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This case report describes a patient who presented with panhypopituitarism secondary to hypothalamic metastasis. A primary hypothalamic abnormality was suggested by computed tomographic (CT) demonstration of a small enhancing circular mass centered within the hypothalamus. Sellar radiographs and cerebral angiography were normal.
Hypothalamic glioma was found in a 17 year old male patient 15 years after irradiation of right optic glioma. It was totally removed by surgery. He was first admitted to Ryukyu University Hospital at the age of 1 year and 11 months, with right exophthalmos and visual disturbance. Diagnosis was anterior type of right optic glioma. This was treated by irradiation (47 Gy). Follow-up CT scans revealed no regrowth in intraorbital and intracranial regions. At the age of 17 years, CT scan and MRI showed an enhanced mass in the hypothalamus above the suprasellar cistern. Total removal was performed by bifrontal interhemispheric trans-lamina terminalis approach. Histological diagnosis was pilocytic astrocytoma. Postoperatively, consciousness remained disturbed for 3 weeks because of infarction in the bilateral medial nucleus of the thalamus. Radical excision of hypothalamic glioma was reviewed in the literature. We concluded that such excision has a potentially good outcome, and should be attempted when circumstances indicate its feasibility.
Five cases of metastatic cancer in the pituitary gland are reported, four of which were revealed by chiasmatic syndrome and one by diabetes insipidus. Transphenoidal surgical treatment was possible in three cases. This site of metastasis is considered exceptional because of its customary inactivity. It is probable that the metastasis most often remain unrecognized and so, it requires careful study of the slightest sign because of treatment possibilities.
Optic chiasmatic-hypothalamic gliomas (OCHGs) have been considered benign tumors and self-limiting in growth potential because of their histological appearance. Unfortunately, most clinical series have reported significant morbidity and mortality especially with the more extensive, posteriorly positioned tumors. The biological behavior of OCHGs is age-dependent, with patients younger than five years and older than 20 years typically having tumors that exhibit aggressive growth. There are no specific pathological features to help differentiate the clinical behavior of such tumors. The emergence of modern imaging techniques, including magnetic resonance imaging (MRI), has facilitated the monitoring of the natural history of the disease and the determination of the effects of therapy. Most patients with OCHGs survive for many years. While the natural history of an OCHG for any individual may be indeterminate, enough data are now available from large series to make recommendations for treatment. Our current treatment policy for patients with OCHGs in the context of NF-I without visual failure is a conservative one involving CSF shunting for hydrocephalus if present and medical therapy for endocrinologic dysfunction. Patients with or without NF-I with visual deterioration or progressive neurological deficits and a rapidly expanding suprasellar mass lesion are treated surgically. After tumor resection, patients whose vision is significantly compromised or who show progression of their disease on serial neuroimaging scans receive chemotherapy. If chemotherapy proves ineffective in disease stabilization, then considerations of radiation therapy are given to children over five years old.
We describe the results of clinical and endocrinological investigations performed on 10 children and adolescents (5 males and 5 females) with a primary central nervous system germinoma. Eight of 10 patients were between 10-20 yr of age at the time of initial presentation. Polyuria (7 of 10) and a decrease in or cessation of linear growth (5 of 10) were the most common presenting symptoms, while only 2 of 10 patients complained of visual problems. Two patients presented with the syndrome of polyuria, adipsia, hypernatremia, profound muscle weakness, and hyperlipidemia. Initial physical exam revealed abnormal eye findings in 60%, short stature (greater than or equal to 2.5 SD) in 50%, and abnormal pubertal development in 30% of the patients. The neoplasm was located in the suprasellar-hypothalamic region in 8, caudate nucleus in 1, and pineal region in 1. Biopsy performed in 7 patients revealed the classic two-cell germinoma in all cases. Assessment of endocrine function before radiotherapy documented pituitary deficits in all patients studied. Antidiuretic hormone was deficient in 8 of 10 patients and was associated with hypoadipsia in 4. GH was deficient in al patients tested (7 of 7). TSH (5 of 8), ACTH (3 of 7), and gonadotropin (1 of 1) deficiencies were also common before treatment. Plasma PRL concentrations were elevated in 5 of 8 patients, all with suprasellar tumors. The hCG values were elevated only in the patient with sexual precocity (1 of 10). Endocrine evaluation during the postirradiation period revealed additional instances of GH (1), ACTH (1), and gonadotropin (5) deficiencies. All 10 patients are alive without evidence of active disease 6 months to 10 yr after radiation therapy (4500-5100 R). Evidence of hypothalamic-pituitary dysfunction is an early and almost universal feature of central nervous system germ cell tumors. The importance of careful evaluation and follow-up of children with acquired anterior or combined anterior and posterior pituitary dysfunction for a suprasellar tumor is stressed.
