Mucinous adenocarcinoma arising from supratentorial intramedullary neuroenteric cyst with broncho‐pulmonary differentiation

Abstract

Neuroenteric cysts are benign intradural endoderm cysts lined by gastrointestinal (GI) or tracheobronchial epithelial cells. Their malignant transformation is extremely rare and only six cases have been reported. In these cases, tissue lineage of the cystic endoderm cells giving rise to carcinoma was not clearly identified either as respiratory or as GI type. Herein, we report a case of mucinous adenocarcinoma arising from the neuroenteric cyst with broncho-pulmonary differentiation in the right cerebral hemisphere of a Japanese woman in her late 50s. The cyst wall was entirely lined by the following respiratory epithelial components: stratified bronchial ciliated columnar epithelium with basal cells positive for CK5 and p63, terminal bronchiolar Clara cells positive for thyroid transcription factor (TTF)-1, surfactant B and negative for surfactant C, type I pneumocytes positive for TTF-1, negative for surfactant B and C, and type II pneumocytes positive for TTF-1 and surfactant B and C. In addition, nests of hyperplastic single layered mucinous epithelial cells with bronchial goblet cell phenotype were also demonstrated, and histologic features were almost the same as the pulmonary type I congenital adenomatoid malformation (CCAM) with mucinous epithelial proliferation. The mucinous epithelial nests of type I CCAM are liable to develop mucinous adenocarcinoma and frequently accompany K-ras mutation and expression of p16. However, K-ras mutation and p-16 expression were not detected in this case.

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) 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

“…Other reports have indicated that NCs are ~3 times more common in the spine compared with the brain (4); therefore, the lack of studies reporting an intraspinal NC with a malignant transformation is notable. In addition, in 3/9 studies, the NC was located in the supratentorial region (9,11,15), and 2 of those cases reported intramedullary lesions located in the cerebral hemisphere (9,11). Intracranial NCs that developed within the posterior fossa accounted for 70-90% of all intracranial NC cases (1).…”

“…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.…”

Neuroenteric cyst of the left lateral ventricle: case report and review of the literature

“…In exceptional cases, multiple NCs in both the supratentorial and the infratentorial compartments have been documented [2,11,13,17,18]. There are 3 cases showed NCs in the supratentorial region [19][20][21], and 2 of these cases reported intramedullary lesions located in the cerebral hemisphere [19,20]. Intracranial NCs in the posterior fossa make up 70-90% of all intracranial NCs cases [9].…”