Retroperitoneal lymphangioleiomyomatosis associated with endosalpingiosis.
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.
Fukunaga
Masaharu
M
Department of Pathology, Jikei University School, Tokyo, Japan. maasafu@jikei.ac.jp
Mistuda
Aki
A
Shibuya
Kazuhiro
K
Honda
Yoshiko
Y
Shimada
Nagato
N
Koike
Junichi
J
Sugimoto
Motonobu
M
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*