{"paper_id":"93f3d94f-16f4-44a6-8843-50a0b0e84aaf","body_text":"Xanthogranulomatous Oophoritis in the Setting of Post-\nIVF Twin Pregnancy with History of Endometriosis: A Rare\nCase Report with Review of Literature\nChinmay Shrikrishna Pendharkar 1 Sunita B.S. 1 Ganesh Pendkur 1 Dheeraj J. Nandagawe 1\n1 Department of Pathology, Armed Forces Medical College, Pune,\nMaharashtra, India\nInd J Med Paediatr Oncol 2022;43:314 –317.\nAddress for correspondence Chinmay Shrikrishna Pendharkar, MBBS,\nDepartment of Pathology, Armed Forces Medical College, Pune,\nMaharashtra, India (e-mail: drchinmaypendharkar@gmail.com).\nIntroduction\nXanthogranulomatous in flammation is an uncommon form\nof chronic in flammatory process that is destructive to the\ninvolved organs. 1 The usual sites of involvement are the\nkidneys and gallbladder, though it can affect the anorectal\narea, bone, stomach, and testis. 2 The involvement of female\ngenital tract is uncommon and if involved, it generally affects\nthe endometrium. 3 Only a few cases affecting the vagina,\ncervix, fallopian tubes, and ovaries have been described.4 The\nKeywords\n► pathology\n► xanthogranu-\nlomatous oophoritis\n► foamy histiocytes\nAbstract Xanthogranulomatous in flammation is an uncommon, nonneoplastic, chronic process\nin which the affected organ is destroyed by massive cellular in filtration of foamy\nhistiocytes admixed with multinucleated giant cells, plasma cells, fibroblasts, neutro-\nphils, and foci of necrosis. The organs most commonly affected by this entity are kidney\nand gallbladder. Only a few cases involving the ovary have been reported. The etiology\nof this entity is unknown, but the associated risk factors are infection, inappropriate\nantibiotic therapy, use of intrauterine con traceptive device, abnormalities in lipid\nmetabolism, endometriosis, leiomyoma, and recurrent pelvic in flammatory disease.\nThe current case was a 31-year-old female with post-in vitro fertilization (post-IVF) twin\npregnancy, who presented with pain in the abdomen, provisionally diagnosed as\novarian mass with suspicion of malignancy. Patient was treated with antibiotics and an\nelective lower segment cesarean section was planned, due to lower abdominal pain and\nthe presentation of both fetuses being breech. Patient underwent exploratory\nlaparotomy with right oophorectomy and left double-J stenting, in view of left\nhydronephrosis. Diagnosis of xanthogranul omatous oophoritis was established on\nhistopathological examination. There is only a single case; previously documented\nfrom India, in the setting of primary infertility. The present case report stresses that the\ncondition may affect women of any age group and may be encountered in pregnancy,\nas has been observed in this case. Hence, thinking about this differential diagnosis in\nthe cases presenting with similar complaints, would be of great value to avoid\nmisdiagnosing them.\nDOI https://doi.org/\n10.1055/s-0042-1748489.\nISSN 0971-5851.\n© 2022. Indian Society of Medical and Paediatric Oncology. All\nrights reserved.\nThis is an open access article published by Thieme under the terms of the\nCreative Commons Attribution-NonDeri vative-NonCommercial-License,\npermitting copying and reproduction so long as the original work is given\nappropriate credit. Contents may not be used for commercial purposes, or\nadapted, remixed, transformed or built upon. (https://creativecommons.org/\nlicenses/by-nc-nd/4.0/)\nThieme Medical and Scienti fic Publishers Pvt. Ltd., A-12, 2nd Floor,\nSector 2, Noida-201301 UP , India\nCase Report with Review of Literature\nTHIEME\n314\nArticle published online: 2022-07-01\n\ncharacteristic massive in filtration of tissues by foamy his-\ntiocytes along with the plasma cells, lymphocytes, and\npolymorphonuclear leukocytes is evident on histopathology.