{"paper_id":"57d739b5-4e85-44e8-8aed-d7d00451cc61","body_text":"International Journal of Science and Research (IJSR)\n \nISSN: 2319\n-\n7064\n \nImpact Factor 2025: 7.089\n \nVolume 15 Issue 3, March 2026\n \nFully Refereed | Open Access | Double Blind Peer Reviewed Journal\n \nwww.ijsr.net\n \nIgG4\n-\nrelated Disease of the Ovary Masquerading as \nChronic Tubo\n-\nO\nvarian Mass with Deep Infiltrating \nEndometriosis: A \nDiagnostic \nC\nhallenge\n \n \nDr. C P Srujana\n1\n, Dr. Sujata N Datti\n2\n, Dr. Mounica KSN\n3\n, Dr. Harshitha M\n4\n \n \n1\nMain Author, Department of Obstetrics and Gynecology, V ydehi Institute of Medical Sciences and Research Centre, Bengaluru, Ka\nrnataka, \nIndia\n \n \nCorresponding Authors:\n \n \n2\nProfessor, Department of Obstetrics and Gynecology, V ydehi Institute of Medical Sciences and Research Centre, Bengaluru, Karn\nataka, \nIndia\n \n3\nAssistant Professor, Department of Obstetrics and Gynecology, V ydehi Institute of Medical Sciences and Research Centre, Benga\nluru, \nKarnataka, India\n \n4\nDepartment of Obstetrics and Gynecology, Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, Karnataka, Indi\na\n \n \nAbstract\n: \nIgG4\n-\nrelated disease (IgG4\n-\nRD) is a rare immune\n-\nmediated fibro\n-\ninflammatory disorder that can involve multiple organs and \nfrequently mimics malignancy or chronic inflammatory conditions. Genital tract involvement is uncommon, and ovarian involveme\nnt is \next\nremely rare.¹ \nSo far only 4 cases of ovarian involvement have been reported, with all of them mimicking malignancy followed by HPE \nconfirmed diagnosis. The youngest reported till date was 36 years. \nWe report a case of a 20\n-\nyear\n-\nold unmarried woma\nn presenting with \nlong\n-\nstanding lower abdominal pain and cyclical bleeding per rectum, clinically and radiologically suggestive of a chronic tubo\n-\novarian \nmass with deep infiltrating endometriosis. Imaging \nhad \nrevealed a complex adnexal mass with pelvic adhesions and right\n-\nsided \nhydroureteronephrosis. The patient underwent exploratory laparotomy with right salpingo\n-\noophorectomy following bilateral ureteric \nstenting. Histopathological examination revealed dense l\nymphoplasmacytic infiltration with fibrosis, co\nnsistent with IgG4\n-\nrelated disease \nof the ovary\n \nconfirmed on IHC\n. This case highlights the diagnostic dilemma posed by ovarian IgG4\n-\nRD and emphasizes the importance \nof histopathology and immunohistochemistry in establishing the diagnosis and preventing unnecessary radical surgery.\n \n \nKeywords:\n \nIgG4\n-\nrelated disease, Ovary, Tubo\n-\novarian mass, Endometriosis, Pseudotumor\n \n \n1.\n \nIntroduction\n \n \nIgG4\n-\nrelated disease is a systemic fibro\n-\ninflammatory \ncondition characterized by lymphoplasmacytic infiltration \nrich in IgG4\n-\npositive plasma cells, storiform fibrosis, and \nvariable elevation of serum IgG4 levels.² It most commonly \naffects the pancreas, sal\nivary glands, biliary tract, kidneys, \nand retroperitoneum.³ Involvement of the female genital tract \nis rare, with only isolated cases of ovarian IgG4\n-\nRD reported \nin the literature.\n4\n-\n \n6\n \n \nOvarian IgG4\n-\nRD often presents as a mass lesion and closely \nmimics ovarian malignancy, tubo\n-\novarian abscess, or \nendometriosis, leading to extensive surgical intervention\n.