原发性肾上腺淋巴瘤的临床诊治研究

Clinical Diagnosis and Treatment Study of Primary Adrenal Lymphoma

摘要: 目的：总结原发性肾上腺淋巴瘤(Primary Adrenal Lymphoma, PAL)的临床特点、诊治资料，提高对肾上腺淋巴瘤的临床诊治研究。方法：回顾性分析2009年1月至2021年12月安徽医科大学第二附属医院收治的6例PAL患者的临床症状、影像学特征、病理结果及治疗过程。结果：共收治6例原发性肾上腺淋巴瘤患者，男女比例为3:1，年龄40~77岁。其中5例存在乏力、盗汗、发热、体重下降等症状，仅1例无明显临床症状。6例患者乳酸脱氢酶及β-2微球蛋白均高于正常值；4例患者存在肾上腺皮质功能不全。影像学均表现为双侧肾上腺占位，超声引导下穿刺或经腹腔镜肾上腺切除活检病理确诊。经病理诊断确诊为弥漫性大B细胞淋巴瘤3例；ALK阴性的间变性大细胞淋巴瘤1例；血管内大B细胞淋巴瘤1例(例6)；结外NK/T细胞淋巴瘤1例。弥漫性大B细胞淋巴瘤一线治疗以CHOP联合利妥昔单抗为主，可结合鞘内注射甲氨蝶呤(MTX)预防和治疗中枢系统淋巴瘤。结论：原发性肾上腺淋巴瘤患者以双侧肾上腺病变为主，无特殊临床症状，多表现为恶病质和肾上腺皮质功能不全等全身症状。CT和PET-CT有助于该病的鉴别诊断及分级分期。确诊需依赖于穿刺活检或手术切除活检病理诊断，病理以弥漫性大B细胞淋巴瘤为主；该病侵袭性高、预后差，联合利妥昔单抗的CHOP联合化疗较为有效。

Abstract: Objective: To summarize the clinical characteristics, diagnosis and treatment data of primary adrenal lymphoma (PAL) and to improve the clinical diagnosis and treatment research of adrenal lymphoma. Methods: We collected and retrospectively analyzed clinical symptoms, imaging features, pathological results, and treatment processes of 6 patients with PAL admitted to the Second Affiliated Hospital of Anhui Medical University from January 2009 to December 2021. Results: A total of 6 patients with primary adrenal lymphoma were treated, with a male-to-female ratio of 3:1 and an age range of 40 to 77 years. Five cases presented with symptoms such as fatigue, night sweats, fever, and weight loss, while only 1 case showed no obvious clinical symptoms. In all 6 cases, lactate dehydrogenase and β-2 microglobulin levels were elevated. Four patients exhibited adrenal cortical insufficiency. Imaging studies revealed bilateral adrenal masses, and pathological diagnosis was confirmed by ultrasound-guided puncture or laparoscopic adrenalectomy. Pathological diagnoses included 3 cases of diffuse large B-cell lymphoma, 1 case of ALK-negative anaplastic large cell lymphoma, 1 case of intravascular large B-cell lymphoma, and 1 case of extranodal NK/T-cell lymphoma. First-line treatment for diffuse large B-cell lymphoma consisted mainly of CHOP combined with rituximab, which could be supplemented with intrathecal injection of methotrexate (MTX) for the prevention and treatment of central nervous system lymphoma. Conclusion: Primary adrenal lymphoma predominantly manifests as bilateral adrenal lesions without specific clinical symptoms, often presenting as systemic symptoms such as cachexia and adrenal cortical insufficiency. CT and PET-CT are helpful for differential diagnosis, staging, and grading of this disease. Diagnosis depends on pathological diagnosis through puncture biopsy or surgical resection biopsy, with diffuse large B-cell lymphoma being the main pathological type. This disease is highly invasive with a poor prognosis, but CHOP combined chemotherapy with rituximab is relatively effective.