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The pineal hormone melatonin (MLT) is secreted in a circadian rhythm with high serum levels during nighttime and low serum levels during daytime. Several authors have reported an altered secretion pattern of MLT in patients with pineal tumors and have proposed that MLT may be used as a tumor marker. In nine patients, a pineal region tumor was diagnosed by computer-assisted tomography. Before and after surgical removal of the tumor, several day- and nighttime serum samples were collected and MLT concentrations were estimated by radioimmunoassay. Before operation, five patients presented a normal circadian pattern of MLT secretion. In the remaining four subjects, MLT levels were undetectable or at the limit of detection, with no signs of a circadian secretion pattern. Eight patients were reexamined after tumor resection, when all but one had undetectable or very low MLT levels. The remaining subject, with a pineomesencephalic pilocytic astrocytoma, dislocating but not involving the pineal gland, presented a normal circadian secretion pattern of MLT after operation; in this case, tumor resection was possible without damaging the pineal gland. Thus, before operation, MLT deficiency rather than exaggerated serum levels may be used as a marker for pineal tumors that destroy the pineal gland. After tumor resection, serum MLT may serve to demonstrate complete pinealectomy.

Brain scintigrams with 8-10 mci of pertechnetate were studied refering to surgical, histological and other neuroradiological findings in 91 cases with diagnosis or suspect of basal midline lesions. Anterior view of 45 cases was stored in high speed magnetic tape, displayed on CRT of our data processing system and studied of the ratio of average count for regions of interest, 2 cm x 2 cm in size, placed on the areas of lesion, the sagittal sinus and the normal brain hemispheres. In 18 pituitary adenomas, excluding acromegaly and other intrasellar lesions, 89% of cases with surgical indication for optic nerve symptoms were reported as abnormal scintigrams. In 20 craniopharyngiomas, 11 positive cases consisted mainly of solid, recurrent or thick cystic tumors. Five of 6 ectopic pinealomas and all 6 parasellar or medial sphenoidal ridge meningiomas showed positive uptake. Average counts of the regions of interest placed on tumor areas were 169.4% of normal hemispheric areas in 9 pituitary adenomas, 192.5% in 3 solid craniopharyngiomas, 192.3% in 6 meningiomas and 193.3% in ectopic pinealomas. The difference in the average ratio of the lesion count to the normal hemispheric count was statistically significant between cystic craniopharyngioma and adenoma, ectopic pinealoma, meningioma, glioma and solid craniopharyngioma, and between adenoma and acromegaly with p less than 0.005, and between solid craniopharyngioma and acromegaly, and between glioma and acromegaly with p less than 0.025. In the ratio of the lesion count to the sagittal sinus count, on the other hand, the difference of the average ratio was significant with p less than 0.005, only between cystic craniopharyngioma and ectopic pinealoma, and between cystic and solid craniopharyngioma. These facts suggested that the sagittal sinus count was unsuitable to be the standard count of an anterior scintigram to compare with basal midline count. The routine Polaroid scintigram with Tc99m pertechnetate proved their useful clinical diagnostic value for various basal midline lesions, the size of which indicated the surgical procedures. The digital analysis of anterior scintigrams supported the clinical value of the routine brain scintigram in the detection of these lesions. The ratio of the average count of the basal midline lesion to the normal brain area on the anterior scintigram presents more useful clinical information than the ratio of the lesion to the sagittal sinus count. Brain scintigram is found to be very helpful for the differential diagnosis between solid and cystic sellar tumors which is very important for the decision of surgical indications, and is not always possible by any other conventional neuroradiological procedures.

The diagnostic value of brain scintigraphy in intracranial diseases has been well recognized. Numerous reports have appeared in the literature concerning its use in adult cases. The reports dealing with pediatrics cases, however, are quite limited in number. The major factors limiting such studies in children may be considered as follows; (1) infratentorial tumors are common in this age group, in which its diagnostic accuracy has been believed to be quite low, (2) problems of exlosing children to radiation and (3) difficulty in keeping children in one position for a considerable length of time. These difficulties were almost overcome by the use of 99m Tc with a very short half life and a gamma camera, resulting in increased application of this procedure to children. The purpose of this report is to analyse and discuss the scintigraphical characteristics in children and evaluate the diagnostic value in brain tumors. The subjects consist of 199 non-tumoral cases and 53 cases of verified brain tumors. Following conclusions were obtained: (1) Aging changes are noted in normal brain scintigrams of children. One of them is related to the mode of visualization of the basal structures in lateral views. The cranial base appears to be flat before the age of 4 years. A triangular process then appears at the cranial base, approaching that of adult over the age of 10 years. Another change is related to appearance of the temporal and occipital muscles over the cranial vault and the posterior fossa. This becomes apparent about the age of 7 years and produces such masking effects as to interfere with diagnosis in this region over the age of 10 years. (2) The choroid plexus are visualized in about 10% of cases. (3) In brain tumor cases, the detecting rate is 93% in gliomas, and 73% in non-gliomatous tumors. It is particularly low in congenital tumors.

