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Retroperitoneal lymphangioleiomyomatosis associated with endosalpingiosis: Case report

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Fukunaga M, Mistuda A, Shibuya K, Honda Y, Shimada N, Koike J, Sugimoto M. Retroperitoneal lymphangioleiomyomatosis associated with endosalpingiosis. APMIS 2007;115:1460–5.

A case of retroperitoneal lymphangioleiomyomatosis (LAM) arising from endosalpingiosis is described. A 25-year-old woman with no history of tuberous sclerosis or hormonal therapy presented with a painless, palpable abdominal mass. Computed tomographic and magnetic resonance imaging studies of the abdomen demonstrated a 4 cm cystic mass in the retroperitoneum. Macroscopically, the excised retroperitoneal cyst was multilocular and measured 4.0×3.5×3.5 cm. Histologically, the lesion demonstrated three components. The first comprised multiple cysts or glands lined by columnar epithelial cells with cilia. The second component was a condensation of small stromal cells immediately subjacent to the cystic epithelium or glands. The third component was a thick exterior wall composed of plump spindle cells with clear to palely eosinophilic cytoplasm in a fascicular pattern, and slit-like vascular spaces, resembling LAM. Immunohistochemically, the epithelium and glands were positive for cytokeratin 7. The stromal cells were positive for vimentin and CD10. The cells of the LMA-like component showed positive staining for HMB45, alpha-smooth muscle actin, muscle actin and h-caldesmon. The lesion, LAM arising from endosalpingiosis, represents a distinctive pathologic entity that should be recognized and studied further. This type of lesion should be included in the differential diagnosis of retroperitoneal cystic lesions.

Keywords: Lymphangioleiomyomatosis; endosalpingiosis; retroperitoneum

Document Type: Research Article


Affiliations: 1: Pathology 2: Surgery, Toho University Medical Center, Ohomori Hospital Tokyo, Japan

Publication date: December 1, 2007


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