabeth J, Geller James I, Ehrlich Peter F, Cost Nicholas G, Dome Jeffrey S, Fernandez Conrad V, Khanna Geetika
Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 S. Kingshighway, Campus Box 8131, St. Louis, MO, 63110, USA.
Department of Pediatric Oncology, Children's Hospital Boston/Dana-Farber Cancer Institute, Boston, MA, USA.
Pediatr Radiol. 2017 Nov;47(12):1615-1621. doi: 10.1007/s00247-017-3926-6. Epub 2017 Jul 8.
Renal medullary carcinoma is a rare renal malignancy of childhood. There are no large series describing the imaging appearance of renal medullary carcinoma in children.
To characterize the clinical and imaging features of pediatric renal medullary carcinoma at initial presentation.
We retrospectively analyzed images of 25 pediatric patients with renal medullary carcinoma enrolled in the Children's Oncology Group renal tumors classification, biology and banking study (AREN03B2) from March 2006 to August 2016. Imaging findings of the primary mass, and patterns of locoregional and distant spread were evaluated in correlation with pathological and surgical findings.
Median age at presentation was 13 years (range: 6-21 years), with a male predominance (3.2:1). The overall stage of disease at initial presentation was stage 1 in 1, stage 2 in 2 and stage 4 in 22. Maximum diameter of the primary renal mass ranged from 1.6 to 10.3 cm (mean: 6.6 cm) with a slight right side predilection (1.5:1). Enlarged (>1 cm short axis) retroperitoneal lymph nodes were identified at initial staging in 20/25 (80%) cases, 10 of which were histologically confirmed while the others did not undergo surgical sampling. Enlarged lymph nodes were also identified in the mediastinum (14/25; 56%) and supraclavicular regions (4/25; 16%). Metastatic disease was present in the lungs in 19/25 (76%) and liver in 6/25 (24%). The pattern of lung metastases was pulmonary lymphangitic carcinomatosis: 10 cases (9 bilateral, 1 unilateral), pulmonary nodules with indistinct margins: 6 cases, pulmonary nodules with distinct margins: 2 cases, while 1 case had pulmonary nodules with both indistinct and distinct margins. Pulmonary lymphangitic carcinomatosis was pathologically confirmed in 4/10 cases. All cases with pulmonary lymphangitic carcinomatosis had associated enlarged mediastinal lymph nodes.
Renal medullary carcinoma in children and young adults presents at an advanced local and distant stage in the majority of patients. The diagnosis of renal medullary carcinoma should be considered when a child or young adult presents with a poorly defined/infiltrative, centrally located renal mass, especially in the setting of known sickle cell hemoglobinopathy. Distant metastases are common at initial presentation in the lungs, distant lymph nodes and liver and often involve multiple sites simultaneously. Pulmonary lymphangitic carcinomatosis, a distinctive and uncommon form of lung metastasis in children, is common in this patient population.
肾髓质癌是一种罕见的儿童期肾恶性肿瘤。目前尚无大量系列研究描述儿童肾髓质癌的影像学表现。
描述儿童肾髓质癌初诊时的临床和影像学特征。
我们回顾性分析了2006年3月至2016年8月参加儿童肿瘤学组肾肿瘤分类、生物学和库研究（AREN03B2）的25例儿童肾髓质癌患者的影像资料。评估原发肿块的影像学表现以及局部和远处转移的模式，并与病理和手术结果进行相关性分析。
初诊时的中位年龄为13岁（范围：6 - 21岁），男性占优势（3.2:1）。初诊时疾病的总体分期为1期1例，2期2例，4期22例。原发肾肿块的最大直径为1.6至10.3厘米（平均：6.6厘米），略偏向右侧（1.5:1）。20/25（80%）例患者在初始分期时发现腹膜后淋巴结肿大（短轴>1厘米），其中10例经组织学证实，其余未进行手术取样。纵隔（14/25；56%）和锁骨上区域（4/25；16%）也发现有肿大淋巴结。19/25（76%）例患者存在肺转移，6/25（24%）例患者存在肝转移。肺转移的模式为肺淋巴管癌病：10例（9例双侧，1例单侧），边缘不清的肺结节：6例，边缘清晰的肺结节：2例，1例既有边缘不清又有边缘清晰的肺结节。10例肺淋巴管癌病中有4例经病理证实。所有肺淋巴管癌病患者均伴有纵隔淋巴结肿大。
儿童和青年肾髓质癌在大多数患者中初诊时即处于局部晚期和远处转移阶段。当儿童或青年出现边界不清/浸润性、位于肾中央的肿块时，应考虑肾髓质癌的诊断，尤其是在已知镰状细胞血红蛋白病的情况下。远处转移在初诊时常见于肺、远处淋巴结和肝脏，且常同时累及多个部位。肺淋巴管癌病是儿童一种独特且不常见的肺转移形式，在该患者群体中较为常见。
