Abstract

SOEIRO, Bárbara; CORREIA-PINTO, João; VASCONCELOS, Alexandre  and  SALGADO, Matilde. Isolated Nodal Retroperitoneal Sporadic Lymphangioleiomyomatosis. Medicina Interna [online]. 2020, vol.27, n.4, pp.25-28. ISSN 0872-671X.  https://doi.org/10.24950/CC/93/20/4/2020.

Lymphangioleiomyomatosis is a rare disease (1/1 000000) 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 post-menopausal women is described. This case reports an atypical evolution, with two >10mm diameter lesion, with3-year follow-up without pulmonary, renal or other forms of lymphatic involvement.

Keywords : Lymphangioleiomyomatosis; Lymph Nodes; Menopause.

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