Retroperitoneal lymph node lymphangioleiomyomatosis: tumor with an ambiguous clinical course

Lymphangioleiomyomatosis (LAM) is a mesenchymal tumor included in PEComa family. Lung is the most frequent localization of LAM where it has bilateral spread and leads to respiratory failure. Development of LAM can be provided by tuberous sclerosis or it can be of sporadic nature. Extrapulmonary LAM usually develops in posterior mediastinal, retroperitoneal and pelvic lymph nodes. It is intriguing that primary nodal LAM, developed in the absence of pulmonary LAM, encounters relatively rare and discovers incidentally. Despite the well-known relationship of pulmonary LAM with tuberous sclerosis and occurrence of the tumor in young female patients, clinically occult LAM lesions can be encountered in wide age range and usually these aren't associated with tuberous sclerosis. Prognostic significance of incidentally found nodal LAM is still under debate as well as probability of metachronous lung involvement. Here we present small case series of accidentally discovered LAM in retroperitoneal and pelvic lymph nodes of four female patients.