title: Retroperitoneal lymphangioleiomyomatosis associated with endosalpingiosis.
abstract: 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 x 3.5 x 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.
authors.affiliation: Department of Pathology, Jikei University School, Tokyo, Japan. maasafu@jikei.ac.jp
authors.forename: Masaharu
authors.initials: M
authors.lastname: Fukunaga
authors.forename: Aki
authors.initials: A
authors.lastname: Mistuda
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Shibuya
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Honda
authors.forename: Nagato
authors.initials: N
authors.lastname: Shimada
authors.forename: Junichi
authors.initials: J
authors.lastname: Koike
authors.forename: Motonobu
authors.initials: M
authors.lastname: Sugimoto
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D005184:Fallopian Tube Diseases / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D018192:Lymphangioleiomyomatosis / Q000378:metabolism / Q000473:pathology*; D008202:Lymphangioma / Q000473:pathology; D010052:Ovariectomy; D011247:Pregnancy; D011271:Pregnancy, Ectopic / Q000473:pathology / Q000601:surgery; D012186:Retroperitoneal Neoplasms / Q000378:metabolism / Q000473:pathology*