Pseudoxanthomatous Salpingitis: A Rare Granulomatous Pathology of the Fallopian Tube

ABSTRACT

Uncommon presentations of granulomatous salpingitis are pseudoxanthoma salpingitis (PSX) and xanthogranulomatous salpingitis (XGS). The clinical features and image modalities can mimic ovarian malignancy. Thus, a proper pre-operative evaluation of this entity is vital to avoid radical treatment. Here, we report a case of a 31-year-old woman with primary infertility diagnosed with pseudoxanthoma salpinx. This patient was a known case of endometriosis and presented with gross ascites.

INTRODUCTION

Pseudoxanthoma salpingitis (PXS) is an uncommon condition of the fallopian tube characterised by the presence of haemosiderin-laden pigmented histiocytes within the lamina. PXS is commonly linked with ovarian endometriosis[1,2,3,4] and following pelvic irradiation.[5] PXS destroys the involved tissues. The pathophysiology of this condition is still unclear but may be explained by the response shown by the macrophages to haemorrhage in the fallopian tube lumen.

Clinically, xanthogranulomatous inflammation is seen as a lesion that looks like a mass in the pelvic cavity invading the adjacent tissues, which mimics malignant lesions clinically and radiologically.[1,2,3] This form of chronic inflammation is very rare and most commonly seen in the gall bladder and kidney.[3] Very few cases of xanthogranulomatous salpingitis have been reported in the literature, most of which were related to pelvic inflammatory disease.[4] Xanthogranulomatous salpingitis could be distinguished from pseudoxanthomatous inflammation by the presence of pigmented haemosiderin-laden histiocytes in the distended plicae without any inflammatory cells.[5,6,7,8]

Here, we present a case of pseudoxanthoma salpingitis which occurred on a background of endometriosis. The patient presented with recurrent ascites, which is an unusual presentation secondary to endometriosis.

CASE REPORT

A 31-year-old woman with primary infertility underwent laparoscopic bilateral chocolate cystectomy and myomectomy elsewhere. Postsurgery, she developed persistent abdominal distension. A computed tomography scan showed minimal ascites with a small haemorrhagic cyst on the left ovary. The ascitic-fluid analysis was reported as suspicious of pseudomyxoma peritonitis. A repeat analysis gave an inconclusive report, and empirical anti-tuberculosis treatment (AKT) was given for 6 months.

The patient was referred to our centre in view of the increase in ascites from moderate to severe in a few months. Blood investigations were normal. She was taken up for laparoscopy. The Palmer’s point port entry was difficult due to adhesions with dense loculated haemorrhagic ascites hence converted to Hassan’s open entry [Figure 1]. Intraoperatively massive haemorrhagic ascites were seen involving the whole pelvic and abdominal cavities; the bladder was pulled up, adherent to a mobility-restricted uterus. B/L fallopian tubes had unhealthy surfaces and were seen as tubo-ovarian masses. Ascitic fluid was suctioned out, and bilateral tubal clipping was done. Biopsies taken from multiple sites, such as the peritoneum, tubes, endometrium and omentum, were sent for histopathology.

Figure 1.

Laparoscopy picture after ascites aspiration showing bilateral tubo-ovarian mass with unhealthy tubal surface

The histopathology showed expansion of tubal plicae by a collection of macrophages, and congested vessels suggestive of pseudoxanthomatous salpingitis [Figures 2 and 3]. Ascitic fluid had few scattered lymphoid cells and red blood cells with no endometriotic spots/malignancy. Postoperative recovery was good; downregulation with Gonadotropin-releasing hormone (GnRH) analogues followed by ART was planned.

Figure 2.

Histopathology picture showing pseudoxanthoma salpinx

Figure 3.

Histopathology picture showing macrophages and congestion

Downregulation with four doses of GnRH agonist (injection leuprolide 3.75 mg) was given, followed by an antagonist protocol for ovarian stimulation. Eleven oocytes were retrieved, and six embryos were formed, which were cryopreserved. A repeat scan after 4 weeks showed re-accumulation of more than 1000 mL fluid occupying all compartments, but the patient was asymptomatic. Three more doses of agonist suppression were given, which did not reduce the ascites, and 500 mL of ascitic fluid was aspirated transvaginally.

A downregulated FET was performed, and the patient had a positive Beta-human chorionic gonadotropin (hCG) with twin sacs. The antenatal period was uneventful. At 35 weeks, elective lower segment cesarean section (LSCS) was done for oligohydramnios with breech presentation of both twins. Both babies were healthy. Postoperatively, the mother had a good recovery.

DISCUSSION

Furuya et al., in 2002,[1] proposed that although the tubal plicae are expanded with numerous histiocytes in both xanthogranulomatous and pseudoxanthogranulomatous salpingitis, the histiocytes are different in these two lesions. The cells in PXS have brown cytoplasmic lipofuscin, whereas the cells (histiocytes) in xanthogranulomatous salpingitis have foamy vacuolated cytoplasm with multiple inflammatory calls. A clinical history of pelvic inflammatory disease or pyometra is supportive of xanthogranulomatous salpingitis.

Eight of the 11 cases of PXS reported were associated with endometriosis, and none had a history of Pelvic inflammatory disease (PID).[1] Seidman and Woodburn in 2015 characterised the clinical features and pathological findings in 49 cases with PXS and established its close relation with endometriosis with the presence of iron in histiocytes.[9] Few authors classify both under xanthogranulomatous salpingitis.[10,11]

The association of pseudoxanthoma salpingitis in women with endometriosis is indeed a rare phenomenon and is not seen in the majority of young females with pelvic endometriosis. The possible mechanism could be bleeding from endometriosis, which causes accumulation of foamy macrophages.[7,12,13]

Recurrent haemorrhagic ascites are rarely known to occur with endometriosis.[13] The most common causes of haemorrhagic ascites are ruptured haemorrhagic cysts, ruptured ectopic, acute pancreatitis, Meigs syndrome or splenic rupture, which were excluded in this case through imaging and other modalities. Recurrent ascites in our patient led to diagnostic laparoscopy, where histological diagnosis of pseudoxanthoma salpingitis was made.

Endometriosis as a cause of ascites is usually a diagnosis of exclusion. The definitive diagnosis is made intraoperatively. The diagnosis of peritoneal endometriosis through cytology can be difficult because the acquired samples of ascitic fluid may reveal only nonspecific haemosiderin or lipofuscin-laden macrophages. Whereas, peritoneal biopsy histology may be of more help as they would reveal more hyperplastic endometrial cells embedded within a dense stroma.

CONCLUSION

The diagnosis of pseudoxanthoma salpingitis is rare and remains a histological diagnosis; however, it is important to know its correlation to endometriosis. Knowledge about this rare entity is important for clinicians, pathologists and radiologists since the features mimic malignant lesions. Endometriosis should be considered in cases of recurrent haemorrhagic ascites once the other causes are excluded. Hence, a proper history, radiological examination, pre-operative diagnosis and an intraoperative evaluation may lead to less aggressive management.

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.

Data Availability Statement

Data will be available from corresponding author upon reasonable request.