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一名患有镰状细胞贫血的青少年的肉瘤样肾细胞癌。

Sarcomatoid Renal Cell Carcinoma in an Adolescent with Sickle Cell Anaemia.

作者信息

Ahmad H R, Faruk J A, Bugaje M A, Solomon A, Samaila M O A, Akuse R M

机构信息

Haematology/Oncology Unit, Department of Paediatrics, Ahmadu Bello University/Teaching Hospital, Zaria, Nigeria.

Nephrology Unit, Department of Paediatrics, Ahmadu Bello University/Teaching Hospital, Zaria, Nigeria.

出版信息

Case Rep Oncol Med. 2017;2017:2129450. doi: 10.1155/2017/2129450. Epub 2017 Oct 31.

DOI:10.1155/2017/2129450
PMID:29225982
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5684584/
Abstract

Malignancies have been reported to occur in people with sickle cell disease. Renal medullary carcinoma (RMC), also tagged seventh sickle cell nephropathy, is an aggressive cancer seen almost exclusively in people with sickle cell disease with more than 160 cases reported worldwide, but only few cases were reported in patients with sickle cell anaemia (HBSS) and from Nigeria. Sarcomatoid renal cell carcinoma is a renal tumour of any histologic variant containing foci of high-grade malignant spindle cells. We report an adolescent girl with sickle cell anaemia (HBSS) who presented with left renal tumour, histology of which confirmed a diagnosis of sarcomatoid renal cell carcinoma (sRCC). Surgical debulking and palliative care with chemotherapy were given, and she demised 10 months after. The rarity of the case and challenges of managing a cancer in the background of a chronic haematologic disorder are highlighted.

摘要

据报道,镰状细胞病患者会发生恶性肿瘤。肾髓质癌(RMC),也被称为第七种镰状细胞肾病,是一种侵袭性癌症,几乎仅见于镰状细胞病患者,全球报告了160多例,但镰状细胞贫血(HBSS)患者及来自尼日利亚的患者中报告的病例很少。肉瘤样肾细胞癌是一种具有高级别恶性梭形细胞灶的任何组织学变体的肾肿瘤。我们报告一名患有镰状细胞贫血(HBSS)的青少年女孩,她出现左肾肿瘤,其组织学证实诊断为肉瘤样肾细胞癌(sRCC)。给予了手术减瘤和化疗姑息治疗,她在10个月后死亡。强调了该病例的罕见性以及在慢性血液系统疾病背景下管理癌症的挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a925/5684584/1c57319ae747/CRIONM2017-2129450.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a925/5684584/c91297861cb1/CRIONM2017-2129450.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a925/5684584/78d46e4e9a91/CRIONM2017-2129450.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a925/5684584/1c57319ae747/CRIONM2017-2129450.003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a925/5684584/c91297861cb1/CRIONM2017-2129450.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a925/5684584/78d46e4e9a91/CRIONM2017-2129450.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a925/5684584/1c57319ae747/CRIONM2017-2129450.003.jpg

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本文引用的文献

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Is renal medullary carcinoma the seventh nephropathy in sickle cell disease? A multi-center Nigerian survey.肾髓质癌是镰状细胞病的第七种肾病吗?一项尼日利亚多中心调查。
Afr Health Sci. 2016 Jun;16(2):490-6. doi: 10.4314/ahs.v16i2.17.
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Renal medullary carcinoma.肾髓质癌
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