Xanthogranulomatous Oophoritis Parodying as Ovarian Neoplasm: A Rare Case Report

INTRODUCTION Xanthogranulomatous inflammation of the ovary is a benign chronic inflammatory process destroying the normal anatomy of the organ, characterized by the presence of foamy histiocytes, plasma cells, fibroblasts, neutrophils, giant cells, and foci of necrosis. The gallbladder and kidney are often affected by this rare process. Other unusual sites associated with the xanthogranulomatous process are bones, urinary bladder, testis, epididymis, fallopian tubes, ovary, stomach, and the anorectal area.[1] In the female genital tract, xanthogranulomatous inflammation involves the endometrium, fallopian tubes, or ovaries, affecting them locally or completely. Clinically, they present as a pelvic cavity tumor, encasing the adjoining tissues, and due to its rare incidence, it is often misinterpreted as a malignant lesion.[2] Here, we report a rare case of endometriosis associated with xanthogranulomatous oophoritis in a 47-year-old female. CASE REPORT A 47-year-old woman presented to the gynecology clinic with abdominal pain and distension and also loss of appetite for the last 1 month. However, there was no loss of weight. Occasional bleeding per vagina was present for the last 15 days. Per abdominal examination revealed distension with a vague mass palpable in the left iliac fossa. Per vaginal examination revealed a normal cervix, and no palpable growth was noted. Usual investigations, including complete blood count, showed a decrease in hemoglobin (8.6 g/dL) and leukocytosis (18000 cells/cmm). Contrast-enhanced computed tomography abdomen revealed an ill-defined heterogeneous lesion with septations in the left adnexa measuring 5.2 cm × 4.3 cm, representing a tubo-ovarian cystic mass. Following this, raised beta-human chorionic gonadotropin levels of 8.9 mIU/mL (normal range >1 in females), cancer antigen 125 (CA-125) of 86.3 U/mL (normal range: 0–35 U/mL), serum alpha-fetoprotein of 2.2 ng/ml (normal range: 1.0–12.5 ng/ml), lactate dehydrogenase of 175/L (normal range: 100–412 U/L), and serum carcinoembryonic antigen of 2.1 ng/ml (normal range: 0–2.9 ng/ml) were noted. Clinical diagnosis was a malignant ovarian tumor. The cytology report of peritoneal fluid was negative for malignant cells. Following this, she was planned for total abdominal hysterectomy with bilateral salpingo-oophorectomy as well as removal of bilateral pelvic lymph node, paracolic lymph node, pelvic peritoneum, and omentum. Intraoperatively, a left ovarian mass of 5 cm × 4 cm size with omental adhesions was noted. No intraoperative frozen pathological examination was done in this case. We received the specimen consisting of the uterus along with bilateral adnexa [Figure 1] and other specimens such as bilateral pelvic lymph nodes, paracolic and subdiaphragmatic peritoneum, pelvic peritoneum, and omentum. Grossly, the uterus was measuring 8 cm × 7 cm × 4 cm. Attached left ovary measured 5 cm × 3 cm × 1.8 cm, with a gray-white surface in the cut section, showing a partly solid and partly multiloculated cystic cavity filled with serous fluid. The gross examination did not detect any pathology in the right ovary (3 cm × 1.5 cm × 1 cm), right fallopian tube (3 cm), and left fallopian tube (3 cm). The uterus showed leiomyoma measuring 0.4 cm × 0.4 cm. The cervix was unremarkable. The omentectomy specimen measuring 30 cm × 12 cm × 11 cm, grossly, did not show any deposits. Microscopic examination of the multiple sections studied from the left ovary showed the structure of the ovarian parenchyma with corpus albicantis. Few foci showed endometrial glands and stroma within the ovarian consistent with the features of endometriosis. Adjacent foci showed sheets of foamy macrophages and chronic inflammatory cells. Many foci showed congested blood vessels, a collection of macrophages and lymphocytes [Figure 2a and b]. The sections studied from the ovarian mass showed no evidence of granulomas or malignancy. A section from the right ovary showed features of a cortical cyst with corpus albicantia. Multiple sections from the omentum studied showed lobules of mature adipocytes arranged in lobules separated by fibrous septa. Foci of fat necrosis with foamy macrophages and chronic inflammatory cells were noted. There was no evidence of malignancy. Sections from the paracolic and subdiaphragmatic peritoneum and pelvic peritoneum showed fibrocollagenous tissue and congested blood vessels. Sections from the bilateral pelvic lymph nodes revealed reactive hyperplasia. The section from the endometrium showed the features of proliferative endometrium. Myometrium was nil-particular. There was no adenomyosis. Ziehl–Neelsen stain for acid-fast bacilli and periodic acid–Schiff stain were done to rule out tuberculosis and fungal infections. The final diagnosis of xanthogranulomatous oophoritis associated with endometriosis was made.

