Spontaneous Involution of an Intra-Axial Brain Stem Lesion: A Case Report

S, Rao; Constantini, Shlomi; Gomori, John M.; Siegal, Tali; Epstein, Fred J.

doi:10.1159/000120972

Cited by 17 publications

(11 citation statements)

References 7 publications

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“…Schmandt, et al, 44 reviewed 22 patients in whom regression of astrocytomas was demonstrated. In this review, 20 of the lesions were in the hypothalamic-chiasmatic region and two were in the brainstem with a pontine epicenter (the cases reported by Rao 40 and Lenard 29 and their colleagues). Patients ranged in age from 5 months to 8 years at the time of presentation.…”

Section: Spontaneous Regression Of Brainstem Lesionsmentioning

confidence: 95%

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Spontaneous regression of a diffuse brainstem lesion in the neonate

Thompson¹,

Kosnik²

2005

Journal of Neurosurgery: Pediatrics

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The authors present two cases of diffuse brainstem lesions that regressed without treatment. Two newborns presented with cranial nerve palsies and limb weakness at birth. Magnetic resonance (MR) images obtained in the 1st week of life revealed a large, expansive pontomedullary lesion in each patient. Findings of clinical and imaging examinations were highly consistent with the characteristics of diffuse brainstem glioma. After consultation with the parents of both infants, all parties agreed to forgo the treatment modalities available at the time. Neither patient underwent surgery, radiation treatment, or chemotherapy; both underwent routine neurological and MR imaging examinations. Within weeks the patient in Case 1 started to improve clinically and at 4 years of age has reached nearly all developmental milestones. Serial MR images demonstrated a steady decrease in the size of the lesion. The patient in Case 2 improved in a similar manner and is now 10 years old. The findings from these two cases should encourage families and clinicians to consider that a subcategory of diffuse lesions may exist, particularly in the neonatal period. It must be stressed, however, that nearly all patients with diffuse brainstem lesions experience a poor outcome, regardless of tumor grade or treatment. Brainstem gliomas, spontaneous regression of central nervous system tumors, and the differential diagnoses of brainstem lesions are discussed.

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Section: Spontaneous Regression Of Brainstem Lesionsmentioning

confidence: 95%

“…Rao, et al, 40 reported on a 4-year-old child who presented with a 9-month history of diplopia, facial asymmetry, and unsteadiness. On examination the patient was found to suffer from sixth and seventh cranial nerve palsies, nystagmus, and pyramidal and dysmetric signs.…”

Section: Spontaneous Regression Of Brainstem Lesionsmentioning

confidence: 99%

Spontaneous regression of a diffuse brainstem lesion in the neonate

Thompson¹,

Kosnik²

2005

Journal of Neurosurgery: Pediatrics

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“…Two additional cases suggest spontaneous regressions of juvenile pilocytic astrocytomas of the visual pathways due to tumor absence at autopsy years after a partial resection and a biopsy [1]. Four of the 22 cases had a documented increase in tumor size prior to the regression [4, 5, 6]and 3 had a history suggestive of tumor progression before diagnosis [7, 8, 9]. 12 cases had NF1.…”

Section: Discussionmentioning

confidence: 99%

“…As seen in our case, despite tumor regression, clinical symptoms do not necessarily improve. Only 3 of the 22 patients reviewed had clear-cut clinical improvement [7, 9, 12]. Three had normal clinical examinations even prior to the regression and despite their tumor [5, 6].…”

Section: Discussionmentioning

confidence: 99%

Spontaneous Regression of Low-Grade Astrocytomas in Childhood

et al. 2000

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An 8-year-old boy with neurofibromatosis type 1 (NF1) and a biopsy-proven juvenile pilocytic astrocytoma of the hypothalamic/chiasmatic region was followed with serial MRIs over 4 years. Spontaneous tumor regression was followed by progression and biopsy; 6 months later, the tumor regressed again. This bimodal regression is rare, but highlights the variable natural history of low-grade gliomas in children with NF1 and the difficulty in evaluating response of such tumors to therapy.

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“…Notably, spontaneous regression of presumed diffuse brainstem gliomas in younger children, including a neonate, has been reported. [14][15][16] Hence, any atypical clinical and neuroimaging features, such as the congenital presentation in our 2 cases, must be considered cautiously in the diagnosis and management of presumed DIPG.…”

Section: Casementioning

confidence: 96%

Diffuse intrinsic brainstem tumors in neonates

Shah

Ray²,

Bartels³

et al. 2008

PED

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✓ The authors report on 2 newborn infants with the unusual presentation of intrinsic brainstem tumors. Both nondysmorphic, full-term neonates had cranial nerve palsies and hypotonia. Diagnoses of diffuse intrinsic brainstem gliomas were made on the basis of magnetic resonance imaging, which showed large expansive, nonenhancing intrinsic pontine masses. Intrinsic pontine tumors, characteristically seen in school-age children, are most often high-grade gliomas that are almost invariably fatal. However, the microanatomy and natural history of pontine tumors in neonates are unknown. With parental consent, both newborns were treated expectantly with supportive care but died of progressive disease by 2 weeks of age. In one child, postmortem examination revealed a primary brainstem primitive neuroectodermal tumor. The authors conclude that, as in older children, neonatal intrinsic brainstem tumors may be of a highly malignant nature. The rapid tumor progression in both cases indicates that where a diagnostic procedure may pose significant risks, supportive observation can aid in distinguishing malignant from benign tumor growth.

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Spontaneous Involution of an Intra-Axial Brain Stem Lesion: A Case Report

Cited by 17 publications

References 7 publications

Spontaneous regression of a diffuse brainstem lesion in the neonate

Spontaneous regression of a diffuse brainstem lesion in the neonate

Spontaneous Regression of Low-Grade Astrocytomas in Childhood

Diffuse intrinsic brainstem tumors in neonates

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