Retroperitoneal lymphangiomyoma is rare abdominal finding of lymphangiomyomatosis (LAM). We report here on a case of retroperitoneal lymphangiomyoma and this is the first case that? been seen in the Korean literature. Retroperitoneal lymphangiomyoma associated with pulmonary LAM has rarely been reported in radiologic literature. The charateristic findings of this case are a prominent solid component and retroperitoneal lymphadenopathy mimicking a malignant tumor. A 45-year-old woman was admitted for further evaluation of a lower abdominal mass that could be palpated for 2 weeks. US findings showed relatively well-defined mass with septated cystic portions and echogenic solid portions in the lower abdomen. CT findings revealed a well-demarked retroperitoneal mass with septated cystic portions and enhancing solid portions at the right lower abdomen, and there were multiple retroperitoneal lymphadenopathy at the lower abdomen. The patient underwent a mass excision. The pathologic findings were retroperitoneal lymphangiomyoma with multiple lymph node involvement.
Abdomen ; Female ; Humans ; Lymph Nodes ; Lymphangioleiomyomatosis* ; Lymphangiomyoma* ; Lymphatic Diseases ; Middle Aged

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.
Abdomen ; Diagnosis, Differential ; Female ; Humans ; Lung ; Lymph Nodes ; Lymphangioleiomyomatosis ; Lymphangiomyoma* ; Mediastinum ; Pelvis ; Recurrence ; Tuberous Sclerosis

Chylopericardium is a rare disease entity characterized by the accumulation of chylous fluid in the pericardial sac. It usually arises from mediastinal neoplasms, thrombosis of the subclavian vein, tuberculosis, nonsurgical trauma, thoracic or cardiac surgery. The spectrum of symptoms for chylopericardium varies from an incidental finding of cardiomegaly to dyspnea, upper abdominal discomfort, cough, chest pain, palpitation, fatigue. However, most of the patients are asymptomatic. The main purpose of treatment of chylopericardium is the prevention of cardiac tamponade and prevention of metabolic, nutritional, and immunological compromise due to chyle leak. Here, we report a case of chylopercardium secondary to lymphangiomyoma with review of the literature.
Cardiac Tamponade ; Cardiomegaly ; Chest Pain ; Chyle ; Cough ; Dyspnea ; Fatigue ; Humans ; Incidental Findings ; Lymphangioma ; Lymphangiomyoma ; Mediastinal Neoplasms ; Pericardial Effusion ; Rare Diseases ; Subclavian Vein ; Thoracic Surgery ; Thorax ; Thrombosis ; Tuberculosis

Pulmonary lymphangioleiomyomatosis has been observed almost exclusively in women, usually in their reproductive years. Exacerbations with pregnancy and after hormonal manipulation have been documented, and it has been suggested that its pathogenesis is due to the influence of hormonal(estrogenic) stimulus. The clinical, roentgenographic, and histopathologic features of this case of pulmonary lymphangioleiomyomatosis in a 22-year-old male are all characteristic of those described in prior reports, except for the patient's sex. With the following case of pulmonary lymphangioleiomyomatosis in a male, we suggest the possibility of the existence of an additional pathogenetic mechanism.
Adult ; Emphysema/complications ; Humans ; Lung Neoplasms/complications/etiology/*pathology ; Lymphangiomyoma/complications/etiology/*pathology ; Lymphatic System/pathology ; Male ; Muscle, Smooth/pathology ; Thoracotomy/adverse effects ; Tuberculosis, Pulmonary/complications