Subependymomas of the fourth ventricle

Jooma, Rashid; Torrens, Michael; Bradshaw, John; Brownell, Betty

doi:10.3171/jns.1985.62.4.0508

Cited by 51 publications

(49 citation statements)

References 11 publications

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“…7 Subtle calcifications were demonstrated in our Case 1, consistent with the occurrence of tumor calcifications in 32%-59% of subependymoma cases. 11,14 As reported by others, there was no contrast enhancement in the subependymomas described here. According to the literature, cranial CT enhancement characteristics of subependymoma are variable, ranging from absent to little enhancement.…”

Section: Discussionsupporting

confidence: 55%

“…In 1985, a resection in the region of the fourth ventricle could result in a mortality rate of 41%. 11 Artico et al as well as Lombardi et al improved microsurgical techniques and thereby improved outcome of these surgeries by markedly decreasing perioperative mortality rates from 25% initially to 0%. 1,15 Subependymomas are usually asymptomatic or cause only mild symptoms.…”

Section: Discussionmentioning

confidence: 99%

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Simultaneous subependymomas in monozygotic female twins: further evidence for a common genetic or developmental disorder background

Noell¹,

Beschorner

Bisdas

et al. 2014

JNS

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S ubependymoma is a rare, benign, slowly growing tumor (WHO Grade I), belonging to the group of ependymal tumors of neuroepithelial tissue. 16 Studies suggest an incidence of subependymoma in 0.2-0.7 of all CNS tumors and approximately 8% of ependymal tumors, 21 with men affected more often than women at a ratio of 2.3:1. The tumor is typically attached to the ventricular wall. The most frequent site of tumor location is the fourth ventricle followed by the lateral ventricles, but subependymomas are also noted in other locations of the CNS. Most subependymomas are asymptomatic. Clinical manifestations of these tumors are due to obstructive hydrocephalus or symptoms of brainstem compression by large tumors of the fourth ventricle. 20 In some cases, spinal subependymomas can cause paraplegia. Spontaneous intratumoral hemorrhage may also occur. In large tumor cysts, calcifications may be also observed.14 In 1896, Besold described brain tumors in siblings and twins for the first time.2 To date, familial occurrence of subependymomas of the fourth ventricle has been reported only 4 times: twice in siblings, 3,8 in a father and his son, 19 and in identical twin brothers. 4 In this paper we report on the second case of subependymomas of the fourth ventricle in twin siblings. In our cases, the tumors occurred in 41-year-old identical twin sisters. This is the first time subependymomas have been described in female monozygotic twins. Written informed consent to the publication of the clinical data and corresponding images was obtained from the patients. Case ReportsPresentations and Operations. Cases 1 and 2 were 41-year-old monozygotic twin sisters, who were born after a normal pregnancy. Until recently, neither of the sisters had shown any symptoms. However, 1 sister (Case 1) complained of morning vomiting, balance disturbance, In this paper, a rare case of subependymoma of the fourth ventricle in identical female twins is reported. Magnetic resonance imaging and CT showed nearly identical locations of the tumors in the fourth ventricle and similar growth patterns of the tumors in both sisters. Likewise, postoperative histopathological analysis of both tumors revealed the typical histological appearance of subependymomas. Subependymoma is a rare, low-grade glioma of the CNS, slowly growing and usually asymptomatic. If symptomatic, a subependymoma can in some cases lead to sudden death caused by pressure on the brainstem or decompensated secondary hydrocephalus. This case demonstrates the importance of detecting tumors early and thereby preventing symptoms arising from increasing intracranial pressure, and optimizing therapy options.

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Section: Discussionsupporting

confidence: 55%

Section: Discussionmentioning

confidence: 99%

Simultaneous subependymomas in monozygotic female twins: further evidence for a common genetic or developmental disorder background

Noell¹,

Beschorner

Bisdas

et al. 2014

JNS

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“…Infrequently, they are located in the septum pellucidum and spinal cord. 5,6 The CPA and prepontine cistern involvement without extension into the fourth ventricle is uncharacteristic of subependymomas. Hoeffel et al 4 described 4 cases with extension into CPA, but in all of them the tumors arose from the fourth ventricle.…”

Section: Discussionmentioning

confidence: 99%

Subependymoma of the Cerebellopontine Angle and Prepontine Cistern in a 15-Year-Old Adolescent Boy: Fig 1.

Koral¹,

Kedzierski²,

Gimi³

et al. 2007

AJNR Am J Neuroradiol

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SUMMARY:A case of cerebellopontine angle and prepontine cistern subependymoma in a 15-year-old adolescent boy is presented with a review of the literature. Apparent diffusion coefficient values for subependymoma are reported. Differential considerations for the unusual location of this rare tumor are discussed. Subependymomas are rare intracranial neoplasms typically located in the subependymal regions of the ventricles. The patients usually present with slowly progressive symptoms, or the tumors are incidentally discovered at autopsy.1 The typical age of presentation is in the fifth or sixth decades.2 We report a rare case of subependymoma involving the cerebellopontine angle (CPA) and prepontine cistern in a 15-year-old adolescent boy and describe the subependymoma imaging characteristics, including apparent diffusion coefficient (ADC) values. Case ReportA 15-year-old adolescent boy presented to the emergency department with a 4-year history of speech and balance difficulties. More recently, he developed progressive eye movement abnormalities. On examination, he had right partial cranial nerve (CN) VII and right CN VIII palsies, nystagmus, right-sided dysmetria, and hyperreflexia.CT of the head showed a noncalcified, large posterior fossa mass resulting in hydrocephalus. MR imaging of the brain demonstrated a solid, exophytic lesion centered at the right CPA and extending anteriorly, obliterating the prepontine cistern. The mass measured 6.3 ϫ 5.7 ϫ 6.4 cm and showed T1 and T2 prolongation. The lesion did not arise or extend into the fourth ventricle, which was compressed and displaced posteriorly. Although there was marked displacement of the brain stem, particularly of pons and mesencephalon, no peritumoral edema was evident. The chronic nature of the lesion was underscored by scalloping of the dorsal surface of the clivus. There was moderate hydrocephalus without transependymal resorption of CSF, confirming chronicity of ventricular enlargement (Fig 1A, -B). On diffusionweighted imaging (DWI), the lesion did not show restricted diffusion. The ADC values were obtained using a circular region of interest with a diameter of 10 mm at 5 nonoverlapping locations in the tumor. The mean ADC value was 1.34 ϫ 10 Ϫ3 mm 2 /s (range, 1.30 -1.39 ϫ 10

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“…Th e treatment of choice for subependymomas is complete surgical resection, which yields an excellent prognosis with rare recurrences (2,6). A complete surgical resection is more commonly possible with lesions located in the lateral ventricles, with fourth ventricular locations often treated with subtotal resections.…”

Section: Discussionmentioning

confidence: 99%

“…A complete surgical resection is more commonly possible with lesions located in the lateral ventricles, with fourth ventricular locations often treated with subtotal resections. Th ere is little documented additional benefi t from chemotherapy or radiotherapy (2,6).…”

Section: Discussionmentioning

confidence: 99%