Cervicomedullary astrocytomas of childhood: clinical and imaging follow-up

T, Young Poussaint; Yousuf, Naveed; Pd, Barnes; Anthony, Douglas C.; Zurakowski, David; Scott, Russell S.; Tarbell, NJ

doi:10.1007/s002470050671

Cited by 56 publications

(59 citation statements)

References 15 publications

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“…33,38 As with any entity in which a low-grade pathological type is predominant, overall survival is high, but so is the risk of recurrence requiring further treatment. 15,42 Fourteen (45%) of our children developed a recurrence at a median and mean time of 354 and 1193 days, respectively. Four patients died, all with high-grade tumor pathological types and all within 1 year of diagnosis.…”

Section: Discussionmentioning

confidence: 91%

“…4,16,33,38,42 Cervicomedullary tumors are a rare group of diverse tumors. The majority of patients have low-grade tumors with long duration of preoperative symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…The majority of these tumors are histologically benign, slow-growing gliomas that typically present with a long duration of symptoms. 42 These symptoms fall into two categories: lower brainstem dysfunction, and myelopathy. 16,36 Patients whose tumor focus is within the medulla first develop nausea and vomiting, obstructive hydrocephalus, failure to thrive, lower cranial nerve dysfunction, chronic aspiration, sleep apnea, and head tilt.…”

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confidence: 99%

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Cervicomedullary tumors in children

McAbee

Modica

Thompson

et al. 2015

PED

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OBJECT Cervicomedullary tumors (CMTs) represent a heterogeneous group of intrinsic neoplasms that are typically low grade and generally carry a good prognosis. This single-institution study was undertaken to document the outcomes and current treatment philosophy for these challenging neoplasms. METHODS The charts of all pediatric patients with CMTs who received treatment at St. Jude Children’s Research Hospital between January 1988 and May 2013 were retrospectively reviewed. Demographic, surgical, clinical, radiological, pathological, and survival data were collected. Treatment-free survival and overall survival were estimated, and predictors of recurrence were analyzed. RESULTS Thirty-one children (16 boys, 15 girls) with at least 12 months of follow-up data were identified. The median age at diagnosis was 6 years (range 7 months-17 years) and the median follow-up was 4.3 years. Low-grade tumors (Grade I or II) were present in 26 (84%) patients. Thirty patients underwent either a biopsy alone or resection, with the majority of patients undergoing biopsy only (n = 12, 39%) or subtotal resection (n = 14, 45%). Only 4 patients were treated solely with resection; 21 patients received radiotherapy alone or in combination with other treatments. Recurrent tumor developed in 14 children (45%) and 4 died as a result of their malignancy. A high-grade pathological type was the only independent variable that predicted recurrence. The 5- and 10-year treatment-free survival estimates are 64.7% and 45.3%, respectively. The 5- and 10-year overall survival estimate is 86.7%. CONCLUSIONS Children with CMTs typically have low-grade neoplasms and consequently long-term survival, but high risk of recurrence. Therapy should be directed at achieving local tumor control while preserving and even restoring neurological function.

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Section: Discussionmentioning

confidence: 91%

“…4,16,33,38,42 Cervicomedullary tumors are a rare group of diverse tumors. The majority of patients have low-grade tumors with long duration of preoperative symptoms.…”

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Cervicomedullary tumors in children

McAbee

Modica

Thompson

et al. 2015

PED

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“…3,16) However, some cases with atypical neuroimaging and/or clinical progression and some exceptionally aggressive cases required further treatment, such as surgical resection or radiotherapy. 2,5,[7][8][9][14][15][16][18][19][20] In the present case, we decided to perform radical resection of the enhanced tumor because of the unusual aggressive clinical course with hemorrhage and the possibility of malignant components. The surgical cure was successful without neurological deficits.…”

Section: Discussionmentioning

confidence: 91%

“…The overwhelming majority of tectal tumors are low-grade astrocytomas. [1][2][3]10,12,14,16) Accordingly, tectal tumors are often described as a distinctive subset of brain stem glioma with an unusually benign clinical course, and the diagnosis of`benign tectal glioma' is often based on the neuroimaging appearance and indolent clinical course of the lesion. 2,3,13,14) Here we describe a case of tectal pilocytic astrocytoma with the extremely unusual initial manifestation of intracranial hemorrhage.…”

Section: Introductionmentioning

confidence: 99%

Total Resection of a Hemorrhagic Tectal Pilocytic Astrocytoma-Case Report-

Oka

Yamashita

Kumabe

et al. 2007

Neurol. Med. Chir.(Tokyo)

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A 21-year-old man presented with a hemorrhagic pilocytic astrocytoma of the tectal plate manifesting as sudden onset of severe headache, vertigo, nausea, and vomiting. Computed tomography demonstrated acute hydrocephalus and hemorrhage within the brain stem and fourth ventricle. Magnetic resonance (MR) imaging revealed a dorsally exophytic tectal tumor as hypointense on the T 1 -weighted image and hyperintense on the T 2 -weighted image with contrast enhancement. Radical resection of the tumor was selected because of the unusual aggressive clinical course with hemorrhage and suspicion of malignant components. The tumor was totally resected via an occipital transtentorial approach using a neuronavigation system without surgical complications. The histological diagnosis was pilocytic astrocytoma. The patient was discharged home without neurological deficits on the 9th postoperative day. Twenty-three months after the surgery, follow-up MR imaging demonstrated no recurrence. Tectal plate pilocytic astrocytoma is rarely associated with hemorrhage but should be considered in the differential diagnosis of intracranial hemorrhage with acute presentation. Such exceptional tectal tumors should be resected radically and undergo histological examination to decide on further appropriate treatment.

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Cervicomedullary Gliomas

Reeves¹,

Ellenbogen²

Neuro-Oncology of CNS Tumors

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Cervicomedullary astrocytomas of childhood: clinical and imaging follow-up

Abstract: Cervicomedullary tumors are a unique subset of brainstem gliomas in childhood that present with a long duration of symptoms and a greater long-term survival than pontine gliomas.

Cited by 56 publications

References 15 publications

Cervicomedullary tumors in children

Cervicomedullary tumors in children

Total Resection of a Hemorrhagic Tectal Pilocytic Astrocytoma-Case Report-

Cervicomedullary Gliomas

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