Lymphangiomyomatosis Arising in the Pelvic Cavity : A Case Report

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease usually occurring in young women of child-bearing age. It is characterized by a distinctive proliferation of lymphatic smooth muscle cells, especially occurring in the pulmonary parenchyme. The majority of primary LAM occurs in the lung, but there are a few reports of extrapulmonary cases. We report a case of a 21-yr-old female who first complained of low abdominal pain and was referred from a local clinic with the impression of an ovarian cyst. Gynecologic ultrasonography revealed a large posterior pelvic mass with an irregular echogenicity measuring 9.7×4.2 cm in size. Pelviscopy showed a large, thin walled, partly cystic, pelvic mass. The mass was partly removed. Microscopically, the mass was characterized by a haphazard proliferation of smooth muscle cells arranged in fascicular, trabecular, and papillary patterns around a ramifying network of endothelium-lined spaces. The cells were plump or epithelioid with abundant eosinophilic cytoplasm and showed a positive reaction for both alpha-smooth muscle actin and HMB-45 antigen. Surgical and pathological findings were consistent with pelvic retroperitoneal LAM. Despite the numerous treatment attempts, the patient suffered from intractable chylous ascites and developed pulmonary LAM and died due to severe respiratory distress.

Primary retroperitoneal lymphangioleiomyomatosis in a postmenopausal woman: a case report and review of the literature

“…In a pathologic analysis of 188 cases, extrapulmonary LAM without coexisting lung involvement was identified in only three patients (2%) (3) . After reviewing the literature, we identified 21 patients who did not have lung involvement at the time of diagnosis; however, many of these patients were later diagnosed with pulmonary LAM (Table 1) (6)(7)(8)(9) . Our case exhibited no clinical or radiologic features of lung involvement; the presenting feature was an incidental finding on CT scan during evaluation of abnormal uterine bleeding.…”

A case of lymphangioleiomyomatosis originated in the pelvic cavity

“…According to Matsui K et al 3 , the diagnosis of pulmonary lymphangioleiomyomatosis is established after that of extrapulmonary lymphangioleiomyomatosis, usually within 2 years. Also, Kim HS et al 8 reported a case of extrapulmonary lymphangioleiomyomatosis in which chylous pleural effusion was also identified two months after the diagnosis. Therefore, extrapulmonary lymphangiomyomatosis should be carefully followed up to discover any lung lesions that develop after the initial diagnosis.…”

Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases

“…The three primary locations of the E-LAM lesions were the posterior mediastinum, the upper retroperitoneum adjacent to the abdominal aorta, and the pelvic cavity. To the best of our knowledge, only two previous reports have described E-LAM cases in Korea 7,8. The first case of E-LAM was reported by Kim et al7 in 2005, and a second case was reported by Han et al8 in 2008.…”