- University of Costa Rica, School of Medicine, Ciudad Universitaria Rodrigo Facio, San Pedro de Montes de Oca, Costa Rica
- Department of Neurological Surgery, Hospital San Juan de Dios, Paseo Colón, Costa Rica
- Department of Neurological Surgery, Hospital Dr. Rafael Ángel Calderón Guardia, Calle 17, San José, Costa Rica
University of Costa Rica, School of Medicine, Ciudad Universitaria Rodrigo Facio, San Pedro de Montes de Oca, Costa Rica
Department of Neurological Surgery, Hospital Dr. Rafael Ángel Calderón Guardia, Calle 17, San José, Costa Rica
DOI:10.4103/2152-7806.137837Copyright: © Hernández-Durán S This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Silvia Hernández-Durán, Yeh-Hsieh T, Salazar-Araya C. Pedunculated intraventricular subependymoma: Review of the literature and illustration of classical presentation through a clinical case. Surg Neurol Int 30-Jul-2014;5:117
How to cite this URL: Silvia Hernández-Durán, Yeh-Hsieh T, Salazar-Araya C. Pedunculated intraventricular subependymoma: Review of the literature and illustration of classical presentation through a clinical case. Surg Neurol Int 30-Jul-2014;5:117. Available from: http://sni.wpengine.com/surgicalint_articles/pedunculated-intraventricular-subependymoma-review-of-the-literature-and-illustration-of-classical-presentation-through-a-clinical-case/
Background:Subependymomas are rare benign, noninvasive tumors, classified by the World Health Organization as low grade neoplasms. International data estimate their frequency between 0.2% and 0.7% of the intracranial tumors, and they usually are an incidental finding in autopsies. Preferably located in the fourth ventricle, these tumors tend to become symptomatic when they cause hydrocephalous by obstructing cerebrospinal fluid circulation.
Case Presentation:We present the case of a morbidly obese, hypertense, and diabetic patient, who presented with symptoms of gait ataxia, sphincter incontinence, and dysartria in relation to a pedunculated subependymoma in the left lateral ventricle. He underwent a biparietal craniotomy with a microscopic microsurgical approach, through which gross total resection was achieved. No perioperative complications ensued.
Conclusions:Given their benign behavior and their excellent response to surgical treatment, subependymomas should be promptly diagnosed and surgically treated to avoid possible neurological damage when they become symptomatic.
Keywords: Gait ataxia, intraventricular mass, pedunculated, subependymoma
Subependymomas are rare, benign, noninvasive tumors of ependymal origin. Internationally, their frequency is estimated between 0.2% and 0.7% of intracranial tumors, and they are usually incidental findings in autopsies.[
A 51-year-old, morbidly obese male, with past medical history of long-standing hypertension and type 2 diabetes mellitus, consulted because of 3 weeks of gait ataxia, sphincter incontinence, and episodic dysarthria of several minutes duration. Initially, the patient was managed by the Neurology Service, who observed wide-based gait, inability to perform tandem gait, globally reduced reflexes and glove and stocking sensory disturbances. Complementary studies revealed polyneuropathy, likely of diabetic origin, and vetebrobasilar insufficiency. The patient was therefore managed as an ischemic syndrome.
Nevertheless, the patient also underwent a computerized tomography (CT) scan, and magnetic resonance imaging (MRI), which showed an isodense lesion [
(a) T1-weighted coronal view. Hypointense nodular image in the floor of the left lateral ventricle, producing mild-moderate, noncommunicating hydrocephalus. (b) T1-weighted sagittal view. Note the involvement of the brainstem, which explains the symptoms of the patient. (c) T1-weighted axial view showing a nodular, hypointense lesion with small cystic areas in its interior. (d) Axial view, FLAIR. Note the absence of perilesional edema, and the lack of contrast enhancement
Because the patient suffered progressive clinical decline, characterized by dysphasia and paresis of the lower extremities, we placed a ventriculostomy to control his malignant intracranial hypertension. Twenty-four hours later, the patient underwent biparietal craniotomy. We performed a transcallosal approach with microsurgical technique; an anterior longitudinal callostomy was performed, and a fibroelastic intraventricular mass of whitish coloration, limited by a vascular pedicle, measuring approximately 6 × 3 × 4 cm was resected. Gross total resection was achieved, and there were no postoperatory complications.
Biopsy revealed a tumor with small, rounded nuclei and scarce cytoplasm, and cell groups surrounded by abundant fibrillary matrix and cystic areas. Immunohistochemistry was positive for glial fibrillary acidic protein (GFAP) and negative for neurospecific enolase, which was compatible with grade I subependymoma, according to the World Health Organization (WHO) classification [
(a) Gross anatomy of resected tumor, showing a fibroelastic, whitish mass. (b) H and E stain of the mass, exhibiting the characteristic rounded cells with small nuclei and scarce cytoplasm over an abundant fibrillary matrix and cystic spaces. Note the absence of atypical mitoses. (c) Immunohistochemistry stain for neurospecific enolase, showing negativity in the majority of the cells. (d) Immunohistochemistry stain for glial fibrillary acidic protein (GFAP), exhibiting strong positivity
The patient is currently asymptomatic of his neurosurgical condition, with complete resolution of his speech and sphincter disorders, and he is completing rehabilitation at the Costa Rican National Rehabilitation Centre for his sensory and motor deficits in the lower extremities. Postoperative imaging did not show tumor recurrence a year after surgery.
Initially described by Scheinker in 1945,[
The fourth ventricle and the lateral ventricles are among the most common locations of subependymomas, with approximate incidences of 60% and 40%, respectively. Nevertheless, the lesions that tend to express themselves clinically are those located in the lateral ventricles.[
Radiologically, the appearance of subependymomas can vary depending on their location. According to several studies, subependymomas in the lateral ventricles are reported as well-defined, isodense or hypodense nodular images, and they evince minimal contrast uptake.[
Macroscopically, subependymomas present as fibroelastic, whitish nodules.[
Histogenesis of subependymomas is a controversial topic, and several researchers have proposed different cells of origin. Subependymal glia, astrocytes of the subependymal plate, and ependymal cells themselves have all been postulated as likely precursors to subependymomas, but there are not conclusive data.[
The treatment of symptomatic subependymomas is complete surgical excision.[
In conclusion, subependymomas are rare intracranial tumors of benign behavior. Nevertheless, their size and location can produce symptoms because of CSF obstruction or parenchymal irritation. Even though the imaging characteristics are not pathognomonic, these neoplasms have a distinctive histology. Because of their good prognosis and excellent response to surgical management, prompt and accurate diagnosis is vital to provide our patients with good outcomes.
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