\nThe rare but unique histopathological appearance of this\nentity, noted in the pregnant female with post-in vitro\nfertilization (post-IVF) twin conception, warranted the\ncase report of the said patient.\nCase Report\nA 30-year-old female (a primigravida, who was treated for\ninfertility using short protocol using gonadotrophin-releas-\ning hormone antagonist injection cetrorelix, with post-IVF-\nembryo transfer [ET] conception with diamniotic dichorionic\ntwins—period of gestation being 35 weeks) reported to the\nemergency department of a tertiary care hospital with\ncomplaints of moderate to severe pain in lower abdomen,\nincreased frequency of urination, and bleeding, while pass-\ning urine.\nShe was a known case of endometriosis and had presented\nearlier at the beginning of second trimester with endome-\ntrioma, for which she had undergone an emergency explor-\natory laparotomy, right salpingectomy, and right ureteric\ndouble-J (DJ) stenting, in view of hydroureteronephrosis. She\nwas also a known case of hypothyroidism, anemia in preg-\nnancy, receiving thyroid hormone replacement therapy, and\nhematinics.\nPatient was hemodynamically stable. On per abdominal\nexamination, 36 weeks gravid uterus was noted with a tense\nanterior abdominal wall, without tenderness. Cardiac activi-\nty of both the fetuses was also noted. On per vaginal\nexamination, mass was noted in the pouch of Douglas. The\npatient was found to be having leukocytosis (total leucocyte\ncount 14,700/mm\n3). Other hematological and biochemical\nparameters were found to be within reference limits. Urine\nculture was positive for Escherichia coli , sensitive to amika-\ncin, cotrimoxazole, nitrofurantoin, fosfomycin, and minocy-\ncline. The magnetic resonance imaging showed a large\nheterogeneous mass in the right adnexa, measuring 102 /C289\n/C2108 mm (anterior-posterior /C2transverse /C2craniocaudal),\nsuperiorly extending from the level of L3 –4 to the level of\ncoccyx inferiorly. The lesion appeared to be originating in the\nright adnexa and extending to the pouch of Douglas. Tumor\nmarkers like human epididymis protein 4, alpha-fetoprotein,\ncancer antigen 125 (CA 125), CA 19–9, and carcinoembryonic\nantigen were not elevated.\nPatient was treated with antibiotics and an elective lower\nsegment cesarean section (LSCS) was planned, due to lower\nabdominal pain. Both fetuses were having breech presenta-\ntion. The exploratory laparotomy was planned along with\nright oophorectomy and left DJ stenting, in view of left\nhydronephrosis. During LSCS, two live preterm fetuses, a\nfemale and a male, weighing 2.2 and 1.9 kg, respectively,\nwere delivered and required neonatal resuscitation. About\n1.8 L of foul-smelling purulent discharge was drained from\nthe right adnexal mass and was sent for microbiological\ninvestigations. Ziehl–Neelsen staining was negative for acid-\nfast bacilli, and the pus culture did not grow any pyogenic\nbacteria. Appendectomy was performed due to unhealthy\nappearance of the same. The left DJ stenting was performed,\nas planned.\nThe oophorectomy and appendectomy specimens were\nsent for histopathological examination (HPE). On gross ex-\namination, right ovarian mass, measuring 15 /C213 cm,\nshowed lobulated and congested external surface. On cutting\nopen the specimen, two cysts were noted of size 12 /C29a n d\n10 /C26 cm, with the smaller cyst showing the presence of pus.\nOn microscopic examination, the cyst wall was seen to be\ncomprising of fibrocollagenous tissue, infiltrated by sheets of\nfoamy macrophages, lymphocytes, plasma cells, and neutro-\nphils (\n►Figs. 1 –3). Proliferating blood vessels were noted.