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BMC Pulmonary Medicine

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Open Access 01-12-2016 | Case report

Coexistence of pulmonary lymphangioleiomyomatosis and pulmonary angiomyolipoma

Authors: Xuefeng Sun, Ruie Feng, Ye Zhang, Juhong Shi, Kai-Feng Xu

Published in: BMC Pulmonary Medicine | Issue 1/2016

Abstract

Background

Lymphangioleiomyomatosis (LAM) and angiomyolipoma are two different, but related rare diseases. To the best of our knowledge, pulmonary LAM and pulmonary angiomyolipoma have not previously been observed in the same patient.

Case presentation

A 38-year-old woman presented with a dry cough and left flank pain. She had a right nephrectomy for renal angiomyolipoma 17 years ago. A magnetic resonance imaging scan demonstrated a round mass in the left kidney. A chest computed tomography scan demonstrated scattered small thin-walled cysts and multifocal round nodules in both lungs. A lung biopsy via video-assisted thoracoscopic surgery revealed that the cysts and nodules were manifestations of LAM and angiomyolipomas, respectively. After sirolimus therapy, the renal angiomyolipoma and metastasized pulmonary angiomyolipomas shrank, but pulmonary cysts were unchanged.

Conclusions

LAM and angiomyolipoma are significantly associated, and may coexist in the lungs in rare cases. Sirolimus is effective for both renal angiomyolipoma and metastasized pulmonary angiomyolipomas.

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Title: Coexistence of pulmonary lymphangioleiomyomatosis and pulmonary angiomyolipoma
Authors: Xuefeng Sun
Ruie Feng
Ye Zhang
Juhong Shi
Kai-Feng Xu
Publication date: 01-12-2016
Publisher: BioMed Central
Published in: BMC Pulmonary Medicine / Issue 1/2016
Electronic ISSN: 1471-2466
DOI: https://doi.org/10.1186/s12890-016-0286-1

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