SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
A fourth case of bilateral retinoblastoma (RB) associated with pinealoma was identified among 56 patients with heritable RB. Using life-table analysis, the association was 5.6% 2 years after RB diagnosis. Retinoblastoma-pinealoma (RB-P) was uniformly fatal in this series and in 40 additional patients described in the literature. Ninety-five percent of patients with RB-P had bilateral RB, a positive family history of RB, or both. All patients with pinealoma presented with symptoms of increased intracranial pressure due to obstructive hydrocephalus. The advanced stage of the tumors at diagnosis may contribute to their uniform lethality. The incidence of a positive family history of RB in patients with pinealoma was higher than expected (62% of patients), which may be related to earlier surveillance and treatment of patients with positive family histories of RB.
Pathologic survey was performed on 43 cases of intracranial germinoma and 12 cases of pinealoma. The present study suggests that, in Japan, the incidence of teratoma groups including germinoma is remarkably higher than that in U.S. and Europe, whereas the rate of true pinealoma is lower. Using ultrastructural, enzyme-histochemical, and fluorescence-histochemical methods for a few surgical specimens, a strong similarity between intracranial germinoma (so-called "pinealoma" with a two-cell pattern) and seminoma and dysgerminoma was confirmed. The true pinealoma could be classified as pineoblastoma and pineocytoma, according to the degree of pineocyte differentiation of the tumor cells, and as "neuroblastoma-like" and "pineal-like" on the basis of the histologic architecture.
The 6 cases of brain tumor with ipsilateral cerebral hemiatrophy in which 4 cases were experienced in our institute and 2 cases were found in literature were studied in this paper. The specific character which was common in 6 cases were observed in their clinical course and the findings of clinical examinations. The histopathological study was proceeded with a autopsy case to observe the correlation between brain tumor and the ipsilateral cerebral hemiatrophy. As the result of it, we discussed the mechanism of appearance of the ipsilateral cerebral hemiatrophy due to brain tumor in the thalamic region. 1) All six cases were very young in which the age of onset was between 8 to 14 years old, 11 years 8 months old in average. 2) Their clinical course was relatively chronic. The period from onset to first admission was between 1 year 2 months to 4 years, 2 years and 1 month in average. 3) The declining of school work and hemiparesis were recognized as the primary sign of their onset. Main symptoms were hemiparesis, dementia, character and emotional change, and abnormal behavior, but sign of increased intracranial pressure was not observed. 4) The findings of carotid angiogram and pneumoence-phalogram showed ipsilateral hemiatrophy on the tumor side. 5) The brain tumor localized in the thalamic region and its surroundings which was common with all six cases. 6) Histopathological diagnosis was pinealoma, and 3 autopsy cases were ectopic pinealoma and the other 3 cases were suspected too as ectopic pinealoma. 7) In our autopsy case, ipsilateral cortical and subcortical atrophy with ectopic pinealoma was observed. As the pathological findins, degeneration and destruction of ganglion cells, demyelination in the subcortex and damage of axon were observed. These findings suggested that the ipsilateral cerebral hemiatrophy was induced by Waller's and retrograde degeneration as the result of the secondary damages of the thalamic ganglion cells and the afferent and efferent nerve fibers, due to invasive tumor into thalamic region.
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.
The author has a high incidence of pinealoma in his series of intracranial neoplasms. In order to make a diagnosis of pinealoma of the pineal region and of the chiasmal region, pneumoencephalography or ventriculography is very useful in addition to the neurological findings and the plain radiographic findings. The author emphasizes the usefulness of the Millipore filter-cell culture of the cerebrospinal fluid and of the ventriculofiberscopy for the diagnosis of pinealomas in children. The reported cases were treated by a combination of radiation and surgery.
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.
We report on a 10-year-old boy with a normal karyotype and a chromosome 13q14 deletion of the retinoblastoma gene (RB1) by fluorescence in situ hybridization (FISH). He showed subtle signs of overgrowth, including macrocephaly, hepatomegaly, and inguinal hernia. The boy also had cryptorchism and mild developmental delay. In his first months of life, variant Wiedemann-Beckwith syndrome was tentatively suspected and he was included in a careful tumor prevention program. At the age of 11 months, bifocal retinoblastoma of the left eye was diagnosed. Pinealoma was suspected at the age of 19 months and was removed by neurosurgery at the age of 29 months. At 4 years and 4 months, the deletion of the RB1 gene was suspected on clinical grounds and was diagnosed by FISH and molecular studies. At that time, he was a near-normal healthy playful kindergarten child, height 107 cm (-0.3 SD), OFC 52.5 cm (+0.8 SD), developmental age 3-3.5 years. The combination of retinoblastoma, pinealoma, and deletion of the RB1 gene diagnosed by FISH has not been reported previously. The deletion spans at least 370-420 kb in size and is predicted to include proximal and distal neighbor genes. This report may assist in establishing the clinical signs of the contiguous gene syndrome at the RB1 locus on 13q14.
