Intracranial neuroenteric cysts: A concise review including an illustrative patient

Malignant Transformation of a Neurenteric Cyst in the Posterior Fossa Presenting with Intracranial Metastasis: A Case Report and Literature Review

“…1) Although the most common location of neurenteric cysts is ventral to the lower cervical and upper thoracic spine, 1,2,15) they can also arise in the intracranial regions. 1) Bejjani et al reported that 90% of intracranial neurenteric cysts were located in the posterior fossa.…”

“…1,17) Therefore, their epithelium is positive for cytokeratin, carcinoembryonic antigen, and EMA, but negative for GFAP and S-100 protein on immunohistochemical analysis. 1,18) Although most neurenteric cysts are histologically benign, 1,18,19) Perrini et al reported that one-third of cases treated by partial or subtotal resection later experience symptomatic recurrence. 6) On the other hand, pathological proof of malignant transformation in the surgical specimen is extremely rare.…”

“…Conventional radiotherapy and chemotherapy have shown little effect on histologically benign neurenteric cysts. 1,18) As shown in Table 1, among previously reported cases, two patients underwent radiation therapy and one died 1 year later and the other was not followed-up over the long term. 13,14) Sahara et al reported a case of a neurenteric cyst in the foramen magnum with malignant transformation.…”

“…A neurenteric cyst, also known as enterogenous cyst, endodermal cyst, respiratory cyst, and bronchogenic cyst, is a rare benign cystic lesion, 1) generally arising in the intradural extramedullary space in the lower cervical and upper thoracic spine. 2,3) While the incidence of spinal neurenteric cyst has been reported to be 0.3%-0.5% of all spinal tumors, 4,5) neurenteric cysts in the posterior fossa are relatively rare.…”

“…1) Other than the cases of originally malignant neurenteric cysts, [7][8][9][10][11] the malignant transformation of benign neurenteric cysts is extremely rare. [12][13][14] In this report, we describe a case of a neurenteric cyst in the posterior fossa that eventually showed malignant transformation and intracranial metastasis and review of the relevant literature.…”