原发性肾上腺淋巴瘤作为肾上腺功能不全的病因：两例报告
Primary adrenal lymphoma as a cause of adrenal insufficiency, a report of two cases.
作者信息
Grønning Kaja, Sharma Archana, Mastroianni Maria Adele, Karlsson Bo Daniel, Husebye Eystein S, Løvås Kristian, Nermoen Ingrid
机构信息
Department of Endocrinology, Akershus University Hospital, Lorenskog, Norway.
Department of Haematology, Akershus University Hospital, Lorenskog, Norway.
出版信息
Endocrinol Diabetes Metab Case Rep. 2020 Mar 10;2020. doi: 10.1530/EDM-19-0131.
SUMMARY
Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. More than 90% is of B-cell origin. The condition is bilateral in up to 75% of cases, with adrenal insufficiency in two of three patients. We report two cases of adrenal insufficiency presenting at the age of 70 and 79 years, respectively. Both patients had negative 21-hydroxylase antibodies with bilateral adrenal lesions on CT. Biopsy showed B-cell lymphoma. One of the patients experienced intermittent disease regression on replacement dosage of glucocorticoids.
LEARNING POINTS
Primary adrenal lymphoma (PAL) is a rare cause of adrenal insufficiency. Bilateral adrenal masses of unknown origin or in individuals with suspected extra-adrenal malignancy should be biopsied quickly when pheochromocytoma is excluded biochemically. Steroid treatment before biopsy may affect diagnosis. Adrenal insufficiency with negative 21-hydroxylase antibodies should be evaluated radiologically.
摘要
原发性肾上腺淋巴瘤（PAL）是肾上腺功能不全的罕见病因。超过90%起源于B细胞。该病在高达75%的病例中为双侧性，三分之二的患者存在肾上腺功能不全。我们报告了分别在70岁和79岁时出现肾上腺功能不全的两例病例。两名患者21-羟化酶抗体均为阴性，CT显示双侧肾上腺病变。活检显示为B细胞淋巴瘤。其中一名患者在使用糖皮质激素替代剂量时经历了疾病的间歇性消退。
学习要点
原发性肾上腺淋巴瘤（PAL）是肾上腺功能不全的罕见病因。当通过生化检查排除嗜铬细胞瘤后，对于不明原因的双侧肾上腺肿块或疑似肾上腺外恶性肿瘤的个体，应尽快进行活检。活检前的类固醇治疗可能会影响诊断。21-羟化酶抗体阴性的肾上腺功能不全应进行影像学评估。
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