RENAL MEDULLARY CARCINOMA - THE 7TH SICKLE-CELL NEPHROPATHY
RENAL MEDULLARY CARCINOMA - THE 7TH SICKLE-CELL NEPHROPATHY
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DOI：
10.1097/00000478-199501000-00001
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发表时间：
1995-01-01
影响因子：
5.6
通讯作者：
SESTERHENN, IA
中科院分区：
文献类型：
--
作者：
DAVIS, CJ;MOSTOFI, FK;SESTERHENN, IA
在过去的22年中，我们遇到了34例高度侵袭性肿瘤，其显微形态高度预测组织中发现镰状红细胞。除一名患者外，所有患者均被认为患有镰状细胞病，或者在一个病例中患有血红蛋白SC病。这33名患者是本报告的主题，在他们的种族已知的情况下，他们都是11至39岁的黑人。在11至24岁年龄段，男性占多数，比例为3比1。然而，超过24岁，肿瘤在男性和女性中的发生率相同。肿瘤主要位于髓质，直径4 ~ 12 cm。平均尺寸为7 cm;中位数为6 cm。肾皮质和盆腔软组织中的外周卫星，以及静脉和淋巴侵犯，通常存在。病变表现为网状、卵黄囊样或腺样囊性外观，通常在高度促纤维增生性间质中存在低分化区域，伴嗜中性粒细胞，通常由淋巴细胞边缘化。肿瘤在首次发现时通常已经转移，在肾切除术时没有一个局限于肾。术后平均生存时间为15周。这些肿瘤可能发生在肾乳头内或其附近的肾盏上皮，在镰状细胞特征的患者中，相同的部位产生更熟悉的单侧血尿。我们的结论是肾髓样癌代表了镰状细胞病相关的肾脏疾病的另一个例子。其他六种是单侧血尿、乳头坏死、肾病综合征、肾梗塞、不能浓缩尿液和肾盂肾炎。
Over the last 22 years, we have encountered 34 examples of a highly aggressive neoplasm with a microscopic morphology that is highly predictive of finding sickled erythrocytes in the tissue. With the exception of one patient, all are believed to have had sickle cell trait or, in one case, hemoglobin SC disease. These 33 patients are the subject of this report and, where their race was known, they were all blacks between the ages of 11 and 39 years. Between the ages of 11 and 24 years, males predominated by 3 to 1. Beyond age 24, however, the tumors occurred equally in men and women. The dominant tumor mass was in the medulla and ranged from 4 to 12 cm in diameter. Mean size was 7 cm; median, 6 cm. Peripheral satellites in the renal cortex and pelvic soft tissues, as well as venous and lymphatic invasion, were usually present. The lesions exhibited a reticular, yolk sac-like, or adenoid cystic appearance, often with poorly differentiated areas in a highly desmoplastic stroma admired with neutrophils and usually marginated by lymphocytes. The tumors had usually metastasized when first discovered, and none was confined to the kidney at the time of nephrectomy. The mean duration of life after surgery was 15 weeks. These tumors probably arise in the calyceal epithelium in or near the renal papillae, the same site that produces the more familiar picture of unilateral hematuria in patients with sickle cell trait. We have concluded that renal medullary carcinoma represents another example of renal disease associated with sickle cell disorders. The other six are unilateral hematuria, papillary necrosis, nephrotic syndrome, renal infarction, inability to concentrate urine, and pyelonephritis.