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Murine Bilateral Renal Lymphadenectomy
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[Retroperitoneal lymphangioleiomyomatosis - a case reports].

E Lajtman1, M Mlynček, M Matejka

  • 1Gynekologicko-Pôrodnická FN a Univerzita Knostantína Filozofa, Nitra, prednosta prof. MUDr. M. Mlyncek, CSc. lajtmanerik@gmail.com

Ceska Gynekologie
|May 29, 2013
PubMed
Summary

Lymphangioleiomyomatosis (LAM) is a rare lung disease in women. This report details two extrapulmonary LAM cases in the pelvis, treated with progesterone and sirolimus.

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Area of Science:

  • Oncology
  • Pulmonology
  • Gynecology

Background:

  • Lymphangioleiomyomatosis (LAM) is a rare, progressive disorder primarily affecting women of childbearing age.
  • Characterized by abnormal smooth muscle cell proliferation, predominantly in the lungs, leading to respiratory failure and often death.

Observation:

  • Extrapulmonary LAM commonly presents with abdominal mass, pain, and chylous ascites.
  • Two cases of premenopausal females with extrapulmonary LAM in pelvic locations (obturator fossa, external iliac artery) are reported.

Findings:

  • Surgical intervention was performed in both reported cases.
  • Primary treatment involved progesterone, with sirolimus used as a second-line therapy.

Implications:

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  • These cases highlight pelvic locations for extrapulmonary LAM.
  • The treatment approach suggests a potential therapeutic strategy involving progesterone and sirolimus for extrapulmonary LAM.