A 6-year-old girl with vaginal spotting who was diagnosed wi
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Introduction
Perivascular epithelioid cell neoplasms (PEComas) were first described by Bonetti et al. in 1992. They are rare soft tissue neoplasms with histologically and immunohistochemically distinct perivascular epithelioid cells. They present with clear and acidophilic cytoplasm, perivascular distribution, and immunoreactivity for melanocytic markers such as HMB-45 . They have been described in the liver, lung, pancreas, rectum, and female genital tract . There have been four reported cases of vaginal PEComas in the literature, two of which were in pediatric patients. Here, we present an extremely rare case of a vaginal PEComa in a 6-year-old girl.

Case report
A 6-year-old girl presented with intermittent vaginal spotting for 6 months. Her urinalysis at a local clinic showed a 2+ positive occult blood test without any other abnormality. The patient was referred to our hospital for further evaluation. She had been diagnosed with pineoblastoma at the age of 2, for which she received three cycles of neoadjuvant chemotherapy and underwent tumor resection. She has been followed up regularly, and her pineoblastoma has been in complete remission without the need for any additional treatment. Her family history was unremarkable. Physical examination and laboratory results were normal except for the 2+ occult blood result on urinalysis. Follicle-stimulating hormone, luteinizing hormone, estradiol and thyroid-stimulating hormone levels were also normal....

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4175604/
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