Clinical and neuropathological findings in monozygous twin sisters are reported. Twin I died at age 5 months from a cerebellar medulloblastoma, whereas her sister died at age 16 months from a pineoblastoma. The tumors are considered similar referring to histogenesis, structural peculiarities and growth. Therefore, the twins are seen as concordant for the tumor type.

A total of 31 brain tumors (21 verified histologically, 10 by clinical and neuroradiologic methods) in children aged from 7 months to 13 years were investigated by scintigraphy with 99mTc as a radioactive isotope tracer. There were 16 supratentorial and 15 infratentorial tumors. The supratentorial tumors were detected in 88%, and the infratentorial in 13%, of case by brain isotope scanning. The advantages and disadvantages of the method are discussed.

From January, 1969 to December, 1975, 1700 patients underwent sequential brain scintigraphy with Tc-99-m-pertechnetate. All the scintigrams were obtained on a scintillation camera with high-resolution collimator in our clinic. Intracranial tumors were confirmed in 449 cases and 21 cases out of those were pineal tumors which were diagnosed by clinical symptoms, air study, angiography and Conray-or Myodil-ventriculography. In these pineal tumors, 14 (67%) out of the 21 cases had positive scan on scintigrams. All of our cases of teratoma (one case) and teratocarcinoma (two cases) showed clear abnormal uptake, though it had been reported that they do not tend to have positive results on scintigrams. We also performed scintigraphy on 11 cases out of the 21, both before and after 60Co radiation therapy. In the cases which showed either same grade or more increased abnormal uptake on scintigrams after radiation therapy, compared with that before radiation therapy, compared with that before radiation, their prognoses were poor. On the other hand, those cases which showed decreased abnormal uptake after radiation, have had a healthy daily activities. From these experiences, it may be concluded that brain scintigraphy is clinically valuable not only for making the diagnosis, but for evaluating the effect of radiation therapy and the prognosis in the cases of pineal tumor.

Cystic spongioblastoma and ectopic pinealoma occurring simultaneously were found in a 16-year-old male patient, and produced destruction of the hypothalamus. The clinical course extended for over four years. The clinical picture was characterized by a defect of osmo- and thermoregulation and by defective function of diencephalic nuclei and the sympathetic nervous system. The releasing factors for ACTH, TSH, LH, and FSH were lacking and produced corresponding disturbances of pituitary function. Because of the lack of hypothalamic inhibiting factors the prolactin level was increased, and the HGH level was stimulated by arginine loading and inhibited in the glucose test. The intact neurones in the ventromedial nucleus of the hypothalamus could be seen on microscopical examination. Clinical and endrocrinological findings were more suggestive of the diagnosis than the radiological ones. Computerized tomography showed multiple "tumour" localizations without any displacement signs. The occurrence of ectopic pinealoma and spongioblastoma in the same case would suggest, from the pathological point of view, a common dysontogenetic origin developing from the local elements of the nervous tissue.

This is a report of clinical, morphological, diagnostic, endocrinological and therapeutical experiences with 18 patients with tumours in the pineal region. The histological diagnosis was verified in four cases by autopsy, in seven cases by biopsy, and in one case by microscopical verification of tumour cells in the CSF. In all biopsy cases we are dealing with typical germinomas. In the other clinical cases diagnosis was made by neuroradiological and endocrinological methods. The localization was possible by encephalotomography or CT scan, according to Kageyama, Particular attention was given to the endocrinological dysfunctions which originate in the hypothalamus. Also the hypothalamic dysfunctions after irradiation were discussed. Since the results of primary surgical approach and biopsy have been unsatisfactory, we preferred a non-operative schedule for treatment of pineal tumours.

The computed axial tomographic (CAT) findings in 22 children with intracranial neoplasms are presented. CAT offers a rapid, non-invasive method of assessing the locality and extent of these tumours, as well as the presence of calcification and hydrocephalus. CAT is of value in the follow-up treatment, but an absolute histological diagnosis is not always possible.

