
TOTAL VIEWS: 453
IgG4-related sclerosing disease (IgG4-RSD) is an uncommon autoimmune entity, manifesting as tumefactive lesions and fibrosis in a spectrum of organs, including the pancreas, biliary tract, and salivary glands. Involvement of the esophagus is exceedingly rare. We present herein a case of an invasive IgG4-related sclerosing esophagitis pseudotumor, confirmed through surgical intervention and pathological assessment. A 26-year-old female exhibited symptoms of dysphagia and significant weight loss over a six-month period. Esophagoscopy disclosed a mass-like lesion localized to the upper esophagus. Preoperative biopsies, performed on two occasions, identified squamous epithelial cells, pointing towards a mesenchymal tumor in the differential diagnosis. Following a comprehensive multidisciplinary consultation, surgical resection was elected owing to the lesion’s extensive nature and the patient’s pronounced symptoms. Histopathological examination substantiated the presence of an esophageal tumor with the hallmarks of invasive IgG4-related sclerosing esophagitis. Postoperative recovery was uneventful, and the patient has remained asymptomatic throughout the follow-up period. This case illustrates the necessity of including IgG4-related disease in the differential diagnosis of esophageal tumors and accentuates the significance of surgical management in symptomatic and invasive instances of IgG4-related sclerosing esophagitis pseudotumors.
IgG4-related disease; sclerosing esophagitis pseudotumor; surgical treatment; case report; literature review
[1] Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539-51.
[2] Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet.
2015;385(9976):1460-71.
[3] Perugino CA, Stone JH. IgG4-related disease: an update on pathophysiology and implications for clinical care. Nat Rev Rheumatol. 2020;16(12):702-14.
[4] Kamisawa T, Anjiki H, Egawa N, Nakajima H. Allergic manifestations in autoimmune pancreatitis. Eur J Gastroenterol Hepatol. 2009;21(10):1136-9.
[5] Lohr JM, Vujasinovic M, Rosendahl J, Stone JH, Beuers U. IgG4-related diseases of the digestive tract. Nat Rev Gastroenterol Hepatol. 2022;19(3):185-97.
[6] On W, Huggett MT. European Guideline on IgG4-related digestive disease: UEG and SGF evidence-based recommendations. Frontline Gastroenterol. 2022;13(2):171-4.
[7] Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012;25(9):1181-92.
[8] Obiorah I, Hussain A, Palese C, Bedayat A. IgG4-related disease involving the esophagus: a clinicopathological study. Dis Esophagus. 2017;30(12):1-7.
[9] Khosroshahi A, Wallace ZS, Crowe JL, Akamizu T, Azumi A, Carruthers MN, et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015;67(7):1688-99.
[10] Padniewski JJ, Thottam E, Nasr R. IgG4 sclerosing disease of the esophagus: a case-based review. Rheumatol Int. 2020;40(10):1733-7.
[11] Koizumi S, Kamisawa T, Kuruma S, Tabata T, Chiba K, Iwasaki S, et al. Organ Correlation in IgG4-Related Diseases. J Korean Med Sci. 2015;30(6):743-8.
Surgical Treatment of Invasive IgG4-related Sclerosing Esophagitis Inflammatory Pseudotumor: A Case Report and Literature Review
How to cite this paper: Weixin Liu, Xuefeng Zhang, Jiacong Wei, Ling Qi, Yong Li. (2026) Surgical Treatment of Invasive IgG4-related Sclerosing Esophagitis Inflammatory Pseudotumor: A Case Report and Literature Review. International Journal of Clinical and Experimental Medicine Research, 10(2), 165-169.
DOI: http://dx.doi.org/10.26855/ijcemr.2026.03.020