Pediatric glial neoplasms including brain-stem gliomas.
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The authors review the therapeutic results of 20 patients (aged 12 months to 30 years, mean age 9 years) with benign pilocytic astrocytoma of the chiasmatic/hypothalamic region, seen at the University Hospital Hamburg-Eppendorf between February 1980 and April 1993. Six patients suffered from neurofibromatosis 1 (NF-1). The patients were divided into two subgroups relative to tumour extension and growth patterns, as revealed by CT scans and/or MR imaging. Fourteen patients revealed a large globular suprasellar tumour extending into the hypothalamus and/or the anterior third ventricle (group A). A suprasellar tumour with optic tract involvement could be identified in six cases (group B). Subtotal (70-90%) resection was achieved in ten tumours (50%), the majority of which were of the large globular type. There were no deaths. Postoperative morbidity was comprised of visual and endocrine impairment in four patients and right hemiparesis and dysphasia in one patient. Radiation therapy was given in nine cases (45%). Three tumours (38%) were reduced in size by irradiation. Tumour progression was seen in seven patients (35%). The presence of young age and NF-1 were predictors of progressive disease in our series. Growth capacity was not related to the extent of cytoreductive surgery.
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BACKGROUND AND PURPOSE: Chordoid glioma is a new clinicopathologic entity that occurs in the region of the hypothalamus/anterior third ventricle. The aims of this study were to describe the characteristic radiographic features of chordoid glioma, identify specific imaging features that may enable differentiation of chordoid glioma from other suprasellar tumors, and increase neuroradiologists' awareness of this newly described tumor, facilitating prospective diagnosis. METHODS: CT scans and/or MR images of six patients with chordoid glioma were reviewed retrospectively to determine whether any characteristic radiographic features would emerge. Reports of the clinical presentation, pathologic findings, and radiographic findings of another six patients were reviewed and included, for a total patient population of 12 (mean age +/- SD, 46 +/- 13 years). RESULTS: Imaging features were strikingly similar for all tumors. In each case, the mass was ovoid, was well circumscribed, was located in the region of the hypothalamus/anterior third ventricle, and enhanced uniformly and intensely. Tumors were hyperdense to gray matter on CT scans and were isointense on T1-weighted MR images and slightly hyperintense on long-TR MR images. In two patients, vasogenic edema extended into the optic tracts, and in three, there was hydrocephalus. CONCLUSION: Chordoid glioma is a recently described unique histopathologic entity that has been added to the World Health Organization glioma classification scheme and must be included in the differential diagnosis of a suprasellar mass. Distinctive imaging features are its location, ovoid shape, hyperdensity on CT scans, and uniform intense contrast enhancement.
The authors report the case of a young woman with advanced breast cancer who developed diabetes insipidus due to pituitary involvement and also gastric metastases. This patient had a normal brain CT scan. Gastric metastases were diagnosed when she was operated for a perforated gastric ulcer. Although very rare, and even if the brain CT scan is normal, pituitary metastases should be diagnosed in the presence of suggestive clinical symptoms. Abdominal pain also warrants investigation in these patients in an early attempt to document any possible gastric metastases.
Suprasellar and sellar region tumors in children constitute a diverse group of lesions. We advocate an aggressive surgical approach to these tumors for diagnostic and therapeutic purposes. With detailed knowledge of the microneurosurgical anatomy of this region, the neurosurgeon can choose from a number of operative approaches on the basis of tumor size and location. The benefits of radiation therapy for craniopharyngiomas and optic chiasmatic/hypothalamic tumors must be carefully weighted against the substantial long-term risks associated with irradiation of the developing brain in this region. During the next decade, the role of primary chemotherapy for germinoma and adjuvant chemotherapy for optic pathway gliomas will be determined. Also, the role of stereotactic radiation therapy, of great value to some children with recalcitrant tumors in the suprasellar region, may be better defined.
PP therapy, combination therapy with Cis-platinum (CDDP) and tetrahydropyranyladriamycin (THP-ADR), were performed on a case of recurrent medulloblastoma. CDDP and THP-ADR involve agents difficult to pass the blood brain barrier (BBB), but can pass BBB on the metastatic lesion. PP therapy is considered to be one of the most effective agents for the treatment of recurrent malignant brain tumor.
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A retrospective analysis of 70 pediatric patients (less than 16 years of age) with histologically proven or presumed primary neoplasms of the thalamus, hypothalamus, and brainstem, treated with combined surgery and postoperative radiotherapy or radiotherapy alone at the Washington University Medical Center from January 1950 through December 1983, is reported. Overall survival for all patients at 5 and 10 years was 34.9% and 32.7%, respectively. Follow-up of the 22 surviving patients ranged from 3.0 to 20.0 years (median, 10.6 years). Statistical analysis of multiple prognostic factors was performed. Prognostic factors found by single variate analysis to significantly influence survival were primary site of disease, extent of surgery, race, cranial nerve paresis at diagnosis, and dose of radiation. Factors evaluated but found to be insignificant were age at diagnosis, duration of symptoms before diagnosis, sex, and volume irradiated. Multivariate analysis revealed that only total radiation dose and race were of prognostic significance.
