Perineal recurrence of langerhans cell histiocytosis

초록

Langerhans cell histiocytosis (LCH) is a rare disorder, characterized by abnormal proliferation of Langerhans cells, which can affect various organ systems. The most common clinical findings are localized bone lesions, with eosinophilic granuloma, which occur at any age, although most patients present under the age of 15 years. We experienced a case of recurrent LCH, invading the bulbous urethra, in the perineal area of a 16-year-old boy. The primary tumor developed in the mastoid bone, and recurred in the ureter, maxillary sinus mucosa and perineum, in the order. The perineal lesion and part of bulbous urethra were surgically removed, through a perineal approach, and the urethra primarily reconstructed.

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