Meningeal tumors in children. 1983.
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Adolescent↗
Biomedical subjects
L N Sutton
Publications and source records attributed to L N Sutton.
Subependymal giant cell astrocytomas in children.
Between 1977 and 1991, at the Children's Hospital of Philadelphia, 10 patients, 5-16 years of age, were diagnosed as having subependymal giant cell astrocytomas. These patients accounted for 1.4% of all pediatric brain tumors seen during that time interval. One patient received a course of radiation therapy, which was ineffective in preventing tumor growth. All underwent surgical resections with the goal being maximal tumor debulking, if not complete resection. In 6 patients this was accomplished by the frontal transventricular route and, in the more recent patients, surgery was performed using a transcallosal approach. There were 2 perioperative deaths, and 2 other patients died later of causes unrelated to tumor progression. The remaining 6 patients remain alive and stable at a mean of 6.7 years of follow-up (range 1.8-12.4). None of these patients has received additional radiation therapy. Two patients have no other evidence of tuberous sclerosis. The use of modern radiographic and surgical techniques has made the treatment of this disease safer than in the past.
Adolescent↗
Vascular complications of surgery for craniopharyngioma and hypothalamic glioma.
Vascular complications of surgery for suprasellar tumors include carotid artery laceration, vasospasm, and delayed aneurysmal dilatation of the carotid artery. We report updated follow-up (5-11 years) of 9 patients previously reported with fusiform dilatation of the carotid artery (FDCA) following radical surgical excision of craniopharyngioma, who represent 15.7% of 57 patients operated between 1982 and 1993. None of these patients have experienced hemorrhage or other symptoms referable to the carotid artery abnormality, and none have required treatment. We also report an additional 2 cases of FDCA which were discovered 8 and 11 years after surgery for chiasmatic/hypothalamic astrocytoma. One of these patients experienced headaches and possible hemorrhage, and underwent surgery for the lesion with a poor result. Unless further experience suggests otherwise, it is recommended that asymptomatic patients with this lesion be simply observed, and that surgical exploration for FDCA be reserved for those patients who experience symptoms.
Adolescent↗
Seizure control following tumor surgery for childhood cortical low-grade gliomas.
Detailed preoperative electroencephalographic (EEG) studies are now recommended for children with seizures and cortical tumors to define seizure foci prior to surgery. To develop a historical perspective for better evaluation of results from series reporting tumor removal combined with resection of seizure foci, the authors reviewed seizure outcome in 60 children with seizures and low-grade neoplasms treated consecutively since 1981 by surgical resection without concomitant EEG monitoring or electrocortical mapping. Forty-seven of the 60 tumors were totally or near-totally resected; 45 patients were seizure-free and two were significantly improved 1 year following surgery. Of the 50 children in this series with more than five seizures prior to surgery, 36 were seizure-free, two were significantly improved, and 12 were not improved. Factors associated with poor seizure control included a parietal tumor location, a partial tumor resection, and a history of seizures for more than 1 year prior to surgery. The children at highest risk for poor seizure control at 2 years had experienced seizures for more than 1 year prior to surgery and had undergone partial resection of their parietal low-grade glial tumors or gangliogliomas. In contradistinction, the best seizure control was seen in patients with totally resected low-grade gliomas or gangliogliomas who had experienced seizures for less than 1 year (concordance rates for being seizure-free ranged from 78% to 86%). Long-term seizure control remained excellent. These results suggest that seizure control can be obtained 2 years following tumor surgery in the majority of children with presumed tumors after extensive tumor resection without concomitant EEG monitoring or electrocortical mapping.
Adolescent↗
High-resolution 1H-magnetic resonance spectroscopy of pediatric posterior fossa tumors in vitro.