In thirty-seven MR examinations of intracranial tumours equivalent sections were obtained in a multi-echo technique before and after intravenous injection of 0.1 mmol. gadolinium DTPA/kg body weight. From this comparison the following preliminary conclusions have been drawn concerning the demonstration of the tumour, its delineation and type: Contrast administration does not unequivocally improve the sensitivity. In 55% of the cases, differentiation between tumour and oedema respectively normal brain tissue was easier after Gd-DTPA. Diffusely infiltrating gliomas remain a problem, since their extent is uncertain with or without contrast medium. The structure of the tumour can already be adequately characterized by the multi-echo technique. In order to diagnose the type of tumour, the criteria which apply to Gd-DTPA are similar to those used for iodine-containing contrast media in CT.

The authors report the case of a 31-year-old female patient who 10 years earlier had displayed diabetes insipidus and hyperglycemia and for 4 years galactorrhea and amenorrhea. Since one year her visual acuity had gradually declined to blindness. CT-scan revealed the presence of a tumoural formation in the optic chiasma and hypothalamic region. The histological examination of the operative-removed tumour showed that it was a type B pinealoma. The postoperative course was good for 42 hrs after which the patient died suddenly. Necropsy disclosed the presence of a myocardial infarct. The authors distinguish three categories of ectopic pinealoma: 1. tumours of the pineal gland with an aberrant development at the level of the 3rd ventricle, 2. metastatic pinealoma, and 3. ectopic pinealomas with an independent development in patients with normal pineal gland. The case reported below is of the 3rd category which is also the most veridical.

This work outlines the endocrine abnormalities associated with intracranial germinomas (14 patients before treatment). Diabetes insipidus of various intensity was present in all cases. Adipsia is often present as well. In six patients, the first neuroradiologic examination after the onset of DI was normal. Growth hormone secretion was deficient in 11 of 13 patients. Abnormalities of TSH response to TRH were present in nine of ten (four insufficient and five exaggerated responses to TRH). Anterior pituitary dysfunction could not be predicted by the tumor site as determined by radiologic criteria. Tumoral markers have also been studied. Elevated plasma tumoral markers were found in four patients of 11 studied. Tumoral germinoma cells were present in CSF in five patients of ten, in one of them before radiologic confirmation. Pertinent endocrine evaluation and search for tumoral markers was of great value in systematic follow-up of patients with central diabetes insipidus, and could lead to early diagnosis and treatment of the tumor.

The records of 12 patients with a diagnosis of pinealoma or ectopic pinealoma irradiated at Montefiore Hospital and Medical Center between 1961 and 1971 were reviewed. Diagnosis was made clinically and radiographically. Histology is available in three cases. Eight cases were treated with small fields (4 X 4 to 6 X 8 cm), two cases with intermediate, and two with whole brain fields. Doses between 5000 and 6000 rads were given by Cobalt-60 or 33 MeV x-rays. Eight patients (66%) showed no evidence of active disease from 4 to 14 years after irradiation. Of eight patients treated with small fields, seven are free of disease (88%). Of the remainder, 50% are free of disease. One patient subsequently developed cerebrospinal spread. The challenge to the radiotherapist is to identify the more aggressive or extensive lesion with the ultimate aim of achieving cure with the most limited treatment.

Our current policy for the treatment of pineal tumours is presented, based upon our experience of 139 cases of pineal tumours in a period of 44 years from 1941 through 1984. First of all, it should be emphasized that germinomas are extremely radiosensitive and are treated successfully by radiotherapy alone. Accordingly, the treatment of choice for germinomas would be radiotherapy without surgery, if the diagnosis could be made confidently by the modern armamentarium of investigations, including CT, cerebrospinal fluid cytology and serum level of tumour markers, such as alphafetoprotein and human chorionic gonadotropin. When the diagnosis of germinoma is suspected although not straightforward, CT is reexamined after a trial of diagnostic radiation of 20 Gy. If the tumour size has not been reduced at all, it is unlikely that the tumour is a germinoma and surgery should be considered for removal as well as establishment of the histological diagnosis.

Real-time ultrasound was used to assist localization and surgical removal of ten cases of tumours of pineal region and third ventricle. Two illustrative cases are reported in which intraoperative ultrasound greatly facilitates the surgical procedure.

