Neonatal Subependymal Giant Cell Astrocytoma

Medhkour, Azedine; Traul, David; Husain, Muhammad

doi:10.1159/000058432

Cited by 29 publications

(5 citation statements)

References 11 publications

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“…The number of patients included in each reviewed paper ranged from a single patient in a case report to 250 patients in a literature review 24,25 . These 44 papers yielded 468 cases of intracranial tumors in children 6 months of age and less 1,3–5,8–10,12,13,15,17–19,21,26,27,29–31,33–35,37,38,40–46,51–55,57–59,61,62,65–67 . There was significant variability in brain tumor histologies (Table 2).…”

Section: Resultsmentioning

confidence: 99%

Surgical Treatment of Brain Tumors in Infants Younger than Six Months of Age and Review of the Literature

et al. 2012

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Object Brain tumors are rare in infants under 6-months of age. These tumors can be challenging to treat surgically. We analyzed a modern series of patients treated by a multidisciplinary team at a tertiary care center and performed a literature review of this unique population. Methods Retrospective clinical data was collected for patients surgically treated for intracranial mass lesions at The Children’s Hospital of Philadelphia from 1998 to 2007. Dermoid cysts and other skull-based lesions were excluded from the analysis. Results Sixteen patients under 6-months of age underwent surgery for primary intracranial mass lesions. The median age of the patients at surgery was 5.2 months (range 1.4 to 6 months of age). Children most often presented with a bulging fontanelle, hydrocephalus, or macrocephaly (7 patients). Vomiting was seen in 5 patients, cranial nerve palsies in 1 patient, and seizures in 3 patients. All patients had tumor resections and post-operatively were monitored in the intensive care unit. The final pathology consisted of atypical teratoid/rhabdoid tumor (3 cases), primitive neuroectodermal tumor/medulloblastoma (3 cases), choroid plexus papilloma (2 cases), astrocytoma (2 cases), ganglioglioma (2 cases), desmoplastic infantile ganglioglioma (2), glioblastoma multiforme (1), and choroid plexus carcinoma (1). Two intra-operative deaths occurred. Of the surviving 14, a gross total resection was achieved in 4. Adjuvant therapy was determined by a multidisciplinary team composed of neuro-oncology, neurosurgery, and radiation oncology. Seven patients were treated with chemotherapy, 1 patient had proton beam therapy. Five-year overall survival was 45%. The eight surviving patients had neurological sequelae, and developmental outcome was variable. Conclusions Brain tumors are uncommon in children under 6-months of age. Patients present with a variety of tumor pathologies. Children who survive have neurological sequelae. More studies are necessary to understand the impact that different treatment options, tumor pathology, and tumor location have on neurological outcome.

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Section: Resultsmentioning

confidence: 99%

Surgical Treatment of Brain Tumors in Infants Younger than Six Months of Age and Review of the Literature

et al. 2012

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“…SEGAs are low‐grade astrocytomas (World Health Organization [WHO] grade 1), which do not typically respond to radiation therapy or chemotherapy. Less commonly, more aggressive CNS tumors may occur, in the retina or in other locations 4–6. Finally, given the genetic basis of tuberous sclerosis, there is a risk for inducing second malignancies through utilization of standard chemotherapeutic agents or radiation therapy 7…”

mentioning

confidence: 99%

Rapamycin causes regression of astrocytomas in tuberous sclerosis complex

Franz

Leonard

Tudor

et al. 2006

Annals of Neurology

573

380

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“…Most patients with congenital SEGA undergo early surgery, although most reports document relatively poor surgical outcomes in neonates with a significantly high-risk operative morbidity and mortality. [7,11,13] e reason lies in the fact that in neonates, the particular tumor seems to acquire more aggressive clinical features while the perioperative risk for major brain surgery in neonates is significantly high. e presence of cardiac rhabdomyomas also contributes, being the cause of cardiac complications and severe intraoperative arrhythmia.…”

Section: Discussionmentioning

confidence: 99%

Fetal subependymal giant cell astrocytoma: A case report and review of the literature

Karagianni

Karydakis

Giakoumettis

et al. 2020

Surgical Neurology International

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Background: Subependymal giant cell astrocytomas (SEGAs) appear approximately in 10% of patients with tuberous sclerosis. These tumors are most commonly diagnosed in childhood and adolescence, with in utero diagnosed SEGAs being an extremely rare entity. Case Description: We present the case of a congenital SEGA detected in an antenatal ultrasound and further investigated with fetal magnetic resonance imaging (MRI) scans at 22 and 32 weeks of gestational age. At 9 days of age, the child underwent craniotomy and partial excision of the tumor, followed by a second more extensive operation 13 days later. The patient was subsequently administered mammalian target of rapamycin inhibitor (everolimus). Conclusion: In the latest follow-up MRI, at the age of two, the SEGA remained unchanged. Management of these tumors in neonates is challenging, mainly due to high morbidity and mortality of surgical treatment in these ages.

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Neonatal Subependymal Giant Cell Astrocytoma

Cited by 29 publications

References 11 publications

Surgical Treatment of Brain Tumors in Infants Younger than Six Months of Age and Review of the Literature

Surgical Treatment of Brain Tumors in Infants Younger than Six Months of Age and Review of the Literature

Rapamycin causes regression of astrocytomas in tuberous sclerosis complex

Fetal subependymal giant cell astrocytoma: A case report and review of the literature

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