Neurenteric cysts: risk factors and management of recurrence

Background: Neurenteric cysts are rare central nervous system lesions with excellent outcome after total excision. The authors report their experience in the management of these lesions, especially if recurrence occurs. Risk factors of recurrence were analyzed, which have never been reported according to the literature review. Methods: Twelve patients underwent surgical treatment for neurenteric cysts from April 1998 to July 2015. We retrospectively reviewed the clinical, radiological, operative, and pathological findings for these patients. Correlations between clinical characteristics and recurrence were analyzed. Results: This series included six intracranial and six intraspinal cases. Total resection was achieved in 4 of the 12 cases and partial resection in 8. Six of the patients that underwent partial resection suffered from recurrence, and an additional operation was needed in five. Three of the recurrent intracranial lesions had refractory symptoms and were finally controlled through cyst-peritoneal or ventricle-peritoneal shunts. Risk factors of recurrence were revealed as age ≦30 years old (p = 0.02), subtotal resection in the first operation (p = 0.01), and size of supratentorial NECs >30 ml (p = 0.04). Two-thirds of recurrences occurred in the first 2 years after treatment. Conclusions: NECs express high recurrence rates when total excision cannot be achieved. The preoperative diagnosis error plays a major role leading to an incorrect surgical strategy and recurrence. MRI with FLAIR and DWI is recommended to improve the preoperative diagnosis. Although timely reoperation is indicated for symptomatic recurrence, adhesion may cause reoperation to become more challenging. C-P or V-P shunts are suggested as the second-line treatment, resulting in excellent prognosis. Patients with mucin-secreting cells noted histologically are susceptible to recurrence.