Intracranial midline dermoid and epidermoid cysts in children. J Neurosurg

Department of Pediatrics, Operative Unit of Pediatric Neurosurgery, Catholic University, Medical School, Rome, Italy.
Journal of Neurosurgery (Impact Factor: 3.74). 06/2004; 100(5 Suppl Pediatrics):473-80. DOI: 10.3171/ped.2004.100.5.0473
Source: PubMed


Dermoid and epidermoid cysts are rare space-occupying lesions of the central nervous system. Although characterized by a slow growth rate, they are often associated with serious complications. Surgery is the only effective treatment, and radical resection of the entire cyst, whenever possible, generally succeeds in achieving a cure. Authors of large series have only occasionally reported on pediatric cases, and these reports often lack a specific analysis of those cerebral midline lesions.
The authors report on the treatment of 19 patients (16 with intracranial intradural dermoid and three with epidermoid cysts located along the cerebral midline). All patients underwent surgery at the Catholic University Medical School in Rome. The patients ranged in age from 3 months to 16 years. Nine cysts were located in the posterior cranial fossa, six in the frontobasal subarachnoid spaces, two in the third ventricle, and two in the quadrigeminal plate cistern. In the cases presenting with dermal sinus tracts, attempts at resecting the dermoid cyst and the associated dermal sinus were made in a single stage to achieve an en bloc removal. In the cases without dermal sinus tracts, and in the three with epidermoid cysts, a standard craniotomy was performed to reach the lesion. There were no surgery-related deaths, and the morbidity rate was low. Total and subtotal resections were achieved in 15 and four cases, respectively; however, regrowth of the residual tumor was observed only in two of them. After repeated resection, tumor progression has not been observed. At a mean follow-up period of 92.7 months, excellent clinical results were achieved in 18 patients.
Because surgery is the only effective treatment modality for these lesions, radical resection should be performed in all cases to avoid tumor recurrence; however, because the cyst capsule can adhere firmly to vital structures and attempts at its radical removal can be dangerous, subtotal resection may be a wise option in selected cases.

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    • "Treatment of symptomatic ECs is surgical resection, and gross total resection may be curative.17 18 19 Following surgery, close surveillance is essential because ECs may recur or residual cyst components may regrow; the exact incidence or the factors that predispose ECs to recur are not known. "
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    ABSTRACT: Epidermoid cysts (ECs) are benign congenital cysts that typically develop from misplaced stratified squamous epithelium during neural tube closure. They are most commonly encountered in the cerebellopontine angle, the fourth ventricle, and the region of the sella turcica. Recurrence of an EC or regrowth of residual components following surgery is known to occur, but the exact incidence is not defined. Repeat surgical removal is generally considered but may be complicated by significant morbidity. We present a novel endoscopic method by which to manage a recurrent EC of the infratemporal fossa using a silastic stent through a transpterygoid approach that permits periodic drainage of recurrent cyst material in the outpatient setting with minimal morbidity.
    Preview · Article · Dec 2013 · Journal of Neurological Surgery
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    • "They are considered to be the product of a defective closure of the neural tube, which occurs between the third and fifth gestational week.4 Associated dermal sinuses cause earlier onset of clinical symptoms such as infection.4 Other common symptoms including headaches, seizures, and chemical meningitis, and visual disturbances occur late in the clinical course because of its slow-growing nature.2,3,6 "
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    ABSTRACT: Dermoid cysts are rare congenital intracranial tumors. Among these tumors, extradural localization is extremely rare. We report a patient with an extradural dermoid cyst of the right parasellar region, causing right visual disturbance. Computed tomography revealed a hypodense mass lesion with rimlike calcification at the right parasellar region, accompanying marked erosion of the adjacent skull base. The tumor appeared as a heterogeneous intensity on magnetic resonance imaging and was surrounded by thin gadolinium enhancement without inner enhancement. The right optic nerve was compressed by the tumor. Surgical resection was successfully performed using a right frontotemporal extradural approach. The entire tumor was completely resided extradurally and was enclosed by saclike, stretched dura mater and extended deeply into the skull base. Histopathologic findings were consistent with the features of dermoid cyst. The postoperative course was uneventful, and the visual disturbance improved. Neuroradiological features, strategies for surgical treatment, and mechanisms responsible for preoperative symptoms are discussed.
    Preview · Article · May 2011
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    ABSTRACT: It is rare for a dermoid cyst to develop intratumoral hemorrhage. A 61-year-old woman had a sudden-onset left hemiparesis and slow response to verbal requests for one week when unenhanced computed tomography scanning revealed a mixed iso- and hypo-dense heterogeneous lesion in the right fronto-temporal area. T1-weighted magnetic resonance imaging (MRI) of the brain showed a mixed hyper- and hypo-intense tumor in the right fronto-temporal area. The tumor became hyperintense on T2-weighted MRI and was faintly enhanced at tumor periphery on T1-weighted MRI. The tumor was excised and pathological examination revealed a dermoid cyst with intratumoral hemorrhage. The post-operative course was complicated by hemorrhage in the tumor bed, which was evacuated immediately. The patient improved and could walk without support two weeks after the second operation. After 1 year of follow-up, she was well and without neurological deficits. To the best of our knowledge after a literature review, only two previous cases of dermoid cyst have featured intratumoral hemorrhage.
    Full-text · Article · Jan 2006 · Clinical Neurology and Neurosurgery
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