J Pathol Transl Med.  2020 May;54(3):258-262. 10.4132/jptm.2020.02.10.

Gastric IgG4-related disease presenting as a mass lesion and masquerading as a gastrointestinal stromal tumor

• Ramakrishna B
• Yewale R
• Vijayakumar K
• Radhakrishna P
• Ramakrishna BS

• KMID: 2501662
• DOI: http://doi.org/10.4132/jptm.2020.02.10

Abstract

IgG4-related disease of the stomach is a rare disorder, and only a few cases have been reported. We present two cases that were identified over a 2-month period in our center. Two male patients aged 52 and 48 years presented with mass lesion in the stomach, which were clinically thought to be gastrointestinal stromal tumor, and they underwent excision of the lesion. Microscopic examination revealed marked fibrosis, which was storiform in one case, associated with diffuse lymphoplasmacytic infiltration and an increase in IgG4-positive plasma cells on immunohistochemistry. Serum IgG4 level was markedly elevated. Although rare, IgG4-related disease should be considered in the differential diagnosis of gastric submucosal mass lesions.

Keyword

IgG4-related disease; Stomach; Autoimmune diseases; Gastrointestinal stromal tumors

Figure

• 

  Fig. 1. (A) Gastric wall with marked submucosal fibrosis and prominent lymphoid follicles. (B) Diffuse plasma cell rich inflammation. (C) Storiform fibrosis with diffuse inflammation and a lymphoid follicle. (D) Many immunoreactive IgG4-positive plasma cells.

Reference

1. Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012; 25:1181–92.

2. Koizumi S, Kamisawa T, Kuruma S, et al. Immunoglobulin G4-related gastrointestinal diseases, are they immunoglobulin G4-related diseases? World J Gastroenterol. 2013; 19:5769–74.

3. Woo CG, Yook JH, Kim AY, Kim J. IgG4-related disease presented as a mural mass in the stomach. J Pathol Transl Med. 2016; 50:67–70.

4. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012; 366:539–51.

5. Kamisawa T, Funata N, Hayashi Y, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003; 38:982–4.

6. Bulanov D, Arabadzhieva E, Bonev S, et al. A rare case of IgG4-related disease: a gastric mass, associated with regional lymphadenopathy. BMC Surg. 2016; 16:37.

7. Kim DH, Kim J, Park DH, et al. Immunoglobulin G4-related inflammatory pseudotumor of the stomach. Gastrointest Endosc. 2012; 76:451–2.

8. Chetty R, Serra S, Gauchotte G, Markl B, Agaimy A. Sclerosing nodular lesions of the gastrointestinal tract containing large numbers of IgG4 plasma cells. Pathology. 2011; 43:31–5.

9. Na KY, Sung JY, Jang JY, et al. Gastric nodular lesion caused by IgG4-related disease. Pathol Int. 2012; 62:716–8.

10. Baez JC, Hamilton MJ, Bellizzi A, Mortelé KJ. Gastric involvement in autoimmune pancreatitis: MDCT and histopathologic features. JOP. 2010; 11:610–3.

11. Zhang H, Jin Z, Ding S. Gastric calcifying fibrous tumor: a case of suspected immunoglobulin G4-related gastric disease. Saudi J Gastroenterol. 2015; 21:423–6.

12. Rollins KE, Mehta SP, O'Donovan M, Safranek PM. Gastric IgG4-related autoimmune fibrosclerosing pseudotumour: a novel location. ISRN Gastroenterol. 2011; 2011:873087.

13. Otsuka R, Kano M, Hayashi H, et al. Probable IgG4-related sclerosing disease presenting as a gastric submucosal tumor with an intense tracer uptake on PET/CT: a case report. Surg Case Rep. 2016; 2:33.

14. Cheong HR, Lee BE, Song GA, Kim GH, An SG, Lim W. Immunoglobulin G4-related inflammatory pseudotumor presenting as a solitary mass in the stomach. Clin Endosc. 2016; 49:197–201.

15. Skorus U, Kenig J, Mastalerz K. IgG4-related disease manifesting as an isolated gastric lesion- a literature review. Pol Przegl Chir. 2018; 90:41–5.

16. Chari ST. Diagnosis of autoimmune pancreatitis using its five cardinal features: introducing the Mayo Clinic’s HISORt criteria. J Gastroenterol. 2007; 42 Suppl 18:39–41.

17. Miyabe K, Zen Y, Cornell LD, et al. Gastrointestinal and extra-intestinal manifestations of IgG4-related disease. Gastroenterology. 2018; 155:990–1003.

18. Seo HS, Jung YJ, Park CH, Song KY, Jung ES. IgG4-related disease in the stomach which was confused with gastrointestinal stromal tumor (GIST): two case reports and review of the literature. J Gastric Cancer. 2018; 18:99–107.

19. Inoue D, Yoneda N, Yoshida K, et al. Imaging and pathological features of gastric lesion of immunoglobulin G4-related disease: A case report and review of the recent literature. Mod Rheumatol. 2019; 29:377–82.

20. Shinji A, Sano K, Hamano H, et al. Autoimmune pancreatitis is closely associated with gastric ulcer presenting with abundant IgG4-bearing plasma cell infiltration. Gastrointest Endosc. 2004; 59:506–11.