Retroperitoneálna lymfangioleiomyomatóza - kazuistiky
[Retroperitoneal lymphangioleiomyomatosis - a case reports]
Language Czech Country Czech Republic Media print
Document type Case Reports, Journal Article
PubMed
23710987
PII: 40562
- MeSH
- Adult MeSH
- Humans MeSH
- Lymphangioleiomyomatosis * MeSH
- Progesterone therapeutic use MeSH
- Antineoplastic Agents therapeutic use MeSH
- Retroperitoneal Neoplasms * MeSH
- Sirolimus therapeutic use MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
- Names of Substances
- Progesterone MeSH
- Antineoplastic Agents MeSH
- Sirolimus MeSH
Lymphangioleiomyomatosis (LAM) is a rare progressive disease affecting women of childbearing age. The disease is characterised by an abnormal proliferation of immature smooth muscle cells predominantly in the lung. It gradually leads to respiratory failure, and it frequently result in death. Extrapulmonary LAM typically presents with abdominal mass, abdominal pain and chylous ascites. In the case reports we describe two cases of premenopausal females with extrapulmonary LAM. In both cases they occur in pelvic location in the obturator fossa and around the external iliac artery. After surgical procedures patients were primary treated with progesterone. Sirolimus was second-line drugs.