Pacyna Rachel R, Cipriani Nicole A, Mathew Melvy S, Kim Josephine S
Pritzker School of Medicine, The University of Chicago, 5841 S Maryland Ave, Chicago, IL 60637, USA.
Department of Anatomic Pathology Informatics, The University of Chicago, 5841 S Maryland Ave, Chicago, IL 60637, USA.
Gynecol Oncol Rep. 2023 Jan 18;45:101137. doi: 10.1016/j.gore.2023.101137. eCollection 2023 Feb.
Immunoglobulin G4 (IgG4) related disease is a systemic disease that causes fibrosis, tumor-like nodules, and lymphoid hyperplasia with infiltration of IgG4 positive plasma cells. It can manifest in many organ systems; however, there are few cases that report gynecologic organ involvement. It is crucial to correctly diagnose IgG4-related disease versus malignancy because the former is treated with glucocorticoids or rituximab. In this case report, we describe two patients in which IgG4-related disease mimics gynecologic cancer. In the first case, an 85 year old woman presented with diffuse lymphadenopathy and a uterine mass concerning for malignancy. Biopsies were negative for carcinoma. Inguinal lymph node biopsy demonstrated IgG4 positive plasma cells and the patient was treated with rituximab therapy given concurrent severe rheumatoid arthritis. In the second case, a 35 year old woman under surveillance for Stage IB2 squamous cell carcinoma of the cervix (status post definitive chemoradiation therapy) presented with fluorodeoxyglucose (FDG) avid paraaortic lymph nodes on positron emission tomography (PET) imaging with subsequent negative paraaortic lymph node biopsies. Serial imaging and biopsies remained inconclusive despite ongoing diffuse lymphadenopathy and clinical concern for recurrence. Supraclavicular lymph node excision was performed which demonstrated lymphoid hyperplasia with increased IgG4 plasma cells and no evidence of carcinoma, supporting the diagnosis of IgG4-related disease. The patient was treated with high dose steroids with clinical improvement and resolution of abnormal imaging findings. We demonstrate that IgG4-related disease can present with FDG-avid lesions on PET imaging and lymphadenopathy that mimics primary or recurrent gynecologic malignancy. While rare, we conclude the IgG4-related disease is an important differential diagnosis to consider in the workup of primary or recurrent gynecologic malignancy and highlights the value of PET imaging to identify unusual patterns of lymphadenopathy and guide histologic confirmation of disease.
免疫球蛋白G4（IgG4）相关疾病是一种全身性疾病，可导致纤维化、肿瘤样结节以及IgG4阳性浆细胞浸润的淋巴组织增生。它可累及多个器官系统；然而，报道妇科器官受累的病例较少。正确诊断IgG4相关疾病与恶性肿瘤至关重要，因为前者采用糖皮质激素或利妥昔单抗治疗。在本病例报告中，我们描述了两例IgG4相关疾病酷似妇科癌症的患者。第一例，一名85岁女性，表现为弥漫性淋巴结病和可疑恶性的子宫肿块。活检未发现癌细胞。腹股沟淋巴结活检显示IgG4阳性浆细胞，鉴于患者同时患有严重类风湿关节炎，给予利妥昔单抗治疗。第二例，一名35岁女性，因IB2期宫颈鳞状细胞癌（确诊后接受同步放化疗）接受监测，正电子发射断层扫描（PET）成像显示氟脱氧葡萄糖（FDG）摄取增加的主动脉旁淋巴结，随后主动脉旁淋巴结活检为阴性。尽管持续存在弥漫性淋巴结病且临床怀疑复发，但系列成像和活检结果仍不明确。进行了锁骨上淋巴结切除术，结果显示淋巴组织增生伴IgG4浆细胞增多，未发现癌细胞，支持IgG4相关疾病的诊断。该患者接受了高剂量类固醇治疗，临床症状改善，异常影像学表现消失。我们证明，IgG4相关疾病可在PET成像上表现为FDG摄取增加的病变以及酷似原发性或复发性妇科恶性肿瘤的淋巴结病。虽然罕见，但我们得出结论，IgG4相关疾病是原发性或复发性妇科恶性肿瘤检查中需要考虑的重要鉴别诊断，强调了PET成像在识别异常淋巴结病模式和指导疾病组织学确诊方面的价值。
