Zeng Jiafei, Li Jin, Luo Shuai, Wang Jinjing
Department of Pathology, Affiliated Hospital of Zunyi Medical University, Zunyi City, Guizhou Province, People's Republic of China.
World J Surg Oncol. 2025 May 23;23(1):200. doi: 10.1186/s12957-025-03855-6.
Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma, predominantly affecting the elderly. The disease often involves multiple tissues and organs, leading to a wide range of nonspecific symptoms. Early detection is challenging due to the absence of prominent signs such as enlarged lymph nodes or significant blood and bone marrow abnormalities in the early stages. Moreover, biopsy specimens may be insufficient for accurate diagnosis, resulting in a high false-negative rate and delayed treatment initiation.
A 61-year-old male presented with a bilateral adrenal mass detected during routine examination. The patient had a history of diabetes, managed with regular antidiabetic medications and good blood sugar control for over a year. Symptoms included dizziness and mild bilateral flank discomfort. Imaging revealed patchy, soft tissue lesions in both adrenal glands on an upper abdominal CT scan. The larger lesion measured 71 × 37 mm on the left, and 51 × 31 mm on the right. Enhanced imaging showed mild to moderate enhancement, with neoplastic lesions or metastasis suspected. A CT-guided percutaneous biopsy was performed. Histopathological examination revealed large or medium-sized lymphoma-like cells localized to endothelial markers CD31 and CD34-positive lumen epithelium, with strong diffuse positivity for B-lymphocyte markers. The final diagnosis was IVLBCL. The patient was treated with rituximab combined with CHOP chemotherapy and discharged without significant symptoms.
IVLBCL is often overlooked due to its varied and nonspecific clinical presentation, making early diagnosis difficult. The disease progresses rapidly and has a poor prognosis, with definitive diagnosis relying on pathological findings. Increased awareness of this rare lymphoma is crucial, particularly for diagnosing needle biopsy specimens in patients with mild symptoms and no distinct space-occupying lesions. Careful morphological examination, supported by targeted immunohistochemical staining (for B-cell and endothelial markers), is essential for timely diagnosis.
血管内大B细胞淋巴瘤（IVLBCL）是弥漫性大B细胞淋巴瘤的一种罕见亚型，主要影响老年人。该疾病常累及多个组织和器官，导致出现广泛的非特异性症状。由于早期缺乏如淋巴结肿大或明显的血液及骨髓异常等突出体征，早期检测具有挑战性。此外，活检标本可能不足以进行准确诊断，导致假阴性率高且治疗开始延迟。
一名61岁男性在常规检查中发现双侧肾上腺肿块。患者有糖尿病史，使用常规抗糖尿病药物治疗，血糖控制良好已超过一年。症状包括头晕和双侧轻度胁腹不适。上腹部CT扫描显示双侧肾上腺有斑片状软组织病变。左侧较大病变大小为71×37mm，右侧为51×31mm。增强成像显示轻度至中度强化，怀疑为肿瘤性病变或转移。进行了CT引导下经皮活检。组织病理学检查显示，大或中等大小的淋巴瘤样细胞定位于内皮标志物CD31和CD34阳性的管腔上皮，B淋巴细胞标志物呈强弥漫阳性。最终诊断为IVLBCL。患者接受了利妥昔单抗联合CHOP化疗，出院时无明显症状。
IVLBCL因其多样且非特异性的临床表现常被忽视，导致早期诊断困难。该疾病进展迅速，预后不良，明确诊断依赖于病理结果。提高对这种罕见淋巴瘤的认识至关重要，特别是对于症状轻微且无明显占位性病变患者的针吸活检标本的诊断。在靶向免疫组织化学染色（针对B细胞和内皮标志物）支持下进行仔细的形态学检查对于及时诊断至关重要。
