Chylous Cascade: A Rare Case of Primary Abdominal Lymphangioleiomyomatosis Unveiled in the Operating Room

Lymphangioleiomyomatosis is predominantly a pulmonary disease, with only around 100 cases of extrapulmonary LAM reported in the literature. It is characterized by the growth of smooth muscle cells, leading to cystic lung lesions and lymphatic abnormalities. Most cases of LAM involve pulmonary manifestations, and extrapulmonary occurrences are rare. The typical symptoms of LAM include respiratory distress, recurrent pneumothorax, and chylous effusions. Computed tomography (CT) scans often reveal characteristic thin-walled cysts in the lungs. Fewer than 100 cases of purely extrapulmonary LAM have been reported worldwide (as of recent reviews). The pathogenesis of LAM is believed to be influenced by hormonal factors, given its prevalence in women of reproductive age. Immunhistochemistry studies often reveal positive markers for smooth muscle proteins and, in some cases, hormone receptors like estrogen and progesterone. By presenting this case, we aim to contribute to the limited literature on extrapulmonary LAM and raise awareness for timely recognition and diagnosis. This case highlights an unusual presentation of LAM, emphasizing the importance of considering it in differential diagnosis of abdominal masses and lymphatic disorders.