Adnexal masses are quite frequent among premenopausal women, with a prevalence of 34.9%.[3] Very few cases are malignant; however, the majority of malignancies are of tubo-ovarian origin in postmenopausal women. Xanthogranulomatous inflammation of the female genital tract is an unusual entity.[4] Kunakemakorn et al. were the first to report their case of xanthogranulomatous inflammation of the female genital tract in 1976.[5] Numerous hypotheses illustrate the pathogenesis and various etiologies of this condition, which include endometriosis, infections, intrauterine devices, drugs (antibiotics), as well as potential combinations of these factors.[6] The etiological factors also include some microorganisms such as Salmonella typhi, Escherichia coli, Bacteroides fragilis, and Staphylococcus aureus as possible inciting agents. The xanthogranulomatous inflammation commonly impacts the endometrium, followed by the vagina, cervix, fallopian tubes, and ovary. An additional theory for this phenomenon is tissue necrosis due to the prolonged infection, resulting in a collection of lipids and cholesterol from the dead cells, and thus, the phagocytosis of these biological components by the macrophages incites a xanthomatous process.[7] Xanthogranulomatous oophoritis commonly affects the age group of 23–72 years; however, the youngest age group reported is a case of a 2-year-old female.[8] Fever, abdominal pain, abdominal mass, menorrhagia, anemia, and anorexia are the common clinical presentations as observed in our case.[9] The significance of this entity lies in the fact that this can mimic malignancy, as with the clinicoradiological findings. As a result, many of the cases were misdiagnosed as ovarian cancers.[10] The mass usually grows up to 3–7 cm in maximum dimension, and the resultant inflammation causes adhesions with neighboring organs, pelvic structures, and peritoneum, further raising the suspicion of malignancy. Further, CA-125, a serological marker used for diagnosing epithelial ovarian cancer, can also be raised in xanthogranulomatous oophoritis, thus confusing the two diagnoses.[11] Hence, awareness of this entity among the gynecologists should be considered to avoid misdiagnosis as endometrial, ovarian, or tubal malignancy. Macroscopically, a well-circumscribed mass of solid to cystic consistency is seen occasionally entangling the adjoining organs and completely replacing the involved tube and ovary, thus mimicking malignancy. Microscopically, chronic inflammatory cell infiltrate, admixed with focal or sheets of foam cells, and vascular proliferation are seen replacing the normal ovarian parenchyma. Malakoplakia is the closest differential for this condition. Malakoplakia shows cytoplasmic concentric calcific bodies (Michaelis–Gutmann bodies), which are not seen in xanthogranulomatous inflammation. Other inflammatory conditions that mimic this condition are tuberculosis and fungal infections.[12] In this study, Ziehl–Neelsen stain for acid-fast bacilli and periodic acid–Schiff stain were done to rule out tuberculosis and fungal infections. Both were negative, excluding these possibilities. To ascertain the diagnosis, immunohistochemistry with CD68 (foam cells positive), CD3 (T-lymphocyte marker), and CD20 (B-lymphocyte marker) can be helpful. However, they are used very seldomly due to the distinctive histopathological features, as in the present case, and thereby were not used. Xanthogranulomatous inflammation causes systemic inflammation, which may be lethal, and so aggressive treatment in the form of surgery should be strongly considered. Oophorectomy is the treatment of choice.[6] Our patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy along with the removal of bilateral pelvic lymph node, paracolic and subdiaphragmatic peritoneum, pelvic peritoneum, and omentum, as the case was misdiagnosed clinically as ovarian malignancy. Intraoperative frozen pathology may aid in the diagnosis for a decision of cystectomy or oophorectomy. Hence, understanding this inflammatory condition for both the pathologists and gynecologists is important for the prevention of radical surgery and also for avoiding misdiagnosis as a malignancy.

Xanthogranulomatous oophoritis is a rare entity, and so its detailed incidence, long-term prognosis, and recurrence rates are not known. Precisely diagnosing this condition is very essential to decide the best choice of management and to avoid surgical over- or undertreatment. This entity of chronic inflammation, correlating with the unique histopathological features and the necessity for surgical management, claims additional research work to unveil the risk factors and improved insight into the underlying pathogenesis. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Endometriosis; inflammation; malignancy; ovary; xanthogranuloma