Neuroendocrine carcinoma arising in a tailgut cyst

INTRODUCTION: A tailgut cyst, also called retrorectal cystic hamartoma, is a rare congenital lesion that forms most commonly in the retrorectal space. It is presumed to arise from remnants of early embryogenesis. PRESENTATION OF CASE: The following report describes a unique case of a retrorectal cystic hamartoma in a 53 year-old French Canadian man with a history of low back pain. The tumour underwent malignant transformation into a well-differentiated neuroendocrine carcinoma three years after the beginning of symptoms. DISCUSSION: This condition can be found at any age, but occurs especially among middle-aged women. Not only is it frequently misdiagnosed, but also several complications associated to the cyst have been reported such as infection and malignant transformation. This is why complete surgical excision of the tailgut cyst is currently recommended.