Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases
송대현, 최인호, 한강민, 이재준, 홍민의, 이진아, 한정호, 하상윤, 최윤라, 장기택, 송상용,
소속 상세정보
송대현 ( Song Dae-Hyun )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
최인호 ( Choi In-Ho )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
한강민 ( Han Kang-Min )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
이재준 ( Lee Jae-Jun )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
홍민의 ( Hong Min-Eui )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
이진아 ( Yi Chin-A )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Radiology
한정호 ( Han Joung-Ho )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
하상윤 ( Ha Sang-Yun )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
최윤라 ( Choi Yoon-La )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
장기택 ( Jang Kee-Taek )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
송상용 ( Song Sang-Yong )
Sungkyunkwan University School of Medicine Samsung Medical Center Department of Pathology
KMID : 0357920140480030188
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 ELAM 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.
키워드
Lymphangioleiomyomatosis; Abdomen; Pelvis; Lymph nodes; Recurrence
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