Pediatric thalamic glioblastoma associated with Ollier disease (multiple enchondromatosis): a rare case of concurrence.

Ollier disease and Maffucci syndrome are rare syndromes in which there is deforming dysplasia of cartilage, primarily but not exclusively involving the metaphyses and diaphyses of long bones. In a minority of patients, dysplasia can lead to sarcomatous degeneration, producing chondrosarcomas. There also appears to be an association with other neoplasms. Little has been written about the association between Ollier disease and intracranial tumors, and these papers have largely consisted of case reports in adults. The authors present the case of a 6-year-old girl with left arm osseous changes consistent with Ollier disease and a biopsy-proven thalamic glioblastoma multiforme. They then examine the co-occurrence of brain tumors in conjunction with a dyschondroplasia syndrome in children and adolescents to assess the presentation, treatment offered, and disease course of similar cases. Eight other such cases were identified, 6 in patients with Ollier disease (ranging in age from 7 to 18 years), and 2 with Maffucci syndrome (both in late adolescence). Including our own patient, 7 of the 9 cases of comorbid dyschondroplasia and intracranial malignancy occurred in girls. Some patients presented soon after the acute onset of symptoms, and others had a more subtle, protracted course over as many as 2 years. Some tumors were deemed resectable and others not. In only 1 instance was follow-up beyond 1 year reported.

Prognostic factors influencing the outcome of thalamic glioma.

Retrospective analysis of 27 patients of thalamic glioma including adults and children treated over a period of 7 years from 1991-1997 was done. The study group included 19 males and 8 females; 9 patients were less than 15 years and 18 patients more than 15 years of age at the time of diagnosis. The commonest symptoms were headache and vomiting. 12 patients underwent VP shunt as an initial procedure and 7 underwent total or partial surgical resection. Confirmed histopathological examination was possible in 16 patients; while 12 had low grade astrocytoma, 4 cases had high grade histology. All patients were treated with radiotherapy to a total dose of 50-60 Gy in 25-30 fractions. Median follow up was 9.63 months. The disease free survival in these patients was 28% at 2 years. Prognostic factors which included age, sex, duration of symptoms, surgical procedures, histology and radiotherapy dose were evaluated for significance. A subtotal resection conferred a better prognosis.

Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy.

BACKGROUND: The outcome of a child with a primitive neuroectodermal tumors arising supratentorially (SPNET) is not well characterized and may differ from the outcome of a patient with a histologically similar cerebellar tumor (medulloblastoma [MB]). Recently, 5-year progression free survival rates as high as 80% have been reported for children with MB treated with craniospinal radiation (CRT) and chemotherapy including cisplatin, lomustine (CCNU), and vincristine (VCR). METHODS: The authors reviewed the outcome of 22 consecutive patients age 3 years and older (mean age, 10 years; range, 3-18 years) with SPNET who were treated at the study institutions between 1981 and 1996. Tumor location included was 13 pineal, 6 cortical, and 3 thalamic or suprasellar. Five patients had disease dissemination at diagnosis. All patients underwent surgery and staging, followed by CRT and chemotherapy with cisplatin, CCNU, and VCR. RESULTS: Of the 22 patients, 13 had developed disease progression and 10 had died at the time of last follow-up. Overall progression free survival (PFS) was 47% +/- 11% at 3 years and 37% +/- 11% at 5 years. There was a significant difference in PFS between patients with localized disease versus those with disseminated disease (P = 0.04). There was no statistical association between tumor location and survival. Although not significant (P = 0.21), there was a trend toward better survival of those patients with complete or near-complete resection compared with those with partial resection or biopsy. CONCLUSIONS: The results of the current study demonstrate that the outcome for children with SPNET treated with radiation and chemotherapy appears worse than for children with MB treated with identical therapy. This suggests that there may be biologic differences between supratentorial and infratentorial primitive neuroectodermal tumors, thus requiring refinements in treatment.

Pleomorphic xanthoastrocytoma: clinical, imaging and pathological features of four cases.