\n7\n \nWe \nreport a rare case of IgG4\n-\nrelated disease of the ovary \npresenting as a chronic tubo\n-\novarian mass with deep \ninfiltrating endometriosis.\n \n \n2.\n \nCase Report\n \n \nA 20\n-\nyear\n-\nold unmarried woman presented with complaints \nof lower abdominal pain for eight years, insidious in onset and \ngradually progressive, with worsening over the last four \nmonths. Pain was predominantly localized to the lower \nabdomen and aggravated by\n \nwalking and climbing stairs. \nThere was no associated nausea or vomiting. She also \nreported cyclical bleeding per rectum since 2018, suggestive \nof deep infiltrating endometriosis\n \nand had received treatment \nfor the same.\n8\n \n \nShe had no bowel or urinary complaints. Menstrual history \nrevealed last menstrual period\n \none month back\n, associated \nwith bleeding per rectum. She was not sexually active. There \nwere no medical comorbidities. \nThe patient had undergone \npelvic surgery at 3y of age for which documents were not \navailable. She also had diagnostic laparoscopy in 2017in view \nof hematometra, hematosalpinx, endometriosis, was noted to \nhave cervical atresia and was started on Anti tuberc\nular \ntherapy twice suspecting genital tuberculosis for 3 months. \nThe patient was initially started with cat 1 drugs which she \ndefaulted and later start\ned on cat 2 drugs. The patient also \nunderwent examination under anaesthesia followed by \ndrainage of hematometra in 2018 with Cu\n-\nT insertion which \nwas later removed in October 2018. The patient also gave \nhistory of cervical dilatation in \nJ\nuly and november 2019\n \n \nOn examination, she was hemodynamically stable with no \npallor or lymphadenopathy. Abdominal examination revealed \na suprapubic mass corresponding to 16\n–\n18\n \nweeks’ size, firm, \nirregular, non\n-\nmobile, and non\n-\ntender. Per\n-\nrectal examination \nrevealed a 1 × 1 cm non\n-\ntender mass, suspicious for deep \ninfiltrating endometriosis.\n \n \nLaboratory investigations showed hemoglobin of 8.9 g/dL. \nTumor markers were within normal limits. Ultrasonography \nrevealed a large thick\n-\nwalled cystic lesion with tubular \ncomponents in the right adnexa measuring 8.6 × 7.0 × 7.6 cm \nwith pelvic adhesions and\n \nright\n-\nsided hydroureteronephrosis. \nMRI pelvis demonstrated a well\n-\ndefined collection measuring \n7.4 × 8.8 × 9 cm anterior to the right iliopsoas muscle and a \nPaper ID: SR26321051635\nDOI: https://dx.doi.org/10.21275/SR26321051635\n1273 \n\nInternational Journal of Science and Research (IJSR)\n \nISSN: 2319\n-\n7064\n \nImpact Factor 2025: 7.089\n \nVolume 15 Issue 3, March 2026\n \nFully Refereed | Open Access | Double Blind Peer Reviewed Journal\n \nwww.ijsr.net\n \nlesion in the posterior wall of the upper one\n-\nthird of the \nrectum, suggestive of deep infiltrating endometriosis.\n \n \nAn initial diagnosis of Deep infilterating Endometriosis was \nmade based on the clinical findings and sonographic \ninvestigations and \nA multidisciplinary approach was planned\n \ninvolving the General Surgery, Gynaecology and Urology \nteams\n. The patient underwent bilateral DJ stenting followed \nby exploratory laparotomy. Intraoperatively, a frozen pelvis \nwith grossly distorted anatomy was noted.