Matsuo Toshihiko, Tanaka Takehiro, Fuji Tomokazu, Ennishi Daisuke
Department of Ophthalmology, Graduate School of Interdisciplinary Science and Engineering in Health Systems, Okayama University, Okayama, JPN.
Department of Opthalmology, Okayama University Hospital, Okayama, JPN.
Cureus. 2024 Nov 15;16(11):e73775. doi: 10.7759/cureus.73775. eCollection 2024 Nov.
Castleman disease is a lymphadenopathy of unknown cause at a single site, which is designated as unicentric Castleman disease, or at multiple sites designated as multicentric Castleman disease. We present a patient who showed axillary reactive lymphoid hyperplasia, likely due to unicentric Castleman disease, and orbital extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma in a six-year follow-up. A 76-year-old man had a painless left axillary mass for an unknown period and also left complete blepharoptosis with no other systemic symptoms. Suspected of lymphoma, iliac bone marrow biopsy showed no anomalous cells, and positron emission tomography demonstrated abnormal uptake at the left axilla and in the left superior anterior orbit. Incisional biopsy of the left axillary mass demonstrated hyperplastic lymphoid follicles with an atrophic germinal center and prominent small vessels in the follicular center, indicative of unicentric Castleman disease. One year later, annual follow-up positron emission tomography disclosed a high uptake site, next to the previously-identified cyst, in the pancreatic body. Trans-gastric fine needle pancreatic biopsy proved adenocarcinoma and he underwent subtotal stomach-preserving pancreaticoduodenectomy with jejunal anastomosis. He was well for six months after the surgery and thus, underwent resection of the left orbital lesion at 78 years old. The pathology of the orbital lesion showed ambiguous nodular structure with massive infiltration with CD20-positive medium-sized lymphoid cells which were κ monotype in immunoglobulin light chain restriction, indicative of MALT lymphoma. In the four-year period of the COVID-19 pandemic, he was healthy and followed with no treatment until the age of 82 years when he underwent radiation (46 Gy) to the left axillary lesion which did not regress. He then underwent eyelid levator muscle plication for left blepharoptosis since the left orbital lesion remained unpalpable. The six-year follow-up showed that concurrent and independent orbital MALT lymphoma and axillary reactive lymphoid hyperplasia, likely due to unicentric Castleman disease, were both stable. The present case illustrates how important it is to make pathological diagnoses in different anatomical lesions after the initial diagnosis of Castleman disease.
卡斯特leman病是一种病因不明的淋巴结病，单个部位发病者称为单中心卡斯特leman病，多个部位发病者称为多中心卡斯特leman病。我们报告一例患者，在六年的随访中，表现为腋窝反应性淋巴组织增生，可能是由于单中心卡斯特leman病所致，同时还患有眼眶结外黏膜相关淋巴组织边缘区B细胞淋巴瘤（MALT淋巴瘤）。一名76岁男性左侧腋窝有无痛性肿块，持续时间不详，同时伴有左侧完全性上睑下垂，无其他全身症状。怀疑为淋巴瘤，髂骨骨髓活检未发现异常细胞，正电子发射断层扫描显示左侧腋窝和左侧眶上前部摄取异常。左侧腋窝肿块切除活检显示淋巴滤泡增生，生发中心萎缩，滤泡中心小血管明显，提示单中心卡斯特leman病。一年后，年度随访正电子发射断层扫描显示胰体部有一个高摄取部位，紧邻先前发现的囊肿。经胃细针穿刺胰腺活检证实为腺癌，他接受了保留部分胃的胰十二指肠切除术并进行空肠吻合。术后六个月他情况良好，因此在78岁时接受了左侧眼眶病变切除术。眼眶病变的病理显示结节结构不明确，有大量CD20阳性中等大小淋巴细胞浸润，免疫球蛋白轻链限制为κ单型，提示MALT淋巴瘤。在新冠疫情的四年期间，他身体健康，未接受治疗，直至82岁时，对未消退的左侧腋窝病变进行了46 Gy的放疗。由于左侧眼眶病变仍无法触及，随后他接受了左侧上睑下垂的提上睑肌折叠术。六年的随访显示，同时存在且独立的眼眶MALT淋巴瘤和可能由单中心卡斯特leman病引起的腋窝反应性淋巴组织增生均稳定。本病例说明了在卡斯特leman病初步诊断后，对不同解剖部位病变进行病理诊断的重要性。
