Multiple Hybrid Cysts in a Child: Challenge

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A 13-year-old female presented to plastic surgery for removal of three flesh-colored facial nodules with indistinct borders that ranged in size from 5 to 15 mm. Figure 1 shows a representative lesion. The patient has a history of subcutaneous nodules of the face and scalp since infancy and has undergone 2 previous excisions (at ages 4 and 11 years) of these nodules. Histopathologic evaluation of the recently removed nodules revealed cystic lesions lined mostly by squamous epithelium containing hyperkeratotic keratin (Figs. 2A, B). Multiple areas within the cyst walls showed small basaloid epithelial cells adjacent to pale eosinophilic cell outlines (Fig. 2C). The surrounding connective tissue demonstrates a foreign body inflammatory reaction and foci of calcification. These findings are consistent with pathologic findings from previous excisions.
When the patient was 6 year old, her mother was diagnosed with familial adenomatous polyposis, with the genetic test demonstrating a deletion in the exon 1–15 of the APC gene. The patient's mother subsequently died of aggressive colorectal carcinoma 4 years later, at the age of 39. Our patient underwent genetic evaluation, which also revealed deletions in the exon 1–15 of the APC gene. A gastrointestinal endoscopic examination at the age of 10 years revealed numerous adenomatous polyps throughout her gastrointestinal tract (stomach, duodenum, and colon). The patient underwent prophylactic colectomy at the age of 12 years.
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