\n \nNecrotic tissues \nnoted below the rectus\n-\n \nsample taken and sent for \nhistopathology and CBNAAT.\n \nA right tubo\n-\novarian mass \nm\neasuring approximately 8 × 10 cm was identified, densely \nadherent to the uterus, bowel, bladder, and lateral pelvic wall. \n \nOn further dissection of the mass, purulent content was noted, \ncollected and sent for testing and cultures. Very minimal \novarian tissue noted in right side and left ovary and tube were \nnot visualised. In suspicion of malignancy, Surgical oncology \nteam was called intraoperatively. \nRight salpingo\n-\noophorectomy with extensive adhesiolysis was performed.\n \n \n \nThe patient was advised for colonoscopy, but couldn’t be \ndone due to financial constraints. \n \n \nThe pus culture reported to be MDRO positive for escherichia \nColi and was started on Higher Antibiotics. However, the pus \nand tissue tested negative for tuberculosis and malignancy.\n \n \nHistopathological examination revealed dense \nlymphoplasmacytic infiltration with fibrosis\n \nconsistent with \nIgG4\n-\nrelated disease of the ovary.\n9\n \n \n \n \nFigure 1\n: \n(a)H&E; 4\nx\n \nmagnification. (b)H&E; 10x magnification. (c)H&E; 40x magnification. (a)(b)(c)\n-\nOmental tissue \nshows fibrocollagenous tissue with infiltration of chronic inflammatory cells\n-\nplasma cells, lymphocytes, foamy histiocytes \nand eosinophils\n \n \n \nFigure 2:\n \n(a)H&E; 4x magnification. (b)H&E; 40x magnification. (a)(b)\n-\nRight tubo ovarian mass shows fibrocollagenous \ntissue with infiltration of chronic inflammatory cells\n-\nplasma cells, lymphocytes, foamy histiocytes and eosinophils with focal \nareas of hemorrhage.\n \n \nPaper ID: SR26321051635\nDOI: https://dx.doi.org/10.21275/SR26321051635\n1274 \n\nInternational Journal of Science and Research (IJSR)\n \nISSN: 2319\n-\n7064\n \nImpact Factor 2025: 7.089\n \nVolume 15 Issue 3, March 2026\n \nFully Refereed | Open Access | Double Blind Peer Reviewed Journal\n \nwww.ijsr.net\n \nFor confirmation of the histopathological diagnosis, sample \nblocks were sent for immunohistochemistry and Serum IgG\n \nlevels which reported 235mg/Dl, \nwhich confirmed the \ndiagnosis.\n \n \n \nFigure 3\n:\n \nImmunohistochemical characteristics of the \nplasma cell infiltrate, A CD138 marker highlighting the \nprominent plasma cell component\n \n \nThe patient was then discharged after completing the course \nof antibiotics.\n \n \nThe patient was then started on oral steroids for 6 weeks.\n \nThe \npatient was planned for \nfurther follow up with serial \nmonitoring with sonography. Steroids were stopped after 6 \nweeks. Follow up scan after 3 months showed no nodules, \nendometriosis. The patient was then asked for annual follow \nup. The patient has been asymptomatic till date. \n \n \n3.\n \nDiscussion\n \n \nIgG4\n-\nrelated disease is an under\n-\nrecognized cause of ovarian \nmass lesions. The disease often mimics malignancy or \nchronic inflammatory conditions both clinically and \nradiologically.\n4\n-\n7\n \nMost reported cases of ovarian IgG4\n-\nRD \nhave been diagnosed postoperatively following \nhistopathological and immunohistochemical evaluation.\n6\n \n \nThe comprehensive diagnostic criteria for IgG4\n-\nRD include \norgan enlargement or mass, elevated serum IgG4 levels (>135 \nmg/dL), and histopathological features of dense \nlymphoplasmacytic infiltration, storiform fibrosis, and an \nIgG4/IgG ratio greater than 40%\n.