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以双侧肾上腺嗜铬细胞瘤为首发表现的原发性肾上腺淋巴瘤。

Primary adrenal lymphoma with initial presentation concerning for bilateral adrenal pheochromocytomas.

作者信息

Joseph Flavian Grace, Cook Sheila, Gowda Durgesh

机构信息

Department of Endocrinology, Toowoomba Health Service, Toowoomba, Queensland, Australia.

出版信息

BMJ Case Rep. 2017 Aug 22;2017:bcr-2017-220549. doi: 10.1136/bcr-2017-220549.

Abstract

Primary adrenal non-Hodgkin's lymphoma is rarely encountered. Clinical presentation is non-specific with fatigue, abdominal pain and B-symptoms being more commonly reported. We report a case of primary bilateral adrenal lymphoma who initially presented with clinical features suspicious for pheochromocytoma. The patient was recently diagnosed with hypertension and had a family history of early ischaemic heart disease. Plasma free metanephrines were also elevated. Pheochromocytoma was deemed unlikely following multiple investigations and the diagnosis of lymphoma was made following adrenal biopsy. Partial response was noted on positron emission tomography CT scan following four cycles of chemotherapy but failed to remit after six cycles of chemotherapy. Subsequently, he received 20 fractions of radiation to his adrenal glands and is now awaiting further imaging. The presentation of primary adrenal lymphoma can be variable and given that the diagnosis is obtained through tissue sampling, pheochromocytoma should be excluded due to risk of a potentially fatal adrenergic crisis.

摘要

原发性肾上腺非霍奇金淋巴瘤很少见。临床表现无特异性,更常报告的症状有疲劳、腹痛和B症状。我们报告一例原发性双侧肾上腺淋巴瘤病例,该患者最初表现出疑似嗜铬细胞瘤的临床特征。该患者最近被诊断为高血压,并有早发性缺血性心脏病家族史。血浆游离甲氧基肾上腺素也升高。经过多项检查后,嗜铬细胞瘤的可能性被排除,肾上腺活检后确诊为淋巴瘤。化疗四个周期后,正电子发射断层扫描CT显示部分缓解,但化疗六个周期后未能缓解。随后,他接受了20次肾上腺放疗,目前正在等待进一步的影像学检查。原发性肾上腺淋巴瘤的表现可能多种多样,鉴于诊断是通过组织取样获得的,由于存在潜在致命性肾上腺素能危象的风险,应排除嗜铬细胞瘤。

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