Isolated Nodal Retroperitoneal Sporadic Lymphangioleiomyomatosis
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.
| Main Authors: | , , , |
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| Format: | Digital revista |
| Language: | English |
| Published: |
Sociedade Portuguesa de Medicina Interna
2020
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| Online Access: | http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-671X2020000500007 |
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