\n2, 10\n \nThe condition responds \nwell to corticosteroid therapy when diagnosed early.\n11\n \n \nIn our patient, long\n-\nstanding symptoms, distorted pelvic \nanatomy, and imaging findings led to extensive surgical \nmanagement. This case underscores the importance of \nincluding IgG4\n-\nRD in the differential diagnosis of adnexal \nmasses with normal tumor markers\n.\n \n \n4.\n \nConclusion\n \n \nIgG4\n-\nrelated disease of the ovary is a rare entity that can \nclosely mimic malignancy and chronic pelvic inflammatory \nconditions. A high index of suspicion, combined with \nhistopathological and immunohistochemical evaluation, is \nessential for accurate diagnosis. Awareness of this condition \nmay help avoid unnecessary extensive surgery and facilitate \nappropriate medical management.\n \n \nClinical Significance\n \n \n•\n \nIgG4\n-\nRD should be considered in chronic atypical adnexal \nmasses\n \n•\n \nNormal tumor markers do not exclude serious pathology\n \n•\n \nHistopathology and immunohistochemistry are essential \nfor diagnosis\n \n•\n \nEarly recognition may prevent radical surgery\n \n \nDeclarations\n \n \nFunding:\n \nNone\n \n \nConflict of Interest:\n \nNone declared\n \n \nEthical Approval:\n \nNot required for single case report as per \ninstitutional policy\n \n \nPatient Consent:\n \nWritten informed consent was obtained \nfrom the patient\n \n \nReferences \n \n \n[1]\n \nStone JH, Zen Y , Deshpande V . IgG4\n-\nrelated disease. N \nEngl J Med. 2012;\n \n366:\n \n539\n-\n551. \n \n[2]\n \nUmehara H, Okazaki K, Masaki Y , et al. Comprehensive \ndiagnostic criteria for IgG4\n-\nrelated disease. Mod \nRheumatol. 2012;\n \n22:\n \n21\n-\n30. \n \n[3]\n \nDeshpande V , Zen Y , Chan JK, et al. Consensus \nstatement on the pathology of IgG4\n-\nrelated disease. \nMod Pathol. 2012;\n \n25:\n \n1181\n-\n1192. \n \n[4]\n \nPoomalar GK, Rajkumar S, Thara K, Srinivas BH. A \nrare case of immune\n-\nrelated pseudotumor of ovary \nmimicking ovarian malignancy. Int J Reprod Contracept \nObstet Gynecol. 2023;\n \n12:\n \n2838\n-\n2842. \n \n[5]\n \nZen Y , Nakanuma Y . IgG4\n-\nrelated disease: A cross\n-\nsectional study. Histopathology. 2010;\n \n56:\n \n1\n-\n12. \n \n[6]\n \nSah RP, Pannala R, Chari ST. IgG4\n-\nrelated disease of \nthe ovary: A rare entity. Am J Surg Pathol. 2013;\n \n37:\n \n163\n-\n169. \n \n[7]\n \nMinato H, Shimizu J, Arano Y , et al. IgG4\n-\nrelated \ndisease mimicking gynecological malignancy. Pathol \nInt. 2015;\n \n65:\n \n25\n-\n30. \n \n[8]\n \nAbrao MS, Petraglia F, Falcone T, Keckstein J, Osuga \nY , Chapron C. Deep endometriosis infiltrating the \nrectum. Hum Reprod Update. 2015;\n \n21:\n \n329\n-\n339. \n \n[9]\n \nCheuk W, Chan JK. IgG4\n-\nrelated sclerosing disease: A \ncritical appraisal. Adv Anat Pathol. 2010;\n \n17:\n \n303\n-\n332. \n \n[10]\n \nWallace ZS, Naden RP, Chari S, et al. The 2019 \nACR/EULAR classification criteria for IgG4\n-\nrelated \ndisease. Ann Rheum Dis. 2020;\n \n79:\n \n77\n-\n87. \n \n[11]\n \nKamisawa T, Zen Y , Pillai S, Stone JH. IgG4\n-\nrelated \ndisease. Lancet. 2015;\n \n385:\n \n1460\n-\n1471.\n \nPaper ID: SR26321051635\nDOI: https://dx.doi.org/10.21275/SR26321051635\n1275","source_license":"CC0","license_restricted":false}