Childhood asymmetric labium majus enlargement (CALME) couldrepresent 22% of all pediatric vulvar soft tissue masses and 3% of all vulvar lesions biopsied. It is a distinctive clinicopathologic entity of pre- and early puberty.
Synopsis
Age range: 3.9 to 13.2 years
enlargement of 1 or occasionally both labia majora
expansion of the labium majus without definable borders
fibro-fatty tissue from 2 to 8 cm in greatest dimension
usual constituents of vulvar soft tissue, with expansion of the fibrous component
sparsely to moderately cellular interconnected bands encircled lobules of fat, blood vessels, and nerves
bands consisted of plump and occasionally stellate or round fibroblasts immersed in an abundant pale myxoid matrix containing thin collagen fibers
fibrous bands merged with thinner denser fibrous septa simlar to those seen in the vulva from age-matched controls
variably abundant thin parallel elastic fibers
Immunochemistry
fibroblasts immunohistochemically positive for estrogen and progesterone receptors
Ultrastructure
fibroblasts with dilated rough endoplasmic reticulum cisternae and prominent nuclear fibrous laminae
extracellular matrix contained precollagen, collagen, elastic fibers, and numerous proteoglycan granules
Cytogenetics
normal karyotype
Prognosis
Local recurrence: 50%
References
Vargas SO, Kozakewich HP, Boyd TK, Ecklund K, Fishman SJ, Laufer MR, Perez-Atayde AR. Childhood asymmetric labium majus enlargement: mimicking a neoplasm. Am J Surg Pathol. 2005 Aug;29(8):1007-16. PMID: #16006794#