Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases.
- Author:
Dae Hyun SONG
1
;
In Ho CHOI
;
Sang Yun HA
;
Kang Min HAN
;
Jae Jun LEE
;
Min Eui HONG
;
Yoon La CHOI
;
Kee Taek JANG
;
Sang Yong SONG
;
Chin A YI
;
Joungho HAN
Author Information
1. Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. hanjho@skku.edu
- Publication Type:Original Article
- Keywords:
Lymphangioleiomyomatosis;
Abdomen;
Pelvis;
Lymph nodes;
Recurrence
- MeSH:
Abdomen;
Diagnosis, Differential;
Female;
Humans;
Lung;
Lymph Nodes;
Lymphangioleiomyomatosis;
Lymphangiomyoma*;
Mediastinum;
Pelvis;
Recurrence;
Tuberous Sclerosis
- From:Korean Journal of Pathology
2014;48(3):188-192
- CountryRepublic of Korea
- Language:English
-
Abstract:
BACKGROUND: Lymphangioleiomyomatosis (LAM) is a slowly progressive neoplastic disease that predominantly affects females. Usually, LAM affects the lung; it can also affect extrapulmonary sites, such as the mediastinum, the retroperitoneum, or the lymph nodes, although these locations are rare. A localized form of LAM can manifest as extrapulmonary lesions; this form is referred to as extrapulmonary lymphangioleiomyoma (E-LAM). Due to the rare occurrence of E-LAM and its variable, atypical location, E-LAM is often difficult to diagnose. Herein, we report the clinicopathological information from four E-LAM cases, and also review previous articles investigating this disease. METHODS: Four patients with E-LAM were identified at the Samsung Medical Center (Seoul, Korea) from 1995 to 2012. All E-LAM lesions underwent surgical excision. RESULTS: All patients were females within the age range of 43 to 47 years. Two patients had para-aortic retroperitoneal masses, while the other two patients had pelvic lesions; two out of the four patients also had accompanying pulmonary LAM. In addition, no patient displayed any evidence of tuberous sclerosis. Histologically, two patients exhibited nuclear atypism with cytologic degeneration. CONCLUSIONS: E-LAM should be considered in the differential diagnosis of patients presenting with pelvic or para-aortic masses. We also conclude that further clinical and pathological evaluation is needed in patients with E-LAM and nuclear atypism.