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BACKGROUND: Chiasmatic-hypothalamic gliomas are not amenable to surgical resection and therefore are treated with either radiotherapy or chemotherapy. Etoposide (VP-16), administered on a long-term oral schedule, represents a novel chemotherapeutic approach. PATIENTS AND METHODS: Fourteen patients (age range, 2 to 15 years) were treated with etoposide following tumor progression as determined by clinical and neuroradiographic examinations. Thirteen patients had received prior radiotherapy, and 12 received prior nitrosourea-based chemotherapy. Etoposide was administered orally; each cycle consisted of 50 mg/m2 per day on days 1 to 21 and days 36 to 57. Clinical and neuroradiographic examinations were performed from days 58 to 72 prior to the start of each cycle of therapy. Complete blood cell counts were performed weekly. RESULTS: Treatment-related complications included partial alopecia (n = 7), diarrhea (n = 6), weight loss (n = 5), neutropenia (n = 4), and thrombocytopenia (n = 4). Three patients required a transfusion (ie, red blood cell [n = 3] and platelet [n = 2] transfusions), and one patient required antibiotic treatment of neutropenic fever. There were no treatment-related deaths. Fourteen patients were evaluable; in eight of these 14 patients, a response was demonstrated radiographically (complete response [n = 1], partial response [n = 4], and stable disease [n = 3]), with a median duration of response of 8 months. CONCLUSIONS: Long-term treatment with oral etoposide was well tolerated by the patients in this study, and etoposide was a relatively nontoxic chemotherapeutic agent with apparent activity in this small cohort of patients who had recurrent chiasmatic-hypothalamic gliomas.
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PURPOSE: Chiasmatic-hypothalamic gliomas are not amenable to surgical resection and therefore are treated with either radiotherapy or chemotherapy. Here we report the use of etoposide (VP-16) administered on a chronic oral schedule as a novel chemotherapeutic approach. PATIENTS AND METHODS: Fourteen patients, aged 2 to 15 years, were treated with VP-16 after clinical and neuroradiographic tumor progression. Thirteen patients had received prior radiotherapy, and 12 received prior nitrosourea-based chemotherapy. VP-16 was administered orally, each cycle consisting of 50 mg/m2/d on day 1 through 21 and 36 through 57. Clinical and neuroradiographic evaluations were performed during days 58 through 72 before initiation of each cycle of therapy. Complete blood counts were performed weekly. RESULTS: Treatment-related complications included the following: partial alopecia (seven patients); diarrhea (six); weight loss (five); neutropenia (four); and thrombocytopenia (four). Three patients required transfusion (three RBC; two platelet), and one patient required antibiotic treatment of neutropenic fever. There were no treatment-related deaths. Fourteen patients were assessable, five of whom demonstrated a radiographic response (one complete and four partial); and three patients demonstrated stable disease, with a median duration of response of 8 months. CONCLUSION: Chronic oral VP-16 is well tolerated, produces modest toxicity, and has apparent activity in this small cohort of patients with recurrent chiasmatic-hypothalamic gliomas.
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The authors report their series of 45 patients harboring inoperable, low-grade cerebral neoplasms, treated in the past 6 years with 125I stereotactic brachytherapy. The majority of these tumors were grade I and II astrocytomas and oligodendrogliomas (82.2%). A 2.6- to 6-year follow-up shows good results in 65.6% with reduction or disappearance of the lesions on CT images and good social reentry. Nine patients (23.7%) died prior to follow-up. Young patients (less than 40 years) responded well to interstitial radiotherapy, while patients over 40 with the same histological findings of low-grade tumors responded poorly to this type of treatment. Diffuse infiltrating cortico-subcortical tumors, optochiasmatic gliomas, hypothalamic and lower brainstem neoplasms do not respond satisfactorily to 125I radioisotope implantations.
We reviewed our experience of 16 patients with histologically proven optico-hypothalamic gliomas. They ranged in age from 0.3 to 15 years at the time of diagnosis. Fifteen tumors were located in the optic chiasm, optic nerve, optic tract and/or hypothalamus, while one tumor was confined to one optic nerve. All tumors were classified as low-grade astrocytomas, which were mainly composed of pilocytic astrocytes. No patient had associated neurofibromatosis. The initial treatment for tumors included surgery in 12, radiotherapy in 7, and chemotherapy in 4 patients. After treatment, visual function improved in 3 out of 14, and endocrine function improved in 1 out of 4 evaluable patients. The 5-year actuarial survival rate was 84.0%, and that at 10 years 71.1%. Our experience and the literature indicate that: (1) patients with disease limited to the optic nerve are adequately managed by resection alone; (2) chiasmal-hypothalamic gliomas behave variably, and progressive disease may occur late in the course of the illness; (3) gliomas that arise in patients under 2 years of age and involve the optic chiasm may act aggressively despite their histological benignity; (4) the beneficial effects of radiotherapy occur in about half of the patients; (5) although chemotherapy may be an effective adjuvant treatment modality, it is not an alternative to radiation therapy at present. Both surgery and irradiation therefore offer the best treatment now available for patients with progressive disease.
