Supratentorial neurenteric cyst: Analysis of 45 cases in the literature

Object: Supratentorial neurenteric cysts (S-NC) are extremely rare. Patients with these lesions may present with headache, seizures or neurological deficit related to their location. Complete surgical excision, including its capsule, must be the aim of the surgery. However, strong adhesion of the cyst wall to the surrounding neurovascular structures may sometimes necessitate subtotal removal of the cyst capsule. In this study, the authors report two cases and review the literature for reported cases of S-NC to analyze clinical and radiographic presentations as well as surgical approaches and neurological outcomes. Methods: A MEDLINE/PubMed in English language search was performed, revealing 43 S-NC cases. The authors report two additional cases in the study, resulting a total of 45 cases. Each case was analyzed for clinical presentation, lesion location, radiographic features, treatment method, and outcome. Results: There were 25 male, 18 female and 2 newborns. The average age was 42,2 (range 0–78years). The majority of cases (63,6%) occurred in patients between the ages of 20 and 50, but 15.9% were over 70. Frontal lobe was affected in 29 cases (64,4%), parietal lobe in 12, temporal lobe in 4, occipital in 1, and sellar or parasellar region in 4. Three cases were intraventricular. Lesions were in left side in 23 cases, in right side in 16, and on midline in 7. The majority of cases (73,3%) were extra-axial. The most common presenting symptom was headache (42,2%). The average size was 5.38cm (greater measure) Seizures occurred in 35,5% and motor deficit in 17,7%. Other presentations were visual deficit (13,3%) and behavior changes (8,8%). Hemorrhage occurred in 2 cases, one spontaneous and one after cyst aspiration. Craniotomy with resection of the cyst (partial or complete) was performed in 95,5%. One case was treated with cyst aspiration and one case was just observed (surgery was performed in another cyst in posterior fossa). Thirty-two cases showed total improvement of symptoms, and 4 partial improvement after treatment. Two cases presented malignant transformation and two presented recurrent lesion. Complications after treatment occurred in 5 cases: 2 presented seizures in immediate postoperative period, one presented hemorrhage, one had transient SIADH. Infection occurred in just one case. Conclusions: S-NC are rare and challenging lesions. The radiological features are nonspecific, and it is difficult to differentiate enterogenous cysts from other cystic lesions such as arachnoid cyst, epidermoid or glioependymal cyst. In cases with mass effect and refractory symptoms, surgical removal is indicated, including liquid drainage, capsule removal and cisternal communication. Resection of these lesions is associated with favorable outcomes. Keywords: Supratentorial cyst, Intracranial neurenteric cyst