Isolated Nodal Retroperitoneal Sporadic Lymphangioleiomyomatosis

• Autor(a) principal: Soeiro,Bárbara
• Data de Publicação: 2020
• Outros Autores: Correia-Pinto,João, Vasconcelos,Alexandre, Salgado,Matilde
• Tipo de documento: Relatório
• Idioma: eng
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-671X2020000500007
• topic: Lymphangioleiomyomatosis; Lymph Nodes; Menopause

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.