The value of AgNOR staining as a tumor biological marker was tested in 26 children with pilocytic astrocytomas (20) and fibrillary astrocytomas (6). All patients were surgically treated and then followed up by periodic MRI or CT scans. Follow-up ranged from 8 to 84 months, with a mean of 44 months. AgNOR expression was determined by using semi-automated computer-assisted surface area measurements. AgNOR values ranged from 1.4 to 81.4 microm(2) per cell, with a mean of 26.6 and a median of 15.2. The median value was taken as a "cut-off" score separating two groups of patients with low and high AgNOR scores. Of the 13 patients in the low scoring group, 8 had total resections without recurrence, 3 had stable residual tumors, 1 had regressing residual tumor after irradiation and 1 had a recurrence 5 years after neuroradiologically complete resection of a fibrillary astrocytoma. In the group with high AgNOR scores only 2 patients had total resections without recurrence; 5 had stable residual tumors and 6 had residual tumors that showed progression, all within 1 year after surgery. Among the patients with classic juvenile pilocytic astrocytomas of the cerebellum 7 had residual tumor, which progressed in 2 patients, both of whom had high AgNOR scores. Among 7 patients with optic/hypothalamic tumors the 3 with rapidly progressing tumors all had very high AgNOR scores. The determination of AgNOR expression might be helpful in selection of patients with residual tumor after surgery, who may benefit from additional chemotherapy or (stereotactic) radiation therapy.
This paper has reviewed current knowledge of clinical, biochemical, and morphological manifestations of extracranial GRH-producing tumors. Excessive GRH release stimulates pituitary somatotrophs causing elevation of blood GH levels and acromegaly. In some patients with GRH-containing tumor, blood GH concentrations are normal and no acromegaly develops. GRH-producing tumors associated with acromegaly are rare. Based on a critical analysis of the literature, 30 tumors are accepted as definitive. They possess unique features: occurrence in young age, female preponderance, foregut derivation, benign biological behavior, small secretory granules, and frequent association with MEN type I syndrome. The pancreas and lung are common primary sites. GRH-containing tumors unassociated with acromegaly include those of gut and thymus, small cell carcinoma of lung, and medullary carcinoma of thyroid. Several tumors are plurihormonal. In contrast to somatotroph adenoma seen in patients with classical acromegaly, the hypophysial lesion represents somatotroph hyperplasia in acromegalic patients with GRH-producing tumor. This finding indicates that GRH not only increases somatotroph secretory activity but causes somatotroph proliferation. Studies of GRH-producing tumors are of fundamental importance in obtaining a deeper insight into endocrine activity of pituitary somatotrophs and the pathogenesis of GH-secreting pituitary adenomas associated with acromegaly; the importance of GRH in the etiology of acromegaly is still unresolved. The relationship between GRH-secreting tumors and MEN type I syndrome is controversial; further studies are required to elucidate whether they represent two distinct entities or whether GRH-producing tumors accompanied by acromegaly are only forme fruste manifestations of MEN type I syndrome.
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Anterior optic pathways gliomas are classifiable histologically and clinically with other pilocytic gliomas of the midline (of hypothalamus and brainstem), especially of childhood. The very slow course, sometimes imperceptible over the years, that characterizes the majority of them should not be taken to mean lack of growth. Tumultuous courses and acute episodic deteriorations are both possible. Hence the need for therapy. Indefinite abstention cannot be upheld, at least in the great majority of cases. In gliomas of a nerve only the object of surgery is usually removal, using the intraorbital, intracanalicular and intracranial approaches, but in gliomas of the chiasm or chiasm-hypothalamus the object is more exploratory and decompressive. Radiotherapy is generally accepted as affective only in the latter cases. No single standard protocol is possible: treatment had to be individualized case by case.
Occlusion of the intracranial portion of the internal carotid artery were encountered in two cases with suprasellar tumours, who had received irradiation therapy previously and shown excellent clinical improvement postoperatively. Clinical features and the angiographic characteristics were analysed in these two cases and the results were compared with reports in the literature. The occlusive effect of radiation on the arterial wall was conceived as the cause of carotid obstruction. In order to avoid this side effect of radiation therapy, especially for brain tumours of low malignancy in childhood, careful estimation of the radiation dose should be required and cerebral angiographic follow-up during and after the treatment with radiation is indicated, particularly when the patient develops cerebral transient ischaemic symptoms.
In recent years, numerous new entities or variants of recognized central nervous system tumors have been described in the literature, and the morphologic spectrum of these neoplasms is delineated incompletely. The accurate diagnosis and classification of these lesions is important to ensure that patients receive adequate therapy and prognostic information. The clinicopathologic features and differential diagnosis of 4 new entities, including the chordoid glioma of the third ventricle, cerebellar liponeurocytoma, atypical teratoid/rhabdoid tumor, and papillary glioneuronal tumor, are discussed in this review.
Twenty-six patients underwent needle biopsy of tumors in the area of the third ventricle. Operative mortality was zero, and the diagnosis was established in 23 of the 26 patients. There was no permanent neurological deterioration in any of the patients. Needle biopsy yields diagnostic information with small risk, and is particularly helpful in the posterior portion of the third ventricle where tumors are likely to be quite radiosensitive.