High-resolution proton magnetic resonance (MR) spectroscopy was performed on perchlorate extracts of tumors (24 cases) or peritumoral vermis (five cases) obtained at surgery. Fifteen tumors were typical cerebellar astrocytomas and nine were posterior fossa primitive neuroectodermal tumors/medulloblastomas. Spectra obtained from the five samples of peritumoral vermis revealed a pattern of metabolites similar to that reported for cerebellar tissue, but concentrations of most metabolites were low, perhaps due to dilution from peritumoral edema. The astrocytomas were characterized by high levels of valine, alanine, and choline, an increase in the choline:N-acetylaspartate (NAA) ratio, and a shift from glutamate to glutamine. Elevations in lactate, pyruvate, and glucose were the result of ischemia during sampling. The primitive neuroectodermal tumors/medulloblastomas were distinguished from astrocytomas by a greater increase in the choline:NAA ratio, a smaller decrease in the glutamate:glutamine ratio, and a relative increase in glycine, taurine, and inositol levels. These metabolic patterns may be of value diagnostically as in vivo MR spectroscopy achieves higher resolution.
Adolescent↗
Outcome for children with medulloblastoma treated with radiation and cisplatin, CCNU, and vincristine chemotherapy.
It has previously been reported in a single-institution trial that progression-free survival of children with medulloblastoma treated with radiotherapy and 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU), cisplatin, and vincristine chemotherapy during and after radiotherapy was better than the outcome in children treated with radiotherapy alone. To better characterize long-term outcome and duration of disease control, this treatment approach was used for 10 years and expanded to three institutions. Sixty-three children with posterior fossa medulloblastomas were treated with craniospinal local-boost radiotherapy and adjuvant chemotherapy with vincristine weekly during radiotherapy followed by eight 6-week cycles of cisplatin, CCNU, and vincristine. To be eligible for study entry, patients had to be older than 18 months of age at diagnosis and have a subtotal resection, evidence of metastatic disease, and/or brainstem involvement. Patients younger than 5 years of age and without these poor risk factors who received reduced-dose craniospinal radiotherapy (2400 cGy) were also eligible for entry into the study. Sixty-three of 66 eligible patients (95%) were entered and placed on this treatment regimen. Forty-two patients had brainstem involvement, 15 had metastatic disease at the time of diagnosis, and 19 had received a subtotal resection. Progression-free survival for the entire group at 5 years is 85% +/- 6%. Three children have succumbed to a second malignancy, and overall 5-year event-free survival is 83% +/- 6%. Progression-free survival was not adversely affected by younger age at diagnosis, brainstem involvement, or subtotal resection. Five-year actuarial progression-free survival for patients who received reduced-dose radiotherapy was similar to that for patients receiving conventional-dose radiotherapy. Patients with metastatic disease at the time of diagnosis had a 5-year progression-free survival rate of 67% +/- 15%, as compared to 90% +/- 6% for those patients with localized disease at the time of diagnosis (p = 0.037). The authors conclude that overall progression-free survival remains excellent for children with posterior fossa medulloblastomas treated with this drug regimen. Chemotherapy has a definite role in the management of children with medulloblastoma. Further studies are indicated to define which subpopulations of children with medulloblastoma benefit from chemotherapy and what regimens are optimum in increasing disease control and, possibly, in reducing the amount of radiotherapy required.
Adolescent↗
Results of a pilot study of low-dose craniospinal radiation therapy plus chemotherapy for children younger than 5 years with primitive neuroectodermal tumors.
