Bulanov Dimitar, Arabadzhieva Elena, Bonev Sasho, Yonkov Atanas, Kyoseva Diana, Dikov Tihomir, Dimitrova Violeta
Department of General and Hepato-pancreatic Surgery, Medical University of Sofia, University Hospital "Alexandrovska", 1 Georgi Sofiiski Str, 1431, Sofia, Bulgaria.
Department of General and Clinical Pathology, Medical University of Sofia, 2 Zdrave Str, 1431, Sofia, Bulgaria.
BMC Surg. 2016 Jun 2;16(1):37. doi: 10.1186/s12893-016-0151-4.
IgG4-related disease (IgG4-RD) is a newly recognized disorder, characterized by massive IgG4+ lymphocyte and plasma cell infiltration, storiform fibrosis, causing enlargement, nodules or thickening of the various organs, simultaneously or metachronously. Involvement of the gastrointestinal tract is very rare and can be presented as a diffuse wall thickening or polyp or mass-like lesion. Up to now, there have been reported only a few cases of isolated gastric IgG4-RD.
We present an unusual case of IgG4-RD of the stomach with involvement of the regional lymph nodes, clinically manifested as a gastric cancer with related pyloric stenosis. The patient underwent distal gastrectomy, omentectomy and lymph node dissection. The postoperative serum IgG4 level was increased. The diagnosis was confirmed by immunohistochemical study.
In the most of the reported cases there was not sufficient data about the regional lymph nodal status, although the majority of the patients had been operated with presumptive diagnosis of gastric neoplasm. Our case is rare and valuable because it presents a gastric IgG4-related lesion larger than all previously reported in literature, and IgG4-related lymphadenopathy, confirmed histologically, which contributes to better knowledge of the disease.
IgG4相关性疾病（IgG4-RD）是一种新认识的疾病，其特征为大量IgG4+淋巴细胞和浆细胞浸润、席纹状纤维化，可导致各个器官同时或异时出现肿大、结节或增厚。胃肠道受累非常罕见，可表现为弥漫性壁增厚或息肉或肿块样病变。迄今为止，仅报道了少数孤立性胃IgG4-RD病例。
我们报告一例罕见的胃IgG4-RD病例，伴有区域淋巴结受累，临床表现为胃癌伴相关幽门狭窄。患者接受了远端胃切除术、网膜切除术和淋巴结清扫术。术后血清IgG4水平升高。通过免疫组织化学研究确诊。
在大多数报道的病例中，关于区域淋巴结状态的数据不足，尽管大多数患者接受手术时的初步诊断为胃肿瘤。我们的病例罕见且有价值，因为它呈现出一个比文献中先前报道的所有病例都大的胃IgG4相关病变，以及经组织学证实的IgG4相关淋巴结病，这有助于更好地了解该疾病。
