Isolated Nodal Retroperitoneal Sporadic Lymphangioleiomyomatosis

Abstract

Lymphangioleiomyomatosis is a rare disease (1/1 000 000) with multisystemic involvement, most commonly presenting with cystic lung disease. Reports of isolated nodal lymphangioleiomyomatosis rare. Its treatment and prognosis are not well established. We describe a 48-year-old female patient presenting with ureter compression by an extensive retroperitoneal mass. Biopsy showed nodal lymphangioleiomiomatosis. A partial surgical resection of the lesions was performed. She is under yearly thoraco-abdomino-pelvic computed tomography control, last performed 3 years after diagnosis, with no lung involvement or new abdominal lesions. She evolved to menopause after 6 months. Some case series suggest that isolated nodal lymphangioleiomyomatosis may precede lung involvement by one to two years, having size as a risk factor (>10 mm). Relative stabilization of the disease course in postmenopausal women is described. This case reports an atypical evolution, with two >10mm diameter lesion, with 3-year follow-up without pulmonary, renal or other forms of lymphatic involvement.