Serial CT studies performed for 1 year on a female patient suffering from a pinealoma are reported. Under cobalt therapy (5000 rads) a remission of the pinealoma was achieved. A short time later, metastases developed and cobalt therapy was repeated (6000 rads). Rapid tumor remission was seen again, but rapid occurrence of probable metastases too. The question whether radionecrosis or metastases were present cannot be answered.
An ultrastructural study of four pinealomas was carried out to precise eventual specific markers. Dark and clear cells joined with zonulae adherents, extensive and pleiomorphous processes, a complex vacuolar system, and characteristic organelles (lysosome-like structures, clear and dense-core vesicles, vesicle-crowned rodlets and related structures, microtubular sheaves and centriolar derivatives, membranous whorls, fibrous bodies, microtubules, heterogeneous cytoplasmic inclusions) offered a typical pattern. No correlation could be made between the histological and ultrastructural features. The authors stress the ultrastructural similarities between the human tumor cells and the mammalian pineal cells. Pinealomas appeared as a morphological entity distinct from neuronal and astrocytic tumors.
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.
A patient with ectopic pinealoma first presented with apparent anorexia nervosa and hypernatraemic coma. A history of diabetes insipidus two months previously was not known on admission to hospital. The diabetes insipidus was unmasked by the administration of steroids. Neuroendocrinal and neuropathological aspects of the case are discussed with reference to the march of symptoms due to the growth of the tumour. Histochemical evidence is presented supporting the similarity between ectopic pinealoma and seminoma which suggests that they may more properly be referred to as atypical teratomas.
A female case of precocious puberty associated with HCG-producing ectopic pinealoma was reported. The patient, a 5-year-old girl, was referred to the hospital because of headache and choked discs. Physical examination revealed normal physical growth with breast enlargement. Endocrinological study revealed a high plasma HCG concentration of 1192 ng/ml with a normal FSH level. None of HCG, LH and FSH did respond to the LH-RH test. A partial resection of the tumor and an external X-ray irradiation relieved the symptoms and breast enlargement subsided with a remarkable decrease in the plasma HCG level. Histological examination revealed two-cell-pattern pinealoma and electron microscopic findings showed abundant secretory granules in the dark cells. HCG content in the tumor was as high was 400 ng/mg of acetone dried tumor tissue, but no FSH was detectable. Hitherto, all of the reported cases of precocious puberty associated with pineal tumors have been exclusively boys. A normal level of plasma FSH concentration with a somewhat elevated prolactin level might be a contributory factor for the development of precocial sexual development in the present case.
A 13.5-year-old girl who developed puberty due to HCG production by suprasellar ectopic pinealoma was reported. This girl appeared to be in a state of precocious puberty at the age of 5 when ectopic pinealoma was first diagnosed. Her breasts started to develop at 13 years of age in spite of hypopituitarism. Plasma LH was found to increase for several months and gave rise to suspicion of the recurrence of the tumor, which was confirmed by the detection of HCG in plasma and CSF. Precocious puberty or puberty can be a characteristic endocrinological manifestation of an HCG producing tumor not only in boys but also in girls. The measurement of plasma HCG (or LH) can be a useful tumor marker in following the clinical course of an HCG producing tumor.
Thymoma is associated with a wide variety of syndromes. However, an association with peroxidase-negative acute myeloid leukemia and pinealoma, although feasible due to the marked influence of this tumor on the lymphoid system, has not been described previously. A patient with thymic carcinoma and pinealoma who developed peroxidase-negative acute myeloid leukemia as a late event is presented in this report.
HCG-producing ectopic pinealoma of two cell pattern type was demonstrated in a 5-year old girl who presented precocious puberty and bilateral choked discs. The tumor was localized at the anterior third ventricle and suprasellar region. Endocrinological findings are as following: Plasma basal LH was markedly elevated to 306 mIU/ml and HCG was elevated to 1,192 ng/ml. Provocative test of hypophyseal function revealed low response. Plasma estrogen was not detectable. HCG content of resected tumor tissue was elevated to 400 ng/mg. FSH, however, was not detectable. Histological findings of this tumor showed atypical teratoma, so-called two cell pattern pinealoma. Electron microscopic findings revealed two types of cells, dark and clear cells. Many secreting granules were found in the dark cells. In this case, HCG in plasma, CSF and tumor tissue was remarkably elevated. In addition, plasma FSH was also elevated to 8.9 mIU/ml. Precocious puberty associated with tumor in the pineal-suprasellar region has been seen only in boys. There has been no case report of precocious puberty in girls. This case is the first female case is which HCG-producing ectopic penealoma is caused in precocious puberty.
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.
Paired twisted filaments (PTF) forming helices are described in tumor cells of three human pinealomas. Each filament was 8.11 +/- 1.55 nm wide. The maximal width of the helix was 16.62 +/- 2.62 nm. The periodicity of the constrictions was 26.63 +/- 4.49 nm. These characteristics appeared original, suggesting protein filaments possibly specific of pinealocytes. The similarities and discrepancies between PTF and Alzheimer's paired helical filaments (PHF) are discussed.