BACKGROUND: Children younger than 5 years who have posterior fossa (PF) primitive neuroectodermal tumors (PNET) have a poor prognosis. Because the use of low-dose craniospinal radiation therapy (CSRT) alone has been associated with a higher relapse rate in these patients, and because standard dose CSRT is associated with profound late sequelae, the authors embarked on a study using a combination of low-dose CSRT and adjuvant chemotherapy. METHODS: Between January 1988 and March 1990, ten patients with PF PNET were treated on an institutional pilot trial. The trial included 1800 cGy radiation therapy (RT) to the craniospinal axis, a PF boost to 5040-5580 cGy and chemotherapy consisting of vincristine weekly during RT. This was followed by vincristine, cisplatin, and lomustine for eight cycles administered every 6 weeks. Patients between 18 and 60 months of age without evidence of tumor dissemination were eligible for study. Follow-up is available to October 1992, with a median follow-up of 4 years from diagnosis. All patients have completed therapy. RESULTS: Actuarial survival at just more than 4 years is 69%. Three of the ten patients have died after experiencing relapse. In one, the relapse developed in the spine and brain outside the PF; in the second, concurrently in the PF, brain, and spine; and in the third, only in the spine. In one of the three, one of two initial cerebrospinal fluid cytologic examinations showed one clump of tumor cells, and the other sample appeared normal. Neuropsychologic testing has been a routine aspect of the study. A mean intelligent quotient (IQ) score of 103 in six patients surviving at least 1 year is unchanged from the baseline group score of 107. Five children have been tested at baseline and at 2 years after RT; for these children, baseline IQ was 101 and 2-year IQ was 102. These results stand in sharp contrast to earlier studies from this institution that found children younger than 7 years at diagnosis showing marked IQ losses after RT at 1 and 2-year follow-up. CONCLUSIONS: The results of this study suggest that 1800 cGy CSRT in conjunction with the chemotherapy used may produce less neurocognitive damage, perhaps at the expense of relapse along the craniospinal axis. Better means of improving survival without increasing toxicity are needed.
Brain Neoplasms↗
Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy. Case report.
Sinus histiocytosis with massive lymphadenopathy was first described in 1969 by Rosai and Dorfman. The typical clinical characteristics of this disease include painless cervical lymphadenopathy, fever, and weight loss. The condition can present with an extranodal mass in about 25% of patients, and isolated masses without lymph node involvement occur rarely. The authors describe a 5-year-old boy with cavernous sinus syndrome due to an isolated extranodal form of sinus histiocytosis with massive lymphadenopathy in the temporal fossa. Several cases of this disease involving the central nervous system are reviewed. The histopathological and magnetic resonance imaging characteristics are discussed.
Cavernous Sinus↗
Mannitol, intracranial pressure, and vasogenic edema.
The effect of a 2 g/kg intravenous bolus of mannitol on intracranial pressure (ICP) and white matter water content was determined by following the time course of ICP and spatial white matter water content after administration of mannitol in a hemispheric cold lesion model of vasogenic edema in the cat. After mannitol infusion, maximal ICP reduction occurred at approximately 20 minutes and began to return to baseline after 26 minutes. White matter water content in the control hemisphere began to decrease much later, reaching a minimum at 60 minutes. White matter water content in the lesioned hemisphere was not changed significantly after mannitol infusion. We conclude that the rapid reduction of ICP after intravenous administration of mannitol is not solely due to the dehydration of white matter.
Animals↗
Vascular malformation with radiation vasculopathy after treatment of chiasmatic/hypothalamic glioma.
Chiasmatic/hypothalamic gliomas usually are histologically benign astrocytomas that may recur many years after diagnosis and treatment. Three children with chiasmatic/hypothalamic gliomas who were treated at the authors' institution returned 9.5, 11.5, and 2 years, respectively, after radiation therapy (RT) because visual and neurologic deterioration developed. Neuroradiographic studies, including arteriography in two of the patients, showed large mass lesions. These were presumed to be recurrence of tumor, and chemotherapy was administered. Pathologic examination of two children who died and of the third who had a biopsy revealed only a minimal amount of residual, histologically benign astrocytoma, whereas the bulk of the specimen consisted of numerous vessels of variable size. These probably represented incorporation of the rich vasculature in the chiasmal region into the tumor, which underwent degeneration secondary to RT. Radiographic methods did not distinguish progressive tumor growth from the vasculopathy and led to inappropriate clinical diagnoses and treatment.
Adolescent↗
Choroid plexus carcinoma of childhood.
