Selection of a management strategy for pediatric brainstem tumors

Halperin, Edward C.; Wehn, S M; Scott, J. Wilkie; Djang, W T; Oakes, W. Jerry; Friedman, Henry S.

doi:10.1002/mpo.2950170209

Cited by 46 publications

(11 citation statements)

References 37 publications

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“…2,[20][21][22] These tumors are considered inoperable due to their location, regardless of their histology, and carry the worst prognosis of tumors in pediatric neuro-oncology. Conventional treatment for this disease is radiation therapy, which a ADC decreased and FA increased significantly between baseline and the early post-RT period.…”

Section: Discussionmentioning

confidence: 99%

Apparent Diffusion and Fractional Anisotropy of Diffuse Intrinsic Brain Stem Gliomas

Chen¹,

Panigrahy²,

Dhall³

et al. 2010

AJNR Am J Neuroradiol

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Section: Discussionmentioning

confidence: 99%

Apparent Diffusion and Fractional Anisotropy of Diffuse Intrinsic Brain Stem Gliomas

Chen¹,

Panigrahy²,

Dhall³

et al. 2010

AJNR Am J Neuroradiol

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“…Brain stem tumors are uniquely identified by anatomic location in trating lesions of the pons, the characteristic clinical and MRI appearance generally obviates the need for histologic confirmation. Even when biopsies are obtained, they are of limited prognostic value; these lesions almost uniform ly show a malignant clinical course with little suggestion of more favorable outcome in the 33-83% of such cases which demonstrate low-grade histology [2,[4][5][6][7], Dorsally exophytic brain stem gliomas (DEBSGs) have been identified as minimally invasive tumors generally associated with prolonged survival [8], Although these lesions have been reported to be 'low-grade' astrocytic tumors, their histology has not been well characterized. The exophytic nature of these lesions and their 'lowgrade' histology suggest the potential benefit of primary surgical intervention [3].…”

Section: Introductionmentioning

confidence: 99%

Predominance of Pilocytic Histology in Dorsally Exophytic Brain Stem Tumors

et al. 1994

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We report the magnetic resonance imaging (MRI) and clinicohistologic characterization of dorsally exophytic brain stem gliomas (DEBSGs). Between 1983 and 1991, 12 of 51 patients evaluated for the diagnosis of brain stem glioma were found to have DEBSGs emanating from the pons, pontomedulla-ry junction or medulla. Eleven of the 12 patients had classic juvenile pilocytic astrocytomas. Unlike most other brain stem tumors, these patients were young (median 38 months, range 17-75), had a relatively long duration of symptoms (median 7 months, range 2-24) and displayed signs of increased intracranial pressure with limited cranial nerve paresis, absence of pyramidal tract findings, and near normal brain stem auditory-evoked potentials. MRI characteristically showed sharply demarcated lesions with decreased signal intensity on T1, and increased intensity on T2 sequences. Except for cystic areas, these tumors showed bright, uniform enhancement after gadolinium-DTPA. In all patients, 50-100% of the tumor volume could be resected. Three of 10 patients who received no immediate postoperative treatment eventually demonstrated disease progression, and 2 patients with subtotal resections who were treated with radiation and/or chemotherapy postoperatively remain disease-free for extended periods of time. The only death occurred in the 1 patient treated with chemotherapy who died of secondary leukemia. The overall and progression-free survival of these patients at 2 years is 100 and 67% as compared to 18 and 21 %, respectively, for other concomitantly treated non-exophytic brain stem gliomas. The excellent prognosis for this unusual group of brain stem tumors may be associated with the generally benign biology of pilocytic tumors and the ability to achieve significant degrees of resection.

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Brain stem glioma in a child: False diagnosis of radiation necrosis with FDG PET

Fenton

Madden

Foreman

2003

Med. Pediatr. Oncol.

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Brainstem gliomas constitute 10-20% of all pediatric central nervous system tumors, and are diagnosed using computed tomography (CT) and magnetic resonance imaging (MRI) with surgical biopsy infrequently performed [1]. One clinical problem that is difficult to resolve in the treatment of brainstem gliomas is the clinical deterioration seen following completion of fractionated radiation therapy. While CT and MRI will delineate the anatomic lesion, neither can distinguish between radiation necrosis and progressive tumor, which provide similar signs and symptoms. Positron emission tomography (PET) with 18-fluoro-deoxyglucose (FDG) has been reported to be beneficial in resolving this dilemma [2][3][4][5]. Our experience with an 11-year-old girl is instructive in this regard.She presented with a 2-month history of progressive signs and symptoms relevant to a brain stem lesion. An MRI showed an expansile mass in the pons extending into the right cerebellar hemisphere with nodular enhancement (Fig. 1). The findings were consistent with a diffuse pontine glioma. Dexamethasone was started. Conformal radiation therapy to a total dose of 54 Gy was given with concurrent temozolomide. The steroids were slowly tapered and she recovered normal neurologic function, approximately 2 months after diagnosis. MRI scans were performed every 3 months, and showed stable to decreased size of the pontine mass. Ten months after diagnosis, the patient presented with decreased dexterity in her left hand; MRI showed enlargement of the pontine mass with increased nodular enhancement. The differential diagnosis was tumor progression vs. radiation necrosis, albeit the latter would be unlikely after this dose and so short an interval. An FDG PET scan was performed and showed no evidence of abnormal metabolic activity involving the pons (Fig. 2), suggesting radiation necrosis as the etiology for the patient's symptoms. She was followed without treatment, and one month later returned with severe left hemiparesis, headache, and difficulty swallowing. MRI revealed progressive tumor, and for the first time metastatic disease was seen (Fig. 3). Terminal care was arranged and the patient died 2 months later. DISCUSSIONPET was developed in the mid 1970s with the primary goal of providing quantitative measurements and anatomic localization of metabolic processes in vivo. PET scanning relies on the ability of radionuclides to emit positively charged particles or positrons, which, having traveled a short distance in tissue (millimeters), interact with electrons and emit energy in the form of two high energy (511 keV) gamma rays. A wide variety of ligands labeled with positron emitters have been developed to provide insights into many aspects of brain tumor biology. Among these metabolic radiotracers, 18-fluoro-deoxy glucose (FDG) has been the most frequently used for the clinical investigation of brain tumors. Similar to glucose, FDG enters the cell through several membrane transporters and is phosphorylated by hexokinase into a monophosphate derivative. In...

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Selection of a management strategy for pediatric brainstem tumors

Cited by 46 publications

References 37 publications

Apparent Diffusion and Fractional Anisotropy of Diffuse Intrinsic Brain Stem Gliomas

Apparent Diffusion and Fractional Anisotropy of Diffuse Intrinsic Brain Stem Gliomas

Predominance of Pilocytic Histology in Dorsally Exophytic Brain Stem Tumors

Brain stem glioma in a child: False diagnosis of radiation necrosis with FDG PET

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