High-grade spinal cord gliomas are rare and carry a poor prognosis. A number of treatment modalities exist for spinal cord gliomas, but no consensus exists regarding their management. Cordectomy represents a possible option for treating these lesions; however, few cases have been reported in adults, and none have been reported in the pediatric population. The authors describe the use of cordectomy for the treatment of a high-grade spinal glioma in a 9-year-old boy who remains cancer free 14 years following his initial presentation.
R. Webster Crowley, Rebecca M. Burke, M. Beatriz S. Lopes, D. Kojo Hamilton and John A. Jane Sr.
Patrick L. Semple, John A. Jane Jr., M. Beatriz S. Lopes and Edward R. Laws
The aim of this study was to correlate the magnetic resonance (MR) imaging findings in pituitary apoplexy with histopathological results and determine whether the histopathology influences clinical presentation and outcome.
The records of 36 patients with histologically confirmed pituitary apoplexy, who were treated surgically at the University of Virginia Health System between 1996 and 2006, were retrospectively reviewed. The MR images were divided into 3 groups: 1) infarction alone; 2) hemorrhage with or without infarction; and 3) tumor only with no evidence of apoplexy. The histological examination was divided into infarction alone or hemorrhagic infarction/hemorrhage. The MR imaging findings were then correlated with the histopathological results to assess how accurately the histopathology was predicted by the MR imaging. The clinical features and outcomes of the two histopathological groups were also compared.
The MR imaging findings were able to predict the histopathology accurately in the majority of cases. The group of patients with infarction had less severe clinical features and a better outcome than those with hemorrhagic infarction/hemorrhage.
Magnetic resonance imaging findings in the setting of pituitary apoplexy accurately predict the nature of the apoplectic process and help to guide the type and timing of therapy.
T. David Bourne, James W. Mandell, Julie A. Matsumoto, John A. Jane Jr. and M. Beatriz S. Lopes
✓ The authors report the case of a 2-year-old boy with a primary, diffuse leptomeningeal oligodendroglioma in which the deletion of chromosome arm 1p was identified by performing a fluorescence in situ hybridization (FISH) analysis. This previously healthy child initially presented with malaise, anorexia, nausea, vomiting, and macrocephaly. Imaging studies confirmed the presence of hydrocephalus, and a ventriculoperitoneal shunt was placed. The postoperative course was complicated by emesis, continued weight loss, and numerous seizurelike episodes. A contrast-enhanced magnetic resonance imaging study performed approximately 10 weeks postoperatively showed diffuse leptomeningeal thickening and enhancement without evidence of an intraparenchymal mass lesion. A right frontal lobe brain biopsy revealed a hypercellular proliferation of small oligodendroglioma-like cells, which occupied the leptomeninges diffusely and spared the underlying cortical gray matter. The tumor cells displayed prominent perinuclear clearing and had evenly spaced, uniformly round nuclei. Occasional mitotic figures were observed. Background vessels were thin and delicate, and there was no evidence of necrosis. The tumor cells showed strong immunoreactivity for S100 protein; the results of immunohistochemical staining were negative for glial fibrillary acidic protein, vimentin, epithelial membrane antigen, NeuN, and synaptophysin. The deletion of 1p was demonstrated by FISH analysis; 1q, 19p, and 19q were intact. This appears to be the first reported case of a primary diffuse leptomeningeal oligodendroglioma in which a 1p deletion was identified.
Case report and review of the literature
Daniel M. Prevedello, Jay Jagannathan, John A. Jane Jr., M. Beatriz S. Lopes and Edward R. Laws Jr.
Pituitary adenomas are heterogeneous in growth rate, invasiveness, and recurrence. To understand the biological behavior of the individual adenoma more fully, cell proliferation markers such as monoclonal antibodies targeted against the Ki-67 antigen have been applied. The Ki-67 antigen is a protein related to cell proliferation and is expressed in cell nuclei throughout the entire cell cycle. The authors report the case of an extremely rapidly growing pituitary adenoma with cavernous sinus invasion. The lesion, which displayed a high Ki-67 labeling index (LI; 22%), was found in a 54-year-old woman who presented with diplopia and headaches. The patient underwent three transsphenoidal operations in less than 6 months and, ultimately, was treated with fractionated intensity-modulated radiation therapy. The relationships between high Ki-67 LIs and tumor recurrence, invasiveness, and growth velocity in pituitary adenomas are reviewed.
W. Jeffrey Elias, M. Beatriz S. Lopes, Wendy L. Golden, John A. Jane Sr. and Federico Gonzalez-Fernandez
✓ Results of recent studies have led investigators to suggest that the retinoblastoma tumor-suppressor (rb) gene plays an underappreciated role in the genesis of brain tumors. Such tumors cause significant rates of mortality in children suffering from hereditary retinoblastoma. It has been assumed that the pineal gland, which is ontogenetically related to the retina, accounts for the intracranial origin of these trilateral neoplasms. To address this issue, the authors describe an unusual trilateral retinoblastoma variant.
The authors provide a detailed clinicopathological correlation by describing the case of a child with bilateral retinoblastoma who died of a medulloblastoma. The intraocular and intracranial neoplasms were characterized by performing detailed imaging, histopathological, and postmortem studies. Karyotype analysis and fluorescence in situ hybridization were used to define the chromosomal defect carried by the patient and members of her family.
An insertion of the q12.3q21.3 segment of chromosome 13 into chromosome 18 at band q23 was identified in members of the patient's family. This translocation was unbalanced in the proband. The intraocular and cerebellar neoplasms were found to be separate primary neoplasms. Furthermore, the pineal gland was normal and the cerebellar neoplasm arose within the vermis as a medulloblastoma. Finally, the two neoplasms had different and characteristically identifiable cytolological and immunohistochemical profiles.
The findings of the present study, taken together with those of recent molecular and transgenic studies, support the emerging concept that rb inactivation is not restricted to central nervous system regions of photoreceptor lineage and that inactivation of this tumor suppressor pathway may be relevant to the determination of etiological factors leading to medulloblastoma in humans.