Rezkallah Emad Mofid Nassif, Hanna Ragai Sobhi, Elsaify Wael Magdy
General Surgery Department, James Cook University Hospital, Middlesbrough, England.
General Surgery Department, Faculty of Medicine, Assiut University, Assiut, Egypt.
Int J Endocrinol Metab. 2022 Aug 20;20(4):e128386. doi: 10.5812/ijem-128386. eCollection 2022 Oct.
Adrenal lymphoma is a rare condition which may occur in one of two forms; either as primary adrenal lymphoma (PAL), or secondary to a systemic lymphoma. Primary adrenal lymphoma is a very rare diagnosis and the most common histological pattern is diffuse large B-cell non‑Hodgkin lymphoma.
In this study, we represent two examples of adrenal lymphoma, primary and secondary. In addition, we have included a mini-review of the literature regarding this rare presentation.
We retrospectively reviewed all patients who were diagnosed with adrenal lymphoma in our hospital. We represent mainly the most two challenging cases where adrenal surgery was required to confirm the diagnosis. We have included a mini-review of the literature (PubMed data base: 1990 - 2020) on the clinical presentation and management of adrenal lymphoma cases.
Seventeen patients had adrenal lymphoma in our hospital; 16 of them had secondary involvement of the adrenal gland, while the last one had primary adrenal lymphoma. Patients with adrenal lymphoma mainly present with fever, lumbar pain, and/or symptoms of adrenal insufficiency. Primary adrenal lymphoma usually appears as heterogeneous complex large masses with low density on computerized tomography (CT) scan or magnetic resonance imaging (MRI); however, there is no pathognomonic features to diagnose PAL. The diagnosis is confirmed only with tissue biopsy. Chemotherapy is generally the standard treatment for lymphoma, while the role of surgery is limited.
The prognosis of these rare cases is generally poor with only about a third of patients achieving partial or complete remission following treatment.
肾上腺淋巴瘤是一种罕见疾病，可表现为两种形式之一，即原发性肾上腺淋巴瘤（PAL）或继发于全身性淋巴瘤。原发性肾上腺淋巴瘤是一种非常罕见的诊断，最常见的组织学类型是弥漫性大B细胞非霍奇金淋巴瘤。
在本研究中，我们展示了原发性和继发性肾上腺淋巴瘤的两个病例。此外，我们还对有关这种罕见表现的文献进行了简要综述。
我们回顾性分析了我院所有诊断为肾上腺淋巴瘤的患者。我们主要展示了最具挑战性的两例病例，这两例均需要进行肾上腺手术以确诊。我们还对1990年至2020年期间关于肾上腺淋巴瘤病例临床表现及治疗的文献（PubMed数据库）进行了简要综述。
我院有17例肾上腺淋巴瘤患者，其中16例为肾上腺继发性受累，最后1例为原发性肾上腺淋巴瘤。肾上腺淋巴瘤患者主要表现为发热、腰痛和/或肾上腺功能不全症状。原发性肾上腺淋巴瘤在计算机断层扫描（CT）或磁共振成像（MRI）上通常表现为密度较低的不均匀复杂大肿块；然而，没有诊断PAL的特异性特征。只有通过组织活检才能确诊。化疗通常是淋巴瘤的标准治疗方法，而手术的作用有限。
这些罕见病例的预后通常较差，只有约三分之一的患者在治疗后实现部分或完全缓解。
