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伴有未诊断出的镰状细胞性状的肾髓质癌的不寻常表现。

Unusual Presentation of Renal Medullary Carcinoma With Undiagnosed Sickle Cell Trait.

作者信息

Basher Fahmin, Dutcher Giselle, England Jonathan S, Lopes Gilberto

机构信息

Division of General Internal Medicine, Department of Medicine, University of Miami Miller School of Medicine, Jackson Memorial Hospital, Miami, USA.

Divisions of Hematology and Medical Oncology, Department of Medicine, University of Miami Miller School of Medicine, Jackson Memorial Hospital, Miami, USA.

出版信息

Cureus. 2020 Sep 30;12(9):e10731. doi: 10.7759/cureus.10731.

Abstract

Renal medullary carcinoma (RMC) is an extremely rare malignancy that has been described in younger male patients of African descent with a history of sickle cell disease or trait. We describe a rather unique case of RMC in an older male patient who initially presented with acute on chronic urinary retention and concern for infection. Further investigation revealed a history of hematuria and long-standing microcytic anemia, and the patient was found to have sickle cell trait (SCT) as part of a workup for malignancy of unknown primary. Imaging findings initially interpreted as hydronephrosis later characterized a mass in the renal pelvis concerning for a genitourinary malignancy, later biopsy-proven RMC. RMC typically presents in its advanced stages, with associated poor prognosis, and treatment options are limited and have been extrapolated from standard regimens for other genitourinary malignancies. Therefore, early clinical suspicion in patients with microcytic anemia, flank pain, hematuria, and urinary symptoms, can aid in the diagnosis of RMC and allow for prompt intervention.

摘要

肾髓质癌(RMC)是一种极其罕见的恶性肿瘤,多见于有镰状细胞病或特征病史的非洲裔年轻男性患者。我们描述了一例较为独特的RMC病例,患者为老年男性,最初表现为急性慢性尿潴留并伴有感染问题。进一步检查发现有血尿病史和长期小细胞贫血,该患者在对不明原发恶性肿瘤的检查中被发现有镰状细胞特征(SCT)。最初被解释为肾积水的影像学检查结果后来显示肾盂有一个肿块,怀疑是泌尿生殖系统恶性肿瘤,后来经活检证实为RMC。RMC通常在晚期出现,预后较差,治疗选择有限,且是从其他泌尿生殖系统恶性肿瘤的标准方案推断而来。因此,对于有小细胞贫血、侧腹痛、血尿和泌尿系统症状的患者,早期临床怀疑有助于RMC的诊断并能及时进行干预。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cf19/7599047/c926564b177f/cureus-0012-00000010731-i01.jpg

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