Retroperitoneal Lymphangioleiomyomatosis without Pulmonary Feature.
- Author:
Hyun Ah KIM
1
;
Joo Ho LEE
;
Seog Ki MIN
Author Information
1. Department of Surgery, Korea Cancer Center Hospital, Korea Institution of Radiology and Medical Science, Korea.
- Publication Type:Case Report
- Keywords:
Lymphangioleiomyomatosis;
Retroperitoneal mass;
Laparoscopic surgery;
Pulmonary symptom
- MeSH:
Adrenal Glands;
Adult;
Dyspnea;
Female;
Humans;
Laparoscopy;
Lymphangioleiomyomatosis*;
Pleural Effusion;
Pneumothorax
- From:Journal of the Korean Surgical Society
2007;73(4):334-336
- CountryRepublic of Korea
- Language:English
-
Abstract:
Lymphangioleiomyomatosis (LAM) is discovered mainly in the pulmonary area, and the typical presenting symptoms include progressive dyspnea, pneumothorax, and chylous pleural effusion. An initial presentation of LAM with extrapulmonary symptoms is extremely rare. Most reports and reviews have concentrated on the pulmonary symptoms and radiological image of LAM. Some of reports focused on the abdominal abnormalities or retroperitoneal abnormalities in addition to pulmonary features. However, LAM without pulmonary features is extremely rare. We encountered a 36-year-old woman with a retroperitoneal mass measuring 7 cm in the anterior aspect of the right adrenal gland. The patient did not have any pulmonary symptoms or radiological abnormalities, and underwent a laparoscopic excision. The tumor was diagnosed histologically as a lymphangioleiomyomatosis. LAM without pulmonary feature is unusual case. We report this case with a review of the relevant literature.
