原发性双侧肾上腺非霍奇金伯基特样淋巴瘤：原发性肾上腺功能不全的罕见病因。病例报告及文献复习

Dobrinja Chiara, Trevisan Giuliano, Liguori Gennaro

Istituto di Clinica Chirurgica Generale e Terapia Chirurgica, Università degli Studi di Trieste, Trieste, Italy.

Tumori. 2007 Nov-Dec;93(6):625-30. doi: 10.1177/030089160709300621.

AIMS AND BACKGROUND

Primary bilateral adrenal non-Hodgkin's lymphoma is an extremely rare entity. Only 44 cases have been reported in the literature. The most common presenting symptoms are abdominal pain, fever, asthenia, constipation, weight loss or typical symptoms of adrenal insufficiency, hypertension, darkening of skin, orthostatic hypotension or an addisonian crisis.

METHODS

The case is presented of a 57-year-old man suffering from primary bilateral adrenal lymphoma with symptoms of adrenal insufficiency syndrome associated with bilateral, stabbing lumbar pain and a palpable mass on the left side. Laboratory tests revealed a considerable increase in lactate dehydrogenase levels, adrenal insufficiency, and high corticotropin levels.

RESULTS

Abdominal CT scan showed two large adrenal masses. A CT-guided fine needle aspiration biopsy revealed a large B-cell non-Hodgkin's lymphoma. Combination chemotherapy according to the CHOP protocol with cyclophosphamide, doxorubicin, vincristine and prednisolone was initiated, which caused a slight reduction in size of the two adrenal masses. The patient underwent a bilateral adrenalectomy with almost complete excision of the tumors. Replacement therapy with cortisone acetate was initiated postoperatively. Adjuvant combination chemotherapy according to the CHOP protocol was started but renal failure gradually emerged and the treatment could not be completed. The patient died seven months after surgery due to acute bronchial pneumonia and progression of disease.

CONCLUSIONS

Primary bilateral adrenal non-Hodgkin's lymphoma mainly affects adult men. Diagnosis is based on histological examination. Whether associated or not with radiotherapy, chemotherapy is the most recommended treatment. Surgery, where possible, seems to lead to an increase in survival rates, but it is not possible to draw any definite conclusions on its effectiveness as yet.

目的与背景

原发性双侧肾上腺非霍奇金淋巴瘤是一种极其罕见的疾病。文献中仅报道了44例。最常见的症状是腹痛、发热、乏力、便秘、体重减轻或肾上腺功能不全的典型症状，如高血压、皮肤变黑、体位性低血压或肾上腺危象。

方法

本文报道了一名57岁男性，患有原发性双侧肾上腺淋巴瘤，伴有肾上腺功能不全综合征的症状，同时伴有双侧刺痛性腰痛和左侧可触及肿块。实验室检查显示乳酸脱氢酶水平显著升高、肾上腺功能不全以及促肾上腺皮质激素水平升高。

结果

腹部CT扫描显示双侧肾上腺有两个大肿块。CT引导下细针穿刺活检显示为大B细胞非霍奇金淋巴瘤。开始采用环磷酰胺、阿霉素、长春新碱和泼尼松龙的CHOP方案进行联合化疗，这使两个肾上腺肿块的大小略有缩小。患者接受了双侧肾上腺切除术，肿瘤几乎完全切除。术后开始使用醋酸可的松替代治疗。开始按照CHOP方案进行辅助联合化疗，但逐渐出现肾衰竭，治疗无法完成。患者在手术后七个月因急性支气管肺炎和疾病进展而死亡。