Four cases of pleomorphic xanthoastrocytoma (PXA) were collected from among 688 glioma patients who underwent operation at the Institute of Neurosurgery, University of Naples "Federico II" between January 1973 and December 1994. Three were females and one male, ranging in age from 10 months to 65 years. Three tumors were superficial in location, appearing as a meningo-cerebral mass in the temporo-parietal region. In one case, the tumor was situated deep within the brain (capsulo-thalamic region), without contact with leptomeninges. Three patients had experienced epileptic seizures, whereas one patient presented with an ictal episode. Tumor excision was grossly total in two cases, and subtotal in the remaining two. In three cases, histological examination demonstrated a "typical" PXA; conversely one tumor (subtotal excised) was an "atypical" PXA. The two patients with incomplete surgical resection were postoperatively treated with fractionated brain radiation therapy. Of the two patients who had grossly total removals, one showed tumor recurrence 6 years after surgery, and underwent operation (the recurrent neoplasm did not exhibit malignant transformation); the second patient was free of tumor at 14 months following craniotomy. Of the two patients who had undergone subtotal removals, one died because of massive regrowth of the lesion 22 months after surgery, whereas the second patient was asymptomatic at 1 year follow up.

Radiotherapy of intracranial germinomas.

Between 1980 and 1992, 32 patients with intracranial germinomas were treated with radiation. All patients were confirmed histopathologically prior to treatment. Of the 32 intracranial germinomas reviewed, 14 were located in the suprasellar region, 12 in the basal ganglia and thalamus, four in the pineal, and two in both the pineal and suprasellar regions. Three patients had subarachnoid seeding. Craniospinal irradiation was undertaken for 29 patients. The median dose of 54 Gy was delivered to the tumor bed, 36 Gy to the whole brain and 24 Gy to the spinal axis. Five and 10-year survival rates were 96.9 and 96.9%, respectively. Local control was achieved in all patients except one who died of persistent tumor after 2 months following radiotherapy. No intracranial recurrence or spinal metastasis were found. Tumor site did not relate to the prognosis. One patient developed severe intellectual deterioration, three patients had vertebral growth impairment. The present study confirms the excellent result with radiotherapy alone for patients with germinomas.

Radiation therapy for sarcoid of the thalamus/posterior third ventricle: case report.

There are a limited number of previously reported cases involving the use of radiation therapy for sarcoid of the brain. The case of a 22-year-old man with a thalamic/posterior third ventricle sarcoid mass that grew despite steroid medication is presented. The patient was treated with external beam radiation to a total dose of 20 Gy, with 2-Gy fractions over 14 elapsed days. A complete radiographic response was achieved 4 months after radiation was completed. Radiographic follow-up through 8 months postradiation shows no evidence of disease recurrence. Fractionated radiation therapy in low-to-moderate doses appears to be efficacious in steroid-refractory sarcoid of the brain.

Childhood intracranial meningiomas after high-dose irradiation.

BACKGROUND: Irradiation, either alone or in association with other factors, is thought to play a role in the causation of intracranial meningioma. METHODS: The authors report two 15-year-old patients with convexity meningiomas as a result of high-dose irradiation received at a young age and review the English language literature reports of 13 pediatric patients with meningiomas after high-dose irradiation. The clinical characteristics of the 15 patients are presented. RESULTS: There were nine girls and six boys. The mean age at the time of irradiation was 2.5 years (2 months-9 years), and the mean age at diagnosis of meningioma was 13 years (5-15.5 years). The mean radiation dose was 4154 cGy (1500-8000 cGy). In 11 of the 15 patients, the meningioma was located in the calvarial area. Only 1 of 15 had multiple tumors, and only two of the tumors were clearly malignant at diagnosis. In ten patients, gross total resection was recorded, and two patients underwent subtotal resection. Three died of recurrent/disseminated meningiomas. CONCLUSIONS: This study suggests that meningiomas after high-dose radiation in children are mostly calvarial in location, rarely multiple, mostly benign in histologic type, and that complete removal is possible in most patients. The age at the time of radiation is young (mean age, 2.5 years) and the latent period is short (mean, 10.8 years). Although the clinical course of radiation-induced meningiomas in childhood generally is benign, high doses of radiation at a young age are to be avoided, and other means of therapy should be used if possible.