Sixteen patients of the pediatric age group (mean age 6.9 years) harboring a tumorous, cystic or vascular mass lesion of the pineal region were evaluated for clinical, neuroradiological and microsurgical aspects. The heterogenous histological pattern of the lesions reflected various clinical signs and neuroradiological features. Computer assisted tomography and magnetic resonance imaging were the main neurodiagnostic tools, although angiography was an important complementary investigation in selected cases. Microsurgical approach and removal of the tumorous and cystic processes proved to be the rational treatment. Additional treatment such as chemotherapy or radiotherapy was performed in malignant processes upon a precise histological evaluation of the tumor specimen.

A case is described of suprasellar tumor with diabetes insipidus, diminishing visual acuity, inferior bitemporal quadrantanopia, and hypopituitarism in a 19-year-old man. Computerized tomographic scan of the brain after contrast injection demonstrated periventricular enhancement. Diagnosis of germinoma was made on the basis of clinical and radiographic information and a biopsy that revealed malignant cells. The tumor responded well to radiation therapy. Diagnostic difficulties were created by systemic manifestations unrelated to endocrinopathy. Pertinent literature is reviewed.

The authors describe a patient with symptoms of anorexia nervosa who was initially treated on a psychiatric ward but was later found to have a hypothalamic tumor. They recommend repeated neurological and endocrine evaluations for patients with anorexia nervosa who are unresponsive or only partially responsive to psychiatric treatment. Initially negative medical evaluations, obvious psychopathology, and even some response to treatment may all prove insufficient to rule out organic disease.

Magnetic resonance (MR) imaging characteristics of pineal germinomas are described in seven patients imaged with MR and computed tomography (CT). In patients with symptoms of an enlarging process in the quadrigeminal plate cistern, MR imaging was as sensitive as CT scanning in detecting the mass. MR imaging did not detect a normal-sized, calcified neoplastic gland. Germinoma, germinoma with embryonal cell carcinoma elements, and pineoblastoma demonstrated different MR signal characteristics. Although direct coronal and sagittal MR images were useful in defining the relationship of the tumor to the posterior third ventricle, Sylvian aqueduct, and vein of Galen, the ease, rapidity, and sensitivity of CT scanning suggest that CT should remain the modality of choice for initial evaluation and screening of the pineal region, especially in the younger pediatric population, in whom detection of calcification may provide the only clue of an abnormality.

The respective role of surgical ablation versus palliative methods (i.e. shunting followed by irradiation) in the treatment of tumors of pineal region is still questionnable and must take into account the variability of their pathology. Thus, knowledge of tumoral type in each case is the first step to therapeutic discussion. The stereotactic methods allow a faithful biopsy, easy to interpret and without major risks.

Follow-up procedures in children with brain tumors are designed to confirm tumor response to initial therapy, identify recurrence or progression at the time of potential secondary treatment, and establish the patterns of failure. Serial use of neurologic examinations and computerized tomography scans are outlined for all tumor types. Tumors capable of subarachnoid metastasis (medulloblastoma, ependymomas, pineal/suprasellar germ cell tumors, and pineoblastoma) require periodic assessment of the neuraxis by cerebrospinal fluid (CSF) cytology and myelography to establish the frequency of subclinical meningeal seeding in study settings. Serial analysis of specific biochemical markers for medulloblastoma (CSF polyamines) and germ cell tumors (serum and CSF alpha-fetoprotein and beta-human chorionic gonadotropin) are suggested. Serial evoked potentials in brain stem gliomas are recommended.

Pineal region brain tumors occur in and around the pineal gland, a small cone-shaped structure in the posterior portion of the diencephalon. Pineal tumors can be divided into four basic categories: 1) germ cell tumors, 2) pineal tumors, 3) supporting cell tumors, and 4) cysts, or non-malignant conditions. Germinomas, one type of germ cell tumors, are the most common type of pineal region tumor. In overall incidence, pineal region tumors are relatively uncommon; however, because these tumors are mainly diagnosed in childhood and young adulthood, the incidence in that age group has been reported as high as nine percent. Pathophysiology and signs and symptoms are discussed as well as medical treatment and nursing care. The case study is a presentation of a 22-year-old male with a pineal region tumor.

Preoperative CT and MRI studies of 24 patients with space-occupying lesions of the pineal region were analysed. MRI demonstrated all 24 lesions, whereas CT could detect only 20 out of 24. MRI was superior to CT in assessment of size, localisation and relation to adjacent structures. No significant difference was found between CT and MRI in preoperative prediction of tumour histology.

