Xanthogranulomatous Salpingitis Mimicking an Ovarian Tumor: A Diagnostic Challenge.

A 37-year-old nulliparous woman, married for the last 8 years, attended the gynecological outpatient department with complaints of right-sided dull aching pain abdomen on and off for 4 months. She gave no history of discharge per vagina, fever, nausea, vomiting, weight loss, urinary problems, or bowel habit changes. She, as well as her husband, denied the use of any contraceptives in the past. Her menstrual cycles were regular with normal flow and duration. Her vitals were normal on examination. On abdominal examination, an ill-defined cystic mass was palpated on the right side of the abdomen extending from the right iliac fossa to the right hypogastrium. Per speculum examination revealed a healthy cervix as well as vaginal walls. On per vaginal examination, the uterus cannot be felt separately from the adnexal mass. The right-sided forniceal fullness can be felt, which was nontender. The left fornix was free and nontender. Per rectal examination was unremarkable. Ultrasonography revealed a loculated collection of 10 cm × 20 cm in the pelvic cavity in the right paramedian location. The uterus was normal in size. Contrast-enhanced computed tomography abdomen was done, which showed a large hypodense cystic lesion (12.1 cm × 21 cm × 23.2 cm) arising from the right adnexa extending into the abdominal cavity reaching superiorly up to the subhepatic region. A few thin internal septations were noted within the lesion. The lesion was abutting the anterior abdominal wall anteriorly. Her complete blood count was within normal limits. CA 19-9 and CEA were mildly elevated, 45.4 U/ml and 4.78 ng/ml, respectively. CA125 was 12.8 U/ml. The differential diagnosis of an adnexal mass with infertility includes endometrioma, tuboovarian mass, and ovarian malignancy. The patient was subjected to an exploratory laparotomy, suspecting a malignant ovarian tumor. Considering the huge size of the lesion, which was extending up to the xiphisternum and anticipating adhesions as the lesion was fixed, a midline vertical incision was given. As the rectus muscle was separated, a huge cyst (10 cm × 20 cm) was found on the right side, which was adhered to the abdominal wall anteriorly, obscuring the access to the peritoneal cavity. Trying to manipulate the cyst to enter the peritoneal cavity resulted in spontaneous rupture of the cyst, which drained around 2 l of serosanguinous fluid. Once the cyst was ruptured, the peritoneal cavity was explored. The cyst was found to be arising from the right broad ligament. The posterolateral surface of the cyst was densely adherent to the pelvic side walls, the retroperitoneum, the liver base, uterus, bladder, and bowel, completely distorting the anatomy. Both the ovaries as well as the tubes were completely buried in adhesions. After careful adhesiolysis, it was found that the uterus was of normal size and both ovaries seems healthy. However, the right-sided tube had bluish black discolouration, and small cauliflower-like projections were noted from the fimbrial end of the right tube. This unhealthy tube was removed through salpingectomy [ Figure 1 ]. The left-sided tube was also swollen with a closed fimbrial end. Considering the nulliparous state of the patient, salpingostomy was done. During the process of adhesiolysis, a small bowel tear in the ileum was noted, for which primary repair was done. Gross specimen: Fallopian tube with attached fimbriae and soft-tissue cystic specimen of size 6 cm × 2 cm × 1.5 cm showing Blackish discoloration The patient had an uneventful postoperative period and was discharged on postoperative day 10. The histopathology from the fimbrial end of the fallopian tube showed accumulation of foamy macrophages in the wall along with chronic inflammatory cells, mainly lymphocytes, histiocytes, and congestion in the surrounding area, which is suggestive of xanthogranulomatous salpingitis [ Figure 2 ]. A section from the rest of the fallopian tube, was of unremarkable histology. The cyst wall was negative for malignancy. Section from the fimbria Fallopian tube shows accumulation of foamy macrophages in the wall along with chronic inflammatory cells, mainly lymphocytes, histiocytes, and congestion in the surrounding area

Xanthogranulomatous inflammation is a rare form of chronic inflammation which results in destruction of tissues of the affected organs. Although reported to involve the kidney, gallbladder, stomach, anorectal area, bone, urinary bladder, testis, and epididymis, the involvement of the female genital tract is extremely uncommon and is mainly limited to the endometrium. However, few cases of xanthogranulomatous salpingitis as well as oophoritis have been reported in the literature.[ 1 ] Most of these patients will present as pelvic inflammatory disease unresponsive to antibiotics or as an adnexal mass.[ 2 ]

Xanthogranulomatous salpingitis is a very rare condition, often mimics a benign or malignant adnexal mass. Knowledge of the condition and a high degree of suspicion, both by the clinician and the pathologist, will help in the appropriate management. The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. There are no conflicts of interest.

Xanthogranulomatous inflammation affecting the female genital tract is extremely rare and usually presents as an adnexal mass.[ 1 2 3 4 5 ] It affects patients between 18 and 72 years of age, mostly occurring in the third decade of life. Patients usually present with pain abdomen, a lump abdomen, anemia, loss of appetite, and menstrual irregularities. On clinical examination, an adnexal mass is usually palpable. Radiology shows the presence of an adnexal lesion, mostly resembling malignancy.[ 6 ] Xanthogranulomatous inflammation was first reported by Kunakemakorn et al . in 1976 in their report on inflammatory pseudotumours in the pelvis. The exact etiopathology is unknown. However, the inflammation is proposed to be secondary to pelvic inflammatory condition, endometriosis, primary infertility, intrauterine contraceptive device, abnormality in lipid metabolism, and ineffective antibiotic therapy. Portela et al . reported a case of xanthogranulomatous salpingoophoritis in a 35-year-old woman with endometriosis, suffering from chronic pelvic pain and long-standing infertility.[ 7 ] Most of the literature has reported affection of only one ovary or the fallopian tube alone.[ 1 2 4 6 ] The destructive nature of the disease, as well as the tendency to form mass, makes it very difficult to distinguish it from malignancy, both clinically and radiologically. In our case, the patient presented with an adnexal mass and was operated on the suspicion of a malignant ovarian tumor. However, it was an intraoperative surprise to find an inflammatory adnexal cyst with dense adhesions in the peritoneal cavity with minimal changes in the ovaries. Diagnosing the disease clinically is challenging, as seen in our case, and the final diagnosis can be made only with histopathological examination.