A 41-year-old Malay housewife presented with recurrent chylous ascites and progressive cachexia over 17 years. A diagnosis of lymphangioleiomyomatosis (LAM) was established by laparotomy where biopsy of the liver, peritoneum and adhesions from previous surgery showed smooth muscle proliferation in the blood vessels and lymphatics. Clinically and radiologically, there was no evidence of pulmonary involvement. She was treated with dietary fat restriction and medium-chain triglycerides. This is the first case of LAM reported in Malaysia.
This case represents a unique primary ovarian tumour consisting of malignant mucinous elements and granulosa-theca-cell elements, the histogenesis of which remains uncertain. It also underscores the need for thorough sampling of mucinous tumours in order to discover a possible coexisting, different neoplastic component.