A rare case of ovarian Lymphangioma associated with chylous ascites and raised CA-125.

Lymphangiomas are benign congenital malformations of lymphatic system, thought to happen due to obstruction of local lymph flow system. They can occur anywhere in the skin and the mucous membranes. Most common sites are the head and the neck but sometimes, they can be found in the intestines, the pancreas and the mesentery. Lymphangioma of the ovary is an extremely rare lesion. It was first described in 1908. Clinical manifestations can vary from asymptomatic ovarian mass to acute abdomen. They may be very frequently confused with malignant ovarian mass often leading to extensive surgery. A 34-year-old female presented with dull aching pain localized to lower abdomen from last 6 months. Ultrasound and CECT abdomen were suggestive of enlarged right ovary with massive ascites. Ca-125 was 685. Diagnostic laparoscopy revealed chylous ascites and further a frozen section revealed few atypical cells, further strengthening the diagnosis of malignant tumor. Staging laparotomy with total abdominal hysterectomy and salpingo-oophorectomy was finally done. 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 tumors in order to avoid overtreatment. However, a careful follow up for at least 2 years is needed for patients with lymphangioma of the ovary in order to exclude recurrence.