Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases
Korean Journal of Pathology
;
: 188-192, 2014.
Artículo
en Inglés
| WPRIM
| ID: wpr-207975
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.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Pelvis
/
Recurrencia
/
Esclerosis Tuberosa
/
Linfangioleiomiomatosis
/
Diagnóstico Diferencial
/
Abdomen
/
Pulmón
/
Ganglios Linfáticos
/
Linfangiomioma
/
Mediastino
Tipo de estudio:
Estudio diagnóstico
/
Estudio pronóstico
Límite:
Femenino
/
Humanos
Idioma:
Inglés
Revista:
Korean Journal of Pathology
Año:
2014
Tipo del documento:
Artículo
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