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childhood asymmetric labium majus enlargement

 

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#



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