Calcifying fibrous tumor: an unrecognized IgG4-related disease?

Abstract Background Calcifying fibrous tumor (CFT) is a rare benign soft tissue lesion. Case presentation A 30-year-old woman was admitted to our hospital with complaints of epigastralgia. A 15-mm submucosal tumor was identified in the greater curvature of the superior body of the stomach by upper gastrointestinal endoscopy. Endoscopic ultrasonography revealed a hypoechoic lesion with an acoustic shadow consistent with calcification. Computed tomography showed a gastric tumor with calcification. A gastrointestinal stromal tumor was diagnosed, and gastric wedge resection was performed by laparoscopy and endoscopy cooperative surgery. On pathological examination, the tumor was identified to be a CFT. Postoperative serum IgG4 levels were 26.0 mg/dl, which supported the diagnosis of probable immunoglobulin G (IgG) 4-related disease, according to the comprehensive diagnostic criteria of IgG4-related disease. The patient was discharged on postoperative day 7 and remains well with no evidence of tumor recurrence for 2 years after resection. Conclusion We herein reported a patient with a gastric CFT suspected to be complicated with immunoglobulin G4-related disease that was successfully treated by laparoscopy and endoscopy cooperative surgery.

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Case report of isolated gastric IgG4-related lesion and series of literature review

Journal of Clinical Images and Medical Case Reports ◽

10.52768/2766-7820/1161 ◽

2021 ◽

Vol 2 (3) ◽

Author(s):

Xiang-Xi Ye ◽

◽

Tian-Hui Zou ◽

Juan Tan ◽

Zheng Wang ◽

...

Keyword(s):

Salivary Gland ◽

Gastrointestinal Stromal Tumors ◽

Gastrointestinal Involvement ◽

Stromal Tumors ◽

Related Disease ◽

Multiple Organs ◽

Igg4 Related Disease ◽

Immune Mediated ◽

Orbital Tissue ◽

Fibrous Tumor

IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disease that responds to glucocorticoids, which is gradually well known in recent years. It can involve multiple organs of patients, including pancreas, bile duct, gallbladder, salivary gland, orbital tissue, lung, liver, lacrimal gland, kidney, retroperitoneal, aorta, thyroid and lymph node, [1] in which gastrointestinal involvement is relatively rare [2]. Most of the known reports of isolated gastric IgG4-related lesions were accidental found (in physical examination or other site examination), and often treated as Gastrointestinal Stromal Tumors (GIST) lead to surgically resection. In addition, Calcified Fibrous Tumor (CFT) in gastrointestinal tract often occurs with submucosa [3] is histologically similar to IgG4-RD, [4] whether it belongs to IgG4-RD is still controversial.

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