A tailgut cyst in a patient with myelomeningocele – An association or a coincidence? – A case report.

Abstract

Abstract Tailgut cysts (TGC) are rare lesions occurring in the retrorectal space, a potential space anterior to the sacrum and coccyx and posterior to the rectum. They are developmental lesions that have potential for malignant transformation into neuroendocrine tumors, carcinomas, or adenocarcinomas. However, they present with non-specific complaints and are often challenging to diagnose and can lead to misdiagnosis and delay of treatment. We present a case of a a 27-year-old female patient with history of a myelomeningocele who was found to have a left sided pre-sacral cystic mass. Histologic examination of the lesion following surgical removal were consistent with TGC. The presence of a TGC in a patient with myelomeningocele is a rare occurrence. There is some evidence that disturbances in the Sonic Hedge Hog (SHH) signaling pathway can lead to abnormal branching and overgrowth of the notochord contributing to abnormal separation from the hindgut endoderm. Clinicians should have a heightened clinical suspicion for a TGC in patients with known neural tube defects who present with a perirectal mass lesion.