A series of 34 pinealomas and intracranial germinomas in childhood (age less than 20), from two tumor registries, is analyzed. Male to female ratio was 2:1. Median age was 12 years. Visual complaints and endocrine disturbances were the most common presenting symptoms. Papilledema, visual field deficits, and oculomotor abnormalities were the principal findings at diagnosis. Cumulative probability of survival (CPS) of the entire series at one year was 0.65, at two years 0.54, and at five years 0.54. Best survival was with treatment by operation and radiation: CPS at one year was 0.82, at two years 0.82, and at five years still 0.82. Survival was similar with pineal region tumors and with suprasellar tumors. Most long-term survivors, particularly with suprasellar germinomas, had permanent hormonal deficits requiring replacement therapy.

The semeiology of 72 tumors of the pineal area seen between 1949 and 1982 is analyzed. Manifestations, which are polymorphic and often misleading at onset, subsequently become manifold. Though a given syndrome may result from the mingling of several factors it is possible to establish a correlation between clinical findings and anatomical lesions. Conversely, histo-clinical correlations, which are requisite to institute specific therapy, are still as difficult to ascertain. Stereotaxic biopsy is therefore needed before planning therapy.

Germ-cell tumors arising in the neuraxis have similar histopathological characteristics to those germ-cell tumors occurring in the gonads and other midline body sites. As a rare cause of intracranial neoplasm, germ-cell tumors affect predominantly the diencephalon and in particular the pineal region and suprasellar structures. Clinical features are due to local infiltration of these areas by tumor and hydrocephalus. Germinomas or mixed-cell tumors with a germinoma component make up the majority of these tumors and are very responsive to irradiation and chemotherapy. Early clinical recognition of a diencephalic tumor and determination of the specific tumor-cell type may result in curative treatment by surgery, irradiation and chemotherapy.

Sixty-one children with pineal region tumours were managed at the Hospital for Sick Children during the period 1950-1982. Histologic verification of these tumours was possible in forty-six children. Although germ cell tumours were the commonest form, germinomas only made up one third of the pineal region tumours. Since 1967, an aggressive approach has been adopted in the management of pineal region tumours at the Hospital for Sick Children with thirty-four patients having direct surgery on their pineal region mass out of a total of forty-one patients with such tumours. The operative mortality for pineal tumour surgery has steadily fallen so that the operative mortality between 1975 and 1982 was 4.3% and there have been no operative deaths since 1977 among the thirteen patients with pineal region tumours who have been operatively treated. Pineal tumours are operable lesions which should be removed if they are benign. Histologic confirmation should be obtained if they are malignant in order to provide for rational management of these lesions.

We report a case of a presumed pineal germinoma in a 28-year-old man. Although the pineal body, the presumed primary lesion, was small, there were two disseminated tumors, one in the posterior fossa and the other in the left parietal region. The initial symptom was cerebellar ataxia. These two disseminated tumors had attachments to the inferior surface of the cerebellar tentorium and the dura mater of the parietal convexity, respectively, and they were fed by external carotid artery branches, like meningiomas. Neither angiography nor magnetic resonance imaging could provide the differential diagnosis between germinoma and meningioma. Computed tomographic scanning revealed slight enlargement of the pineal body suggestive of a germinoma.

Primary malignant intracranial germ cell tumors are rare lesions responsible for only 0.5% of all central nervous system (CNS) malignancy. With stereotactic localization these lesions can be safely biopsied, and histologic confirmation will affect the ultimate prognosis. This report is a multi-institutional retrospective analysis of 33 patients diagnosed with a primary CNS germ cell tumor. Tumors in 14 patients (42%) were histologically confirmed (13 germinoma and one embryonal cell carcinoma); 19 patients were treated with a presumptive diagnosis. All patients were irradiated with a dose range of 3950 cGy to 6000 cGy to the primary lesions. Eight patients received craniospinal irradiation, and 25 patients were locally treated. The 5-year actuarial survival for the entire population was 64%. The survival rate in patients with histologic confirmation was 79% versus 53% in the unbiopsied population. Radiation doses greater than 5000 cGy, radiotherapy volume, and age were prognostic factors in determining survival.

Tumours of the pineal region are rare. Clinical and CT aspects, and results of the treatment of 12 cases observed in a 5 years period (1979-1984) are described. The authors consider the supracerebellar infratentorial approach, formerly described by Krause in 1926 and reintroduced by Stein in 1971, the best for the surgical management of these tumours.