IgG4-related disease mimicking pancreatic cancer: Case report and review of the literature.
IgG4-related disease mimicking pancreatic cancer: Case report and review of the literature.
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DOI：
10.1016/j.ijscr.2018.07.030
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发表时间：
2018
影响因子：
0.6
通讯作者：
Khalaf H
中科院分区：
文献类型：
--
作者：
Sulieman I;Mahfouz A;AlKuwari E;Szabados L;Elmoghazy W;Elaffandi A;Khalaf H
胰腺肿块的诊断是一个挑战，癌症必须始终考虑。IgG 4相关疾病是胰腺肿块的罕见原因。如果组织病理学检查证实了诊断，胰腺活检并不总是需要的。多器官受累和腹膜炎应引起IgG 4相关疾病的怀疑。当怀疑为良性病变时，大多数胰腺肿块患者的诊断具有挑战性，并且通常需要在良性诊断得到确认之前进行切除。患者男，57岁，因胰头肿块、梗阻性黄疸及颌下淋巴结肿大就诊。他还有复发性眼痛和发红、皮肤病变和良性前列腺肥大的病史。MRI显示胰头肿块伴双管征、主动脉增厚、双侧肾脏病变、弥漫性淋巴结肿大和前列腺肿大。FDG-PET/CT显示与MRI病变对应的异常摄取，血液检查中IgG 4水平升高。腹股沟淋巴结活检显示IgG 4浆细胞浸润，与IgG 4疾病的诊断一致。患者接受IV类固醇治疗，并显示出显著改善。IgG 4相关疾病是一种罕见的实体，其特征在于病变显示IgG 4阳性浆细胞的严重浸润、风暴状纤维化和闭塞性静脉炎。胰腺是最常累及的器官，但也累及其他几个器官系统，这有助于临床怀疑诊断。从任何容易接近的部位进行活检，显示出典型的组织学特征，就足以诊断。患者对类固醇反应迅速，但复发频繁。IgG 4相关疾病是胰腺肿瘤性病变的罕见原因，需要高度怀疑诊断，并应与胰腺肿瘤性病变相鉴别。
Pancreatic masses pose a diagnostic challenge, and cancer has to be always considered. IgG4-related disease is a rare cause of pancreatic masses. Biopsy from the pancreas is not always required if histopathology from a more accessible peripheral site lesion confirms the diagnosis. Multiorgan involvement and aortitis should raise suspicion of IgG4-related disease. Most patients with pancreatic masses pose a diagnostic challenge when a benign lesion is suspected, and often, resection is needed before a benign diagnosis is confirmed. A 57 years old male patient presented with a pancreatic head mass, obstructive jaundice and submandibular lymph node enlargement. He also had a history of recurrent eye pain and redness, skin lesions, and benign prostatic hypertrophy. MRI showed a pancreatic head mass with double duct sign, aortic thickening, bilateral renal lesions, diffuse lymph node enlargement, and prostatic enlargement. FDG-PET/CT demonstrated abnormal uptake corresponding to the MRI lesions, and there were elevated IgG4 levels on blood investigations. Biopsy of an inguinal lymph node revealed infiltrates with IgG4 plasma cells, consistent with the diagnosis of IgG4 disease. The patient was treated with IV steroids and showed significant improvement. IgG4 related disease is a rare entity that is characterized by lesions that show heavy infiltration with IgG4 positive plasma cells, storiform fibrosis, and obliterative phlebitis. The pancreas is the most commonly involved organ, but several other organ systems are involved, and this helps in clinical suspicion of the diagnosis. A biopsy from any easily accessible site that shows the characteristic histological features is sufficient for diagnosis. Patients respond quickly to steroids, but recurrence is frequent. IgG4 related disease is a rare cause of pancreatic tumorous lesions that need a high index of suspicion for diagnosis and should be differentiated from pancreatic neoplastic lesions.