The presentation, growth patterns, and response to therapy of 11 consecutive children with choroid plexus carcinomas were analyzed, and the results were compared with the outcome reported in other series. Patients were a median of 26 months of age at diagnosis. Two patients had thalamic tumors, one had a posterior fossa primary, and the rest had ventricular lesions. Five of 11 (45%) children remain in continuous progression-free remission a median of 48 months from diagnosis. Four of the five in continuous remission had a "gross total" surgical resection, and only one received radiation therapy. Five of six patients with subtotal resections relapsed despite postoperative treatment with radiation therapy (three) and chemotherapy (one). The response to treatment with radiation therapy or chemotherapy at relapse was disappointing, with only one child (treated with etoposide) responding. In combination with other series, 11 of 14 children had prolonged progression-free survival after gross total resection (only two of whom received adjuvant therapy) compared with two of 20 after less than total resections, independent of the type of adjuvant therapy given. Adjuvant therapy for children with choroid plexus carcinomas is of unproven benefit, and this must be considered when analyzing innovative treatment trials for such children, especially for those with totally resected tumors. Patients with partially resected lesions fare poorly with present forms of treatment.
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Three- and four-year cognitive outcome in children with noncortical brain tumors treated with whole-brain radiotherapy.
Cognitive function and school achievement were studied prospectively over 3 to 4 years in 19 children treated for brain tumors with whole-brain radiotherapy; 14 of 19 also received adjuvant chemotherapy. For the group as a whole, mean IQ fell from a baseline of 104 to 92 at follow-up (p < 0.01). Age was inversely correlated with change in IQ over time (r = 0.71; p < 0.001). Children younger than 7 years at diagnosis had a mean IQ loss of 27 points, while children over 7 years at diagnosis showed no significant decrease in IQ. Decline in IQ occurred between baseline and year 2 of follow-up; none could be documented between years 2 and 4. All children younger than 7 years at diagnosis were receiving special education at follow-up; 50% of the children over 7 years at diagnosis were receiving supplemental educational services.
Adolescent↗
Desmoplastic primitive neuroectodermal tumor with divergent differentiation. Broadening the spectrum of desmoplastic infantile neuroepithelial tumors.
We report an unusual large, multicystic, posterior fossa neuroepithelial neoplasm involving the cerebellum, brain-stem, and quadrigeminal cistern of a 9-month-old girl. The neoplasm consisted of variably sized, sharply demarcated nests of small cells with a high nuclear-cytoplasmic ratio and moderately basophilic nuclei, embedded in a desmoplastic, immature-appearing, mesenchymal stroma. The nests contained mitoses but none were seen in the stroma. Glial fibrillary acidic protein (GFAP), neurofilament protein, synaptophysin, and cytokeratin (AE-1) were expressed in the nests. Mesenchymal cells were negative for neural markers but positive for vimentin and desmin. The neoplasm was interpreted as a mixed mesenchymal and primitive neuroectodermal tumor (PNET) with histologic features reminiscent of a recently described intraabdominal desmoplastic small cell tumor. The tumor responded poorly to chemotherapy and a second operation was performed 1 year later. The second specimen bore no resemblance to the original and consisted of epithelial-like nests and clusters of neoplastic cells frequently interrupted by sinusoidal vessels. Tumor cells had medium-sized vesicular nuclei with small nucleoli, and a granular cytoplasm. Occasional less cellular islands of neuropil-like tissue contained larger cells having eccentric, vesicular nuclei with prominent nucleoli and abundant pink cytoplasm. Mitoses were not conspicuous. Many cells expressed synaptophysin, neurofilament protein, and GFAP. Neurofilament protein was strongly positive in the larger, neuron-like cells and synaptophysin stained the neuropil-like areas strongly but was less prominent in the neuronal perikarya. Unexpectedly, the neuropil-like areas expressed epithelial membrane antigen, whereas the neuronal cells were negative for chromogranin A. The peculiar histologic picture, combination of phenotypic markers, and remarkable biologic behavior of this unusual tumor defies classification according to existing nomenclature and exemplifies the broad range of phenotypes expressed by primitive neuro-epithelial neoplasms.
Brain Neoplasms↗
Childhood meningiomas. Experience in the modern imaging era.
