A case of lymphangioleiomyomatosis originated in the pelvic cavity

Abstract

Lymphangioleiomyomatosis is a rare disease that is characterized by proliferation of abnormal smooth muscle-like cells, especially that which occurs in the pulmonary parenchyme. It primarily affects women of child-bearing age. The majority of primary lymphangioleiomyomatosis occurs in the lung, but there are a few reports of extrapulmonary cases. We experienced a rare case of lymphangioleiomyomatosis which originated in the pelvic cavity (in the posterior portion of the uterus), and report with brief review of literatures.

Keywords: Lymphangioleiomyomatosis; Pelvis; Uterus.

Fig. 1

Between the uterus and the right ovary, 5.57×2.91 cm sized solid mass like shadow is seen with high vascularity.

Fig. 2

About 4.0×5.0×4.0 cm sized lobulated mass (☆) is seen in the right posterolateral portion of the uterus. This mass shows low signal intensity on T1W1 (A) and intermediate signal intensity on T2W1 (B). The mass abuts the middle portion of the uterus.

Fig. 3

The tumor is composed of spindle cells, which are arranged in short fascicles around the dilated lymphatic vessels (H&E, ×100). The tumor cells are plump with abundant eosinophilic cytoplasm and nuclei devoid of pleomorphism and arranged around a ramifying network of endothelium-lined spaces (H&E, ×200)(*).

Fig. 4

By the immunohistochemical stainings, those tumor cells are positive for smooth muscle actin (A) and multifocally positve for HMB45 (B), consistent with lymphangiomyomatosis (immunostains, ×100).