A rare case of immune-related pseudotumor of ovary mimicking ovarian malignancy





IgG4-RD, Immunoglobulin G4, IHC, Lymphoplasmacytic infiltration, Ovary


IgG4-related disease (IgG4-RD) is a chronic inflammatory condition affecting various body organs. However, genital tract involvement is rarely reported. A 40 years old reproductive-age woman presented with a solid abdominopelvic mass of 20 weeks size. CECT revealed a large multiloculated solid cystic lesion arising from right adnexa with multiple enlarged lymph nodes and omental thickening with gross right-sided hydroureteronephrosis suggestive of ovarian malignancy stage III. Surprisingly, tumor markers were normal. Intraoperatively, we noticed a 15×15 cm right ovarian mass encasing the right ureter all around and constricting it. The mass was densely adherent to recto-sigmoid and right pelvic wall, which mandated extensive surgery. Histopathological examination with immuno-histo-chemistry (IHC) suggested the diagnosis of IgG4-RD of ovary. Awareness about its occurrence in ovary will help in arriving at the diagnosis which may influence the extent of surgery. Lymphoplasmacytic infiltration with fibrosis in histopathological examination warrants IHC analysis for achieving a diagnosis.


Khosroshahi A, Wallace ZS, Crowe JL, Akamizu T, Azumi A, Carruthers MN et al. Second International Symposium on IgG4-Related Disease. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015;67:1688-99.

Martínez-Valle F, Orozco-Gálvez O, Fernández-Codina A. Update in ethiopathogeny, diagnosis and treatment of the IgG4 related disease. Med Clin (Barc). 2018;151:18-25.

Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD). Mod Rheumatol. 2012;2:21-30.

Maruyama S, Sato Y, Taga A, Emoto I, Shirase T, Haga H et al. Immunoglobulin G4-related disease presenting as bilateral ovarian masses and mimicking advanced ovarian cancer. J Obstet Gynaecol Res 2016;42:103-8.

Sekulic M, Pichler Sekulic S, Movahedi-Lankarani S. IgG4-related disease of the ovary. A first description. Int J Gynecol Pathol. 2017;3:190-4.

Akyol S, Atalay FÖ, Hasdemir S, Yerci Ö. IgG4-Related Disease of the Ovary. Turk Patoloji Derg. 2021;37(1):63-6.

Alorjani MS, Obeidat NA, Ababneh EI, Salem AA, Matalka II. A 47-Year-Old Woman with Immunoglobulin G4 (IgG4)-Related Disease Involving the Right Ovary. Am J Case Rep. 2020;21:e926803.

Sim Y, Chung T, Jung DC, Kim HS, Oh YT. Immunoglobulin G4-Related Disease of the Ovary Mimicking Bilateral Ovarian Malignancies. J Korean Soc Radiol. 2020;81:996-1002.

Ohkubo H, Miyazaki M, Oguri T, Arakawa A, Kobashi Y, Niimi A. A rare case of IgG4-related disease involving the uterus. Rheumatology (Oxford). 2015;54(6):1124-5.

Senda Y, Ikeda Y, Tamauchi S. A uterine pseudotumor of immunoglobulin G4-related disease. J Obstet Gynaecol Res. 2021;47(1):430-5.

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

Kamisawa T, Shimosegawa T, Okazaki K, T Nishino, H Watanabe, A Kanno et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009;58(11):1504-7.

Tacelli M, Celsa C, Magro B, Barresi L, Guastella S, Capurso G, et al. Risk Factors for Rate of Relapse and Effects of Steroid Maintenance Therapy in Patients With Autoimmune Pancreatitis: Systematic Review and Meta-analysis. Clin Gastroenterol Hepatol. 2019;17(6):1061-72.

Watanabe A, Goto T, Kamo H, Komine R, Kuroki N, Sugase T et al. Resection of lesions in the ileum of patients with IgG4-related disease may ameliorate disease progression without steroid administration. Surg Case Rep. 2018;4(1):148.

Hiyoshi Y, Oki E, Zaitsu Y, Ando K, Ito S, Saeki H et al. IgG4-related disease of the ileocecal region mimicking malignancy: A case report. Int J Surg Case Rep. 2014;5(10):669-72.

Seki N, Yamazaki N, Kondo A, Nomura K, Himi T. Spontaneous regression of lung lesions after excision of the submandibular gland in a patient with chronic sclerosing sialadenitis. Auris Nasus Larynx. 2012;39(2):212-5.

Ominato J, Oyama T, Cho H, Shiozaki N, Umezu H, Takizawa J et al. The natural course of IgG4-related ophthalmic disease after debulking surgery: a single-centre retrospective study. BMJ Open Ophthalmol. 2019;4(1):e000295.

Abe A, Manabe T, Takizawa N, Ueki T, Yamada D, Nagayoshi K et al. IgG4-related sclerosing mesenteritis causing bowel obstruction: a case report. Surg Case Rep. 2016;2(1):120.

Okubo T, Oyamada Y, Kawada M, Kawarada Y, Kitashiro S, Okushiba S et al. Immunoglobulin G4-related disease presenting as a pulmonary nodule with an irregular margin. Respirol Case Rep. 2017;5(1):e00208.






Case Reports