A rare case of ovarian lymphangioma with elevated CA-125 causing pseudo-Meigs’ syndrome

Abstract

Lymphangiomas are benign congenital malformations of the lymphatic  system, thought to occur as a result of obstruction of the local lymph flow system. They can occur anywhere in the skin and mucous membranes. The most common sites are the head and neck, but sometimes they are found in the intestines, pancreas and mesentery. Lymphangioma of the ovary is an extremely rare lesion. It was first described in 1908. Clinical  manifestations can vary, from an asymptomatic ovarian mass to an acute abdomen. These may be confused with malignant ovarian masses, resulting in extensive surgery. A 34-year-old woman presented with dull aching pain localised to the lower abdomen, present for the previous 6 months. Ultrasound and a contrast-enhanced computed tomography scan of the abdomen were suggestive of an enlarged right ovary with massive ascites. The cancer antigen 125 (CA-125) level was 685 units/mL. Diagnostic laparoscopy revealed chylous ascites, and further, a frozen section revealed a few atypical cells, additionally strengthening the  diagnosis of a malignant tumour. Staging laparotomy with total abdominal hysterectomy and salpingo-oophorectomy was finally performed. Histopathology revealed lymphangioma, with no evidence of malignancy. CA-125 also showed a rapid decline following surgery. Lymphangiomas should be included in the differential diagnosis of ovarian cystic masses, especially in patients with chylous ascites. It is very important to discriminate such cases from other malignant tumours in order to avoid overtreatment. However, a careful follow-up for at least 2 years is needed for patients with lymphangioma of the ovary, to exclude recurrence.