IR and/or SRS provided results comparable with proton beam or heavy particle therapy in our series of cranial base chordomas probably because the radiation field must have covered the target of the tumour volume sufficiently, and reduction of gross tumour volume reduced the target size for radiotherapy. Pursuit of further effective combinations of IR and stereotactic radiotherapy (SRS, proton beam, heavy particle) after tangible resection, especially for residual and recurrent lesions, will be an acceptable framework to achieve a better therapeutic outcome for cranial base chordomas than at present.

This study describes the clinicopathologic features of 13 cases with pineal parenchymal tumors. Based on the histopathologic findings, especially the extent of atypia and pineocytic differentiation as determined by Bodian's staining, we classified these tumors into pineocytomas (4), pineocytomas with anaplasia (4) and pineoblastomas (5). All the cases with pineocytoma and pineocytoma with anaplasia were adults, and all the cases with pineoblastoma were younger children. One patient with pineocytoma died of other disease 7 months after initial treatment. One patient with pineocytoma with anaplasia died 168 months after initial treatment. The other patients with pineocytoma and pineocytoma with anaplasia survived between 9 and 179 months after surgery. However, all of the five pineoblastoma patients died within 14 months after initial treatment. The mean MIB-1 index in pineoblastomas was significantly higher than that in other types of pineal parenchymal tumors, but there were no differences between pineocytomas and pineocytomas with anaplasia with respect to the MIB-1 index. The mean MIB-1 index in neurofilament protein-immunopositive cases was significantly lower than that in immunonegative cases. With regard to the malignant potential, we emphasize that a clear distinction should be made between pineoblastomas in children and other types of pineal parenchymal tumors in adults.

Leptin is a 16 kDa protein that exerts important effects on the regulation of food intake and energy expenditure by interacting with the leptin receptor in the brain and in many other tissues. Although leptin is produced mainly by white adipose tissue, several laboratories have shown low levels of leptin production by a growing number of tissues including the anterior pituitary gland. Many studies have implicated leptin in anterior pituitary function including the observation that homozygous mutations of the leptin receptor gene led to morbid obesity, lack of pubertal development and decreased GH and TSH secretion. In addition, leptin functions as a neuroendocrine hormone and regulates many metabolic activities. Leptin also interacts with and regulates the hypothalamic-pituitary-adrenal, the hypothalamic-pituitary-thyroid and the hypothalamic-pituitary-gonadal axes. All of the anterior pituitary cell types express the leptin receptor. However, leptin has been localized in specific subtypes of anterior pituitary cells indicating cell type-specific production of leptin in the anterior pituitary. Subcellular localization of leptin indicates co-storage with secretory granules and implicates hypothalamic releasing hormones in leptin secretion from anterior pituitary hormone cells. Leptin signal transduction in the anterior pituitary has been shown to involve the janus protein-tyrosine kinase (JAK)/signal transducer and activation of transcription (STAT) as well as suppressor of cytokine signalling (SOCS). These proteins are activated by tyrosine-phosphorylation in anterior pituitary cells. The various steps in pituitary leptin signal transduction remain to be elucidated.

No abstract

IntroductionExamples of the spontaneous regression of primary intracranial germinomas can be found in the literature. We present the case of a patient with disseminated lesions of primary intracranial germinoma which synchronously shrunk following diagnostic irradiation. We will discuss whether this regression was spontaneous or radiation-induced.Case presentationA 43-year-old Japanese woman presented to our hospital complaining of memory problems over a period of one year and blurred vision over a period of three months. Following magnetic resonance imaging, she was found to have a massive lesion in the third ventricle and small lesions in the pineal region, fourth ventricle, and in the anterior horn of the left lateral ventricle. Prior to an open biopsy to confirm the pathology of the lesions, she underwent a single cranial computed tomography scan and a single cranial digital subtraction angiography for a transcranial biopsy. Fourteen days after the first magnetic resonance image - 12 and eight days after the computed tomography scan and digital subtraction angiography, respectively - a pre-operative magnetic resonance image was taken, which showed a notable synchronous shrinkage of the third ventricle tumor, as well as shrinkage of the lesions in the pineal region and in the fourth ventricle. She did not undergo steroid administration until after a biopsy that confirmed the pathological diagnosis of pure germinoma. She then underwent whole craniospinal irradiation and went into a complete remission.ConclusionsIn our case report, we state that diagnostic radiation can induce the regression of germinomas; this is the most reasonable explanation for the synchronous multiple regression observed in this case of germinoma. Clinicians should keep this non-spontaneous regression in mind and monitor germinoma lesions with minimal exposure to diagnostic radiation before diagnostic confirmation, and also before radiation treatment with or without chemotherapy begins.

A 13-year-old Japanese girl suddenly presented with polydipsia and polyuria on February 7, 2006. She was suspected of having diabetes insipidus by a local pediatrician and was referred to us for further examination and treatment. She was a well-developed and well-nourished girl. She had normal puberty development and regular menstruation, and had never experienced visual disturbance. The maximum urine osmolality remained at only 133 mOsm/kg in a 4-hour water deprivation test, and her plasma osmolality and arginine vasopressin were 291 mOsm/kg and 0.80 pg/ ml, respectively. After a desmopressin test her urine osmolality had increased to 408 mOsm/kg and urine volume decreased. She was diagnosed as having CDI Abstract. In pediatric and adolescent patients, the most common causes for a thickened pituitary stalk with central diabetes insipidus are germ cell tumors, lymphocytic infundibuloneurohypophysitis (LIN), and Langerhans cell histiocytosis (LCH). we describe here a 13-year-old girl who had an abrupt onset of polyuria and polydipsia. Magnetic resonance imaging of the brain revealed thickening of the pituitary stalk, and loss of the physiological hyperintense signal of the posterior pituitary gland. Based on a histopathology, she was diagnosed as having LCH. Another LCH lesion was not detected. The prognoses for LCH patients with single-system and single-site are generally good so we decided on only simple observation. The lesion spontaneously regressed 3 months later, resembling a typical self-limiting course of LIN. In conclusion, the present case suggests that 1) radiological differential diagnosis between LIN and LCH is so difficult that histological confirmation is crucial for correct diagnosis, 2) some past cases of histologically-unconfirmed LIN can include LCH, 3) solitary neurohypophyseal LCH can shrink spontaneously up to near remission level.

Preoperative evaluation of pituitary macroadenomas using 3DAC PROPELLER MR imaging on a 3-tesla system is likely to be a powerful noninvasive method of detecting cavernous sinus invasion, which can potentially dominate the therapeutic strategy for these lesions.

Leptin is an adipocyte-derived cytokine with many functions including signaling the status of body energy stores through activation of the leptin receptor (OBR). Activation of the long form of OB-R (OB-Rb) results in JAK2 phosphorylation, activation of STATs, and subsequent gene expression. Activated STAT3 induces SOCS-3 expression in some cell types, which in turn down-regulates the JAK/STAT pathway. Although both leptin and OB-R are expressed in pituitary cells, the mechanism of signal transduction and its regulation in this organ has not been studied extensively. In these experiments we show that leptin reduces proliferation in a human pituitary cell line (HP75) and also increased apoptosis in these cells. Leptin also increased SOCS-3 mRNA and protein expression and tyrosine-phosphorylation in the HP75 human pituitary cell line. These findings suggest that SOCS-3 plays an important role in the inhibition of proximal leptin signal transduction in the anterior pituitary.