\nFew areas showed fibrosis with hemosiderin-laden macro-\nphages. Endometrial glands or stroma was not seen. The\nfeatures like atypia and dysplasia were characteristically\nabsent. The appendix showed normal histomorphology\nwith periappendiceal in flammation. CD68 immunohis-\ntochemistry con firmed the presence of foamy histiocytes\n(\n►Figs. 4 , 5), while pan-cytokeratin was negative. Diagnosis\nof xanthogranulomatous oophoritis was made, based on the\nhistomorphological features.\nPostoperative recovery of the patient was uneventful.\nFig. 1 The presence of large number of foamy histiocytes\nadmixed with plasma cells, lymphocytes, and occasional neutrophils,\nis seen, which is pathognomonic feature of xanthogranulomatous\noophoritis (hematoxylin and eosin staining; magni fication 100x).\nFig. 2 The presence of large number of foamy histiocytes admixed\nwith plasma cells, lymphocytes, and occasional neutrophils, is\nseen, which is pathognomonic feature of xanthogranulomatous\noophoritis (hematoxylin and eosin staining; magni fication 100x).\nIndian Journal of Medical and Paediatric Oncology Vol. 43 No. 3/2022 © 2022. I ndian Society of Medical and Paediatric Oncology. All rights reserved.\nXanthogranulomatous Oophoritis Pendharkar et al. 315\n\n\nDiscussion\nXanthogranulomatous inflammation is a rare benign in flam-\nmatory disease —commonly noted in the kidney and gall-\nbladder.5–8 It has also been found in other sites like bones,\ntestis, and small intestine. 5,9,10 The involvement of the\nfemale genital tract is somewhat less common, which can\npresent as endometritis, oophoritis, and salpingitis. 4,11,12\nThe ovarian involvement sometimes has been referred to\nas ovarian fibroxanthoma.4 The average age of presentation\nis 38.5 years (commonly cases reporting between 23 and 72\nyears), though there has been a case report stating the\noccurrence of the disease in a 2-year-old child.\n2,13 The\ndisease can present, either unilaterally or bilaterally.13 About\n32 cases have been reported from India and the incidence\nseems to be more in the developing countries than the\ndeveloped countries.\n14\nThe predisposing factors thought of, are pelvic in flamma-\ntory disease (PID), endometriosis, and inadequate antibiotic\ntherapy.15–17 Abnormal lipid metabolism has also been\nconsidered as one of the predisposing factors. 18,19 Previous-\nly, cases have also been reported with infertility and this can\nbe considered as one of the predisposing factors. 16 The\npathogenesis of this condition remains unidenti fied,20\nwith various proposed mechanisms like ineffective clearance\nof bacteria by phagocytosis or as late sequalae of PID. 2 In\ncases of endometriosis, sites of bleeding along with the\nobstruction remain to be the fertile soil for the growth of\nmultitude of microorganisms, which is then followed by\ntissue necrosis and the subsequent release of cholesterol\nand other lipids, which further leads to increased number of\ntissue macrophages, trying to phagocytose these products.\n21\nIt has been also considered by some that xanthogranulom-\natous oophoritis may be the rare aggressive form of the\ncommonly occurring endometriosis.\nCommon presentations include abdominal pain, adnexal\ntenderness, pelvic mass, menorrhagia, fever, etc. On hema-\ntological investigations, leukocytosis is a consistent finding\nwith raised erythrocyte sedimentation rate. 4 The radiologi-\ncal investigations, con firm the ovarian mass, which is well-\ndefined and often raises the suspicion of malignancy. 4 Bac -\nterial infection is seen in many cases, the causative organ-\nisms commonly noted being E. coli , Proteus spp. ,\nStaphylococcus aureus , Bacteroides fragilis , Salmonella typhi ,\nActinomyces, Streptococcus faecalis , viridans streptococci ,\netc.21 The pus culture is negative for acid-fast bacteria. The\nantibiotic therapy alone is not suf ficient and operative\ntreatment is necessary in the form of salpingo-oophorecto-\nmy, depending on the site of lesion. 