Episodic exotropia from lateral rectus neuromyotonia--appearance and remission after radiation therapy for a thalamic glioma.

Sixteen months after radiation therapy for a moderately anaplastic astrocytoma of the thalamus, a 7-year-old boy developed spontaneous episodic contractions in his left lateral rectus muscle. During these episodes, lasting 10 to 30 seconds and occurring 20 or more times a day, he had exotropia, limitation of adduction, and retraction of the left globe. This phenomenon peaked in duration and frequency at 14 months and gradually disappeared after 22 months. As this case confirms, radiation injury to the abducens nerve can cause ocular neuromyotonia, resulting in episodic strabismus. Our case also illustrates that this condition can disappear completely after many months.

Brain tumors in children and adolescents--III. Effects of radiation and hormone status on intelligence and on working, associative and serial-order memory.

The effects on intelligence and memory of two post-surgical conditions (radiation treatment, hormone deficiency and supplementation) were explored in 46 children and adolescents with tumors in a variety of brain sites. Verbal intelligence, but not non-verbal intelligence, varied positively with age at radiation treatment. Memory for word meanings was unrelated to either radiation history or to hormone status. Severe deficits in serial position memory occurred with impaired hormone function and an older age at tumor onset. Severe deficits in working memory were associated with a history of radiation and a principal tumor site that involved thalamic/epithalamic brain regions. Radiation treatment and hormone status affect later cognitive function in children and adolescents with brain tumors. Although the greater vulnerability of the verbal intelligence of the younger radiated child and the serial order memory of the child with later tumor onset and hormone disturbances remain to be explained, and although the form of the relationship between radiation and tumor site is not fully understood, the data highlight the need to consider the cognitive consequences of pediatric brain tumors according to a set of markers that include maturational rate, hormone status, radiation history, and principal site of the tumor.

Stereotaxy and thalamic masses. Survey of 44 cases.

Thalamic masses are generally considered inoperable; little is known of the precise nature of these lesions. Stereotactic biopsy was performed in 44 patients, with no mortality and low morbidity (only 1 case of transitory hemiparesis). The stereotactic biopsy (minimum 5 specimens taken along the major axis of the lesion) showed that the majority of the young patients (less than 40 years) had low-grade glial neoplasms (grade I-II astrocytomas or oligodendrogliomas), while in older patients highly malignant tumors prevailed. Beside the neoplasms in the children and adults, we found granulomas, abscesses, infarcts, hemorrhages and glioses. Present neuroradiological methods cannot establish a final correct diagnosis in thalamic lesions, so stereotactic biopsy is recommended. A follow-up of 1-6 years is presented for 7 patients who underwent stereotactic 125I brachytherapy.

Thalamic tumors in children. Long-term follow-up and treatment guidelines.

The authors review the cases of 60 children (aged 5 months to 18 years) with thalamic tumors who were seen at The Hospital for Sick Children between 1951 and 1983. The diagnosis of thalamic tumor was based on neuroradiological studies; 33 children were first seen in the pre-computerized tomography (CT) era and 27 since the advent of CT scanning. Histological diagnosis was obtained in 37 patients at the time of operation. A further four tumors were verified histologically at autopsy. Thirty-six patients required cerebrospinal fluid shunt placement. Sixteen patients had no surgery on their tumor, three underwent needle biopsy, 20 underwent open biopsy, and 21 had partial resection. Forty-four patients were irradiated. All 20 patients with malignant tumors died, with a mean survival time of 1.1 years. Of 19 patients with benign tumors, 11 died, with a mean survival time of 5.3 years, and eight are still alive after a mean period of 7.2 years since diagnosis. Based on this series, the authors recommend open biopsy of thalamic tumors (and resection if deemed safe), followed by radiation in selected cases.

Cystic thalamic gliomas.

Primary tumours of the thalamus are rare and present several diagnostic and therapeutic problems. Cystic thalamic lesions tend to be low grade astrocytomas and have only been reported in children. Signs and symptoms can be divided into those due to intracranial hypertension and those due to loss of function of thalamic nuclei and surrounding structures. Histologic diagnosis can be established by CT-guided needle biopsy and therapy begun by evacuation of the cyst contents. The prognosis remains uncertain.