Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01185 (vasopressin)
 23,126 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The now nine years old girl with growth retardation, started to be ill with otitis and then diabetes insipidus of central origin at 1974. A treatment with lysin-vasopressin is prescribed. The PNEG in May 1976 shows a little, pea like, suspicious, supra-sellar nodule who is not surgically explored since they was no ophtalmologic symptoms and because a normal CT scan. Corticoid and thyroid substitutive therapy is added until September 1977 when the general status becomes impaired and vomiting starts. Also because some visual loss, a new neuroradiological study is performed showing a supra-sellar tumour and a fourth ventricle mass. The CT scan asserts the double intracranial expansive process and a posterior fossa craniotomy is done with subtotal resection of a vermian tumour and Torkildsen drainage. The histology is : Immature Dysembryoma (seminoma type) or germinoma. The follow-up was good under hormonal care. X Rays Therapy over the posterior fossa, the suprasellar region, the brain and the spinal channel was instaured. Four months later, the CT scan shows normal sized ventricles and no tumour mass at all. This case gives the authors opportunity for comments and to study the concerned literature.
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PMID:[Germinoma (ectopic pinealoma) with double location : supra-sellar and the cerebellum without pineal tumour (author's transl)]. 49 37

The clinical and neuro-endocrinological aspects of 17 suprasellar germinoma patients treated between 1972-1991 are reported. Surgical extirpation was not initially attempted, but all patients received irradiation with or without a biopsy. Sixteen of those have led useful lives with appropriate hormonal replacement therapy during a mean follow up of 8 years. Seven tumours at diagnosis were less than 2 cm in diameter (type 1), nine tumours more than 2 cm (type 2), and one double midline tumour was of unknown size. All 7 type 1 patients required 1-deamino-8-D-arginine-vasopressin (DDAVP) to control diabetes insipidus (DI), but only 2 of the 9 type 2 patients have needed DDAVP since completion of the treatment. Patients with smaller tumours required more DDAVP following tumour disappearance, than those with larger tumours. The prognostic indicators for the post-treatment course of DI and retarded growth appeared to be the tumour size and the age at diagnosis. We also emphasize the absence of metastasis in unoperated cases and the use of tumour markers as a diagnostic criterion which obviates surgical acquisition of tissue to make the diagnosis. Possible reasons are discussed and the literature reviewed.
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PMID:Suprasellar germinomas; relationship between tumour size and diabetes insipidus. 156 35

We report an 8-year-old boy with a primary intrasellar mixed germ-cell tumor who underwent the trans-sphenoidal approach for tumor removal. Initially he suffered from diabetes insipidus. Precocious puberty and left abducens nerve palsy were also observed. Elevation of serum testosterone, beta-human chorionic gonadotropin (HCG), and alpha-fetoprotein (AFP) were found on admission. The histological study revealed mixed cellular types of tumor including germinoma, choriocarcinoma, embryonic cell carcinoma, and teratoma. Postoperative radiation to a total of 5000 cGy was performed. Adjuvant chemotherapy was administered before and after radiation. The boy was disease-free during a 6-month follow-up period. Follow-up magnetic resonance imaging showed no presence of tumor. The signs of precocious puberty disappeared, and the diabetes insipidus was easily controlled. The abducens nerve regained normal function. Serum HCG, AFP, and testosterone levels all returned to normal. Serum antidiuretic hormone increased to reach the lower limit of the normal range.
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PMID:Primary intrasellar mixed germ-cell tumor with precocious puberty and diabetes insipidus. 908 1

