J Gynecol Oncol.  2008 Sep;19(3):195-198. 10.3802/jgo.2008.19.3.195.

A case of lymphangioleiomyomatosis originated in the pelvic cavity

• Han JM
• Lee KH
• Kim SJ
• Rhim CC
• Park YH
• Kang JB
• Jeon SY

• KMID: 2173402
• DOI: http://doi.org/10.3802/jgo.2008.19.3.195

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.

Keyword

Lymphangioleiomyomatosis; Pelvis; Uterus

MeSH Terms

Female
Humans
Lung
Lymphangioleiomyomatosis
Pelvis
Rare Diseases
Uterus

Figure

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  Fig. 1 Between the uterus and the right ovary, 5.57×2.91 cm sized solid mass like shadow is seen with high vascularity.

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  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.

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  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)(*).

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  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).

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