Giant and recurrent enterogenous cyst of the frontal lobe: case report

Abstract

Enterogenous cysts of the central nervous system, also known as endodermic or neuroepithelial cysts are benign congenital lesions. The 2007 AFIP Atlas of Tumor Pathology defines them as “cysts lined by columnar epithelium of presumed endodermal derivation” [7]. They arise from misplaced epithelium of the nasopharynx, respiratory tree or intestinal tract and are characterized by their simple columnar, ciliated or goblet cell epithelium. Enterogenous cysts are extremely rare, as they represent only 0.01% of all tumors of the central nervous system [21]. Only about 100 cases have been described in the literature worldwide [28]. The exact incidence is not known. These lesions have been found in all age groups, from birth to the eighth decade of life [6], although they are most commonly diagnosed in children and young adults after years of slow growth [26]. These cysts are mainly located in the spinal cord. They are particularly common in the lower cervical and upper thoracic segments, and they tend to occupy a location anterior to the spinal cord [22]. The intracranial location is very rare. Intracranial enterogenous cysts are mainly found along the midline of the posterior cranial fossa, anterior to the brainstem, or in the fourth ventricle [5]. Supratentorial cysts are extraordinarily rare, with only 20 cases described so far [10, 14, 23]. A comprehensive review of the literature has yielded only three cases of enterogenous cysts located in the frontal lobe [3, 12, 14]. We present the case of a giant enterogenous cyst of the frontal lobe that reappeared 11 years after its first resection and which, 4 years after a second resection, remains asymptomatic and without radiological signs of the lesion.