Journal
AMERICAN JOURNAL OF SURGICAL PATHOLOGY
Volume 35, Issue 12, Pages 1776-1785Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/PAS.0b013e318235edbd
Keywords
lymphangioleiomyomatosis; uterus; PEComa
Funding
- High Technology Research Center from the Ministry of Education, Culture, Sports, Science, and Technology, Japan
- Ministry of Health, Labour and Welfare, Japan
- Institute for Environmental and Gender-Specific Medicine, Juntendo University, Graduate School of Medicine
- [18390243]
- [19590406]
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Lymphangioleiomyomatosis (LAM), a systemic disorder affecting almost exclusively young women, is characterized by the abnormal proliferation of smooth muscle-like cells (LAM cells). LAM can occur either in association with the tuberous sclerosis complex (TSC) (TSC-LAM) or without TSC (sporadic LAM). Recent studies have demonstrated that LAM is a neoplasm arising from constitutive activation of the mammalian target of rapamycin signaling pathway dysregulated by a functional loss of TSC genes, but the primary organ of origin remains unclear. Therefore, we performed histologic and immunohistologic analyses of gynecologic organs in 20 patients, half with and the other half without pulmonary LAM, to determine how often LAM involves the uterus. The results showed that 9 of 10 (90%) patients with pulmonary LAM had uterine LAM lesions. In contrast, no patients without pulmonary LAM had so. All uterine LAM lesions were accompanied by LAM lesions in retroperitoneal or pelvic lymph nodes and LAM cell clusters, each enveloped by a monolayer of vascular endothelial growth factor receptor-3-positive lymphatic endothelial cells. Furthermore, when we compared uterine lesions of TSC-LAM with those of sporadic LAM, proliferation of HMB45-positive epithelioid-shaped LAM cells and infiltrates with a tongue-like growth pattern was more prominent in the former, whereas the extent of lymphangiogenesis within the myometrium was greater in the latter. These results indicate that uterine involvement is a common manifestation of LAM, and, possibly, that the uterus or an adjacent locale in the retro-peritoneum or pelvic cavity is the primary site of origin of LAM.
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