At the age of 41 and 31 months, respectively, a boy and a girl affected by neurofibromatosis-1 were diagnosed with a visual pathway glioma during surveillance contrast-enhanced head magnetic resonance imaging (MRI). In the first child, the initial MRI showed that the entire optic chiasm, the intracranial tract of the left optic nerve, and hypothalamus were grossly enlarged and enhanced in the post-gadolinium T1-weighted images. Ten months later, the hypothalamic component of the lesion had regressed markedly and there were no more areas of contrast enhancement. In the second child, the initial MRI showed that the optic chiasm, the right optic tract, and geniculate body were enlarged and enhanced after gadolinium injection. At 6-month follow-up, the MRI showed that the right optic tract and the anterior aspect of the optic chiasm decreased in size and the contrast enhancement of the entire lesion was reduced dramatically. These findings, as indicated by other similar reports, confirm that spontaneous regression of visual pathway glioma is a rare but real possibility in children with neurofibromatosis-1. Therefore, clinicians need to be aware of visual pathway glioma's erratic behavior in children with neurofibromatosis-1 with special attention given to the importance of a very conservative attitude toward any type of treatment for such patients.
Between March, 1983, and February, 1989, 19 infants or children with chiasmal/hypothalamic gliomas were treated with chemotherapy after either surgical or radiological diagnosis. The patients ranged in age from 15 weeks to 15.6 years (median 3.2 years) at the start of therapy. Twelve patients were treated immediately after diagnosis because of progressive symptoms, and seven received chemotherapy after either radiographic progression or clinical deterioration, including progressive visual loss or intracranial hypertension. Based on biopsy results, seven of these tumors were classified as juvenile pilocytic astrocytomas, two as astrocytomas, two as highly anaplastic astrocytomas, and one as a subependymal giant-cell astrocytoma. There was associated neurofibromatosis in four patients. The two initial patients were treated with either actinomycin D and vincristine or 5-fluorouracil, hydroxyurea, and 6-thioguanine. The remaining patients received nitrosourea-based therapy; 15 evaluable patients were treated with a five-drug regimen that included 6-thioguanine, procarbazine, dibromodulcitol, 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU), and vincristine and one received 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU) and 5-fluorouracil. Fifteen of the 18 evaluable patients initially managed with chemotherapy either responded to therapy or their condition stabilized. Median time to tumor progression has not been reached at a median follow-up period of 79 weeks (range 6.6 to 303 weeks), and no tumor-related death has occurred with a median follow-up period of 79 weeks (range 18 to 322 weeks) from the initiation of therapy. The four patients who failed therapy or whose disease progressed after chemotherapy were treated satisfactorily with radiation therapy. Initial improvement or stabilization of visual function was obtained in 16 patients. Endocrine function remained stable in all patients during treatment, although three patients required pharmacological treatment for endocrinopathy that was present at diagnosis. These preliminary results suggest that nitrosourea-based cytotoxic regimens are useful for the initial treatment of children with chiasmal/hypothalamic gliomas, and allow potentially harmful radiation therapy to be deferred until progression of disease.
A unique combination of occurrence of the 3rd ventricular germinoma and hypothalamic cystic mixed astrocytoma and oligodendroglioma is encountered in a 15-year-old man who presented clinically with stunted growth of the body, diabetes insipidus, and stupor. The germinoma was diagnosed by biopsy but the hypothalamic glioma was discovered at autopsy. Immunohistochemical localization of glial fibrillary acidic protein (GFAP) in the neoplastic cells confirmed the gliomatous nature of the hypothalamic lesion. The probability of multiple tumors should be kept in mind in dealing with intracranial neoplasms in order that the patient will be properly diagnosed and managed.
Whilst many children diagnosed with cancer can now realistically hope for long term survival, the consequences of cancer treatment can be particularly devastating as they enter adolescence and adulthood. Disruption of the endocrine system can result from such treatment, including growth hormone deficiency, problems in normal pubertal progression and thyroid dysfunction. Fertility can also be affected by cancer treatment received as a child, which can have a devastating impact as the patient enters adulthood. In addition, these children may encounter disorders of growth and bone metabolism due to both their initial disease and aspects of its treatment, resulting in further morbidity in later life. The aetiology and diagnosis of these problems are discussed in this review, along with therapeutic options in order to reduce their impact. Long term follow up and clinical vigilance in this patient group is vital. We must continue to strive towards improved survival from childhood cancer, but equally we must remain aware of the adverse effects of treatment, particularly in the long term, and must aim to reduce the impact of these effects as children enter adolescence and adult life.