Twenty children with meningiomas (ages 18 months to 17 years) received initial therapy at the Children's Hospital of Philadelphia between January 1975 and June 1991, accounting for 2% of children with primary brain tumors seen during that time interval. All were verified histopathologically, and none had had prior irradiation. Fifteen were male and 5 female. Fifteen tumors were intracranial, all located supratentorially. Two of these also had a component within the optic canal. One tumor was entirely within the orbit. Four meningiomas arose within the spinal canal. Associated conditions were neurofibromatosis (NF) type I (1 patient), NF type II (2 patients), and a facial alveolar rhabdomyosarcoma (1 patient). A gross total resection as documented by postoperative scan and operative note was accomplished in 12 patients. Four of these relapsed, at a mean of 3.5 years from initial surgery. In 4 patients a near-total resection (> 90%) was performed. Of these, 2 progressed at 9 months and 1.5 years. One of these died of complications associated with reoperation. In 4 patients a partial resection (50-90%) was performed. Two of these progressed at 4 months and 1 year, and the other 2 have been followed for less than 2 years. Five patients received radiation therapy (RT). One patient received RT as adjunctive therapy after primary surgery because of papillary histology. The other 4 had RT following reoperation for recurrence at a mean of 1.5 years from diagnosis (range, 7 months to 2 years). These 4 patients remain alive and with stable disease at a mean of 6 years from diagnosis (range 2-8.8 years).(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
Proton magnetic resonance spectroscopy of pediatric brain tumors.
1H magnetic resonance spectroscopy allows the regional quantitation of a number of metabolites from the brain in a noninvasive fashion. Spectra were obtained from 5 normal children and 25 children with brain tumors. Choline (Cho), N-acetylaspartate (NAA), creatine and phosphocreatine, and lactate were quantitated in the form of ratios. The brains of normal children showed relatively high concentrations of Cho and NAA and virtually no lactate, as has been shown in adults. Benign astrocytomas and ependymomas were characterized by an elevation of the Cho:NAA ratio and an abnormal accumulation of lactate. Intrinsic malignant tumors were remarkable for an even higher Cho:NAA ratio but had no more lactate than was found in the benign tumors. Proton magnetic resonance spectroscopy may prove useful in characterizing neoplastic tissue in conjunction with more conventional imaging modalities.
Adolescent↗
Midline supratentorial tumors.
Tumors of the supratentorial midline are common in children and are often amenable to an aggressive operative resection. MR imaging is invaluable to planning an operative strategy. A variety of surgical corridors provide access to the deep midline structures, notably the transcallosal, transventricular, and basal routes. The histology is important in determining the value of adjuvant treatments, such as radiation therapy and chemotherapy. These complex tumors are best managed by a multidisciplinary group with a special interest in pediatric neuro-oncology.
Cerebral Ventricle Neoplasms↗
Head injury in very young children: mechanisms, injury types, and ophthalmologic findings in 100 hospitalized patients younger than 2 years of age.
Head injury in the youngest age group is distinct from that occurring in older children or adults because of differences in mechanisms, injury thresholds, and the frequency with which the question of child abuse is encountered. To analyze some of these characteristics in very young children, the authors prospectively studied 100 consecutively admitted head-injured patients 24 months of age or younger who were drawn from three institutions. Mechanism of injury, injury type, and associated injuries were recorded. All patients underwent ophthalmologic examination to document the presence of retinal hemorrhages. An algorithm incorporating injury type, best history, and associated findings was used to classify each injury as inflicted or accidental. The results confirmed that most head injuries in children younger than 2 years of age occurred from falls, and while different fall heights were associated with different injury types, most household falls were neurologically benign. Using strict criteria, 24% of injuries were presumed inflicted, and an additional 32% were suspicious for abuse, neglect, or social or family problems. Intradural hemorrhage was much more likely to occur from motor vehicle accidents and inflicted injury than from any other mechanism, with the latter being the most common cause of mortality. Retinal hemorrhages were seen in serious accidental head injury but were most commonly encountered in inflicted injury. The presence of more serious injuries associated with particular mechanisms may be related to a predominance of rotational rather than translational forces acting on the head.
Accidental Falls↗
Congenital forms of intracranial teratoma.
Intracranial teratoma in the newborn is a well-recognized but infrequent entity. We describe two such cases: one with a massive intracranial teratoma replacing the cerebral hemispheres and the other, a smaller tumor extending into the orbit. Forms of congenital intracranial teratoma and theories of pathogenesis and management are briefly discussed.
Brain Neoplasms↗