3,4,22\nThe gross appearance of the specimens may show cystic\ndegeneration, foci of necrosis, and hemorrhage, in grossly\nenlarged ovary. The microscopic examination shows the\npresence of foamy histiocytes, along with a mixture of\ninflammatory cells like lymphocytes, plasma cells, and occa-\nsional neutrophils.\n21 Foamy histiocytes, the so-called “xan-\nthoma cells, ” are histiocytes with abundant lipid-laden\ncytoplasm having a vacuolated appearance. These impart\nthe yellowish color to the specimen, when seen on the gross\nexamination. There is no cellular atypia, abnormal mitoses,\nor any other microscopic feature suggestive of malignancy.\nCD68 immunostaining shows characteristic staining of\nfoamy histiocytes, which con firms the diagnosis.\n23 The dif-\nferential diagnoses of this condition include malakoplakia,\nchronic infections like tuberculosis, etc. This rare condition\nshould be included in the differential diagnosis of ovarian\ncysts/tumors and endometrioma abscesses.\nIn our case, the mass in the right adnexa, though previ-\nously thought to be of either neoplastic or infective etiology,\nthe HPE revealed the actual diagnosis, which was crucial in\nfurther patient management. Though the entity is overall\nFig. 3 The presence of large number of foamy histiocytes\nadmixed with plasma cells, lymphocytes, and occasional neutrophils,\nis seen, which is pathognomonic feature of xanthogranulomatous\noophoritis (hematoxylin and eosin staining; magni fication 400x).\nFig. 4 The foamy histiocytes show positivity by immunostaining with\nCD68. (CD68 antibody staining; magni fication 100x).\nFig. 5 The foamy histiocytes show positivity by immunostaining with\nCD68. (CD68 antibody staining; magni fication 400x).\nIndian Journal of Medical and Paediatric Oncology Vol. 43 No. 3/2022 © 2022. In dian Society of Medical and Paediat ric Oncology. All rights reserved.\nXanthogranulomatous Oophoritis Pendharkar et al.316\n\n\nrare and cannot be diagnosed without the HPE, it is impera-\ntive to consider this entity in the differential diagnoses for\npatients presenting with similar complaints especially in\ncases having predisposing conditions like PID, endometri-\nosis, inadequate antibiotic therapy, infertility, etc.\nConclusion\nXanthogranulomatous inflammation, occurring in female gen-\nital tract, is extremely rare. The presence of this entity, should\nalways be paid due attention, as meticulous HPE is the key in its\ndiagnosis. This case report, which is the first documented\nreport of occurrence of xanthogranulomatous oophoritis, in a\npregnant female with post-IVF ET pregnancy, having history of\nendometriosis and infertility, stresses the fact that the condi-\ntion can present in varied settings and high level of suspicion is\nof paramount importance for the diagnosis.\nDeclaration of Patient Consent\nThe authors certify that they have obtained all appropri-\nate patient consent forms. In the form, the patient has\ngiven her consent for her images and other clinical\ninformation to be reported in the journal. The patient\nunderstands that her name and initials will not be pub-\nlished and due efforts will be made to conceal their\nidentity.\nConflict of Interest\nNone declared.\nReferences\n1 Jung SE, Lee JM, Lee KY, Han KT, Hahn ST. Xanthogranulomatous\noophoritis: MR imaging findings with pathologic correlation. AJR\nAm J Roentgenol 2002;178(03):749 –751\n2 Bhatnagar K, Narang V, Garg B, Sood N. 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Ann Hematol 2018;97\n(11):2117–2128\nIndian Journal of Medical and Paediatric Oncology Vol. 43 No. 3/2022 © 2022. I ndian Society of Medical and Paediatric Oncology. All rights reserved.\nXanthogranulomatous Oophoritis Pendharkar et al. 317","source_license":"CC0","license_restricted":false}