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Retroperitoneal lymphangioleiomyomatosis associated with endosalpingiosis
Author(s) -
FUKUNAGA MASAHARU,
MISTUDA AKI,
SHIBUYA KAZUHIRO,
HONDA YOSHIKO,
SHIMADA NAGATO,
KOIKE JUNICHI,
SUGIMOTO MOTONOBU
Publication year - 2007
Publication title -
apmis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.909
H-Index - 88
eISSN - 1600-0463
pISSN - 0903-4641
DOI - 10.1111/j.1600-0463.2007.00668.x
Subject(s) - pathology , cytokeratin , anatomy , lymphangioleiomyomatosis , eosinophilic , tuberous sclerosis , medicine , lesion , differential diagnosis , desmin , columnar cell , stromal cell , epithelium , vimentin , immunohistochemistry
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.