Malignant transformation of a neurenteric cyst in the posterior fossa following complete excision

Abstract

We present a case of a 46-year-old man who developed malignant transformation of a neurenteric cyst felt to be completely removed 14 years previously. He re-presented with non-specific symptoms and hydrocephalus, and subsequent evidence of a complex lesion surrounding the lower brain stem and upper cervical cord. Histologically the original lesion was a benign neurenteric cyst, whereas the recurrent lesion showed a well differentiated adenocarcinoma. This is only the second case reported in the literature.

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

“…Our literature search identified three cases of neurenteric cysts that underwent malignant transformation. [12][13][14] Combined with cases of intracranial neurenteric cysts with malignant features, eight cases of malignant neurenteric cysts have been reported in the literature (Table 1). Notably, all of these eight cases were observed in intracranial neurenteric cysts, and malignant transformation of a spinal neurenteric cyst has never been reported, although they occur much more frequently in the spinal cord.…”

“…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. 13) They administered chemotherapy (carboplatin and etoposide) in conjunction with radiation therapy after the second resection, and the patient achieved stable disease for 6 months.…”

“…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.…”

Recurrent intracranial neurenteric cyst with malignant transformation: A case report and literature review

“…The age of the 9 patients with NC and malignant transformation ranged between 25-60 years (6,(9)(10)(11)(12)(13)(14). To the best of our knowledge, no patients have been reported with malignantly transformed NC during childhood.…”

“…To the best of our knowledge, very few reports have described this unusual phenomenon (9)(10)(11)(12)(13)(14)(15)(16). Therefore, the demographic characteristics, treatment and prognosis of this type of NC is not clear.…”