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Case Reports
. 2019 Jul-Aug;36(4):349-352.
doi: 10.4103/lungindia.lungindia_433_18.

Low-dose sirolimus in retroperitoneal lymphangioleiomyomas

Kamonpun Ussavarungsi  1 , Archana T Laroia  2 , Charles D Burger  3
Affiliations
Case Reports

Low-dose sirolimus in retroperitoneal lymphangioleiomyomas

Kamonpun Ussavarungsi et al. Lung India. 2019 Jul-Aug.

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease associated with cystic lung destruction and abdominal tumors, including lymphangioleiomyomas, which frequently occur in the retroperitoneal region. Sirolimus therapy is currently recommended for LAM patients with abnormal or declining lung function with an adjusted dose to maintain a serum trough level of 5-15 ng/mL. We describe a significant reduction of retroperitoneal lymphangioleiomyomas after treatment with low-dose sirolimus therapy (serum trough level <5 ng/mL) in a patient with sporadic LAM.

Keywords: Low-dose sirolimus; lymphangioleimyomas; lymphangioleiomyomatosis.

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Conflict of interest statement

None

Figures

Figure 1
Figure 1
(a) Computed tomography chest demonstrating multiple small cystic lesions throughout the lungs. (b and c) Large multicystic retroperitoneal mass, measuring 26 cm × 14 cm × 8 cm, extending from the level of the superior mesenteric artery and renal arteries, encasing the aorta and inferior vena cava to bifurcation and further extending into the pelvis along the right external and internal iliac arteries. (d) Nearly complete resolution of retroperitoneal lymphangioleiomyomas after 14 months of sirolimus
See this image and copyright information in PMC

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