We report nine consecutive children and adolescents [five females and four males; aged 2 yr 8 months (m) to 18 yr 1 m] studied over the last 5 yr with idiopathic central diabetes insipidus. In addition to vasopressin deficiency, anterior pituitary hormone deficiencies were detected, either on evaluation at presentation or during follow-up studies over the following 3 yr. Four patients had an increased concentration of plasma PRL. One patient had multiple pituitary hormone deficiencies at diagnosis, and two others developed the same by 21 m of follow-up. Brain magnestic resonance imaging scans, performed at presentation, were originally interpreted as normal in four of nine patients, except for absence of the bright posterior pituitary signal; after retrospective review, two of nine were considered normal. All of the brain magnetic resonance imaging (MRI) scans showed positive findings by 14 m of follow-up. The first abnormal finding in all patients was isolated pituitary stalk thickening. Evaluation of cerebrospinal fluid (CSF) for hCG was positive in three of eight evaluated patients; the three positive CSF values were found at presentation and 3 and 9 m after presentation. All eight patients assessed were negative for CSF alpha-fetoprotein and cytology, and no patient had serum tumor markers. Transsphenoidal biopsy of the lesion in seven of nine patients showed a germinoma in six patients and inflammatory cells in one. The six patients with documented germinoma comprise 31% of the intracranial germinomas diagnosed in this age group at the University of California-San Francisco during the last 5 yr. The patient with mononuclear inflammatory cells on biopsy along with one other patient have had spontaneous resolution of their stalk thickening. So-called "idiopathic" central diabetes insipidus warrants close follow-up to determine the etiology, especially if anterior pituitary hormone deficiencies are detected. Normal brain MRI scans or scans that show isolated pituitary stalk thickening merit follow-up with serial contrast enhanced brain MRI for the early detection of an evolving occult hypothalamic-stalk lesion. CSF evaluation is recommended at presentation because elevated CSF hCG may precede MRI abnormalities.
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PMID:Idiopathic hypothalamic diabetes insipidus, pituitary stalk thickening, and the occult intracranial germinoma in children and adolescents. 914 16

Because intracranial germinomas are readily curable with radiation and chemotherapy or radiation therapy alone, the role of radical surgery has become debatable. This study assesses the optimum degree of surgical resection for intracranial germinomas. Twenty-nine patients who underwent surgery for germinoma were retrospectively analyzed (male/female ratio 27:2, median age 18 years). Among these 29 patients there were 10 solitary pineal, seven solitary neurohypophyseal/hypothalamic, and 12 multifocal or disseminated tumors. Biopsy samples were obtained in 16 patients (stereotactically in eight, transsphenoidally in four, and via frontotemporal craniotomy in four). Partial resection was attained in five patients (via a frontotemporal approach in three and occipitotranstentorially in two). Gross-total resection was achieved via an occipitotranstentorial route in eight patients with pineal masses. After surgery, 10 patients were treated with radiotherapy alone, and 19 received radiation and chemotherapy; complete remission was achieved in all 29 patients. The overall tumor-free survival rate was 100% at a median follow-up period of 42 months. There was no significant difference in outcome related to the extent of surgical resection. Postoperative neurological improvement was seen in only two patients, whereas transient postoperative complications, mainly upgaze palsy, were observed in six. One patient experienced a slight hemiparesis, bringing the surgical morbidity rate to 3% (one of 29). It is concluded that radical resection of intracranial germinomas offers no benefit over biopsy. The primary goal of surgery should be to obtain a sufficient volume of tumor tissue for histological examination. If there is strong evidence of germinoma on radiological studies, biopsy samples should be obtained. When a perioperative histological diagnosis of pure germinoma is made during craniotomy, no risk should be taken in continuing the resection.
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PMID:Management of primary intracranial germinomas: diagnostic surgery or radical resection? 925 91

Melatonin deficiency after a pinealectomy has been investigated in animals; however, in humans, this status can be assessed solely by investigating patients with a tumor originating in the pineal gland. This study analyzes secretion of melatonin and pituitary hormones in 14 patients with germinoma originating in the pineal or the hypothalamic-neurohypophyseal region. Thirteen patients had been successfully treated prior to this study. One patient was included in this study before the initiation of treatments. Plasma sampling was performed every 2 hr for 24 hr and melatonin concentrations were measured by radioimmunoassay. Melatonin secretion was nearly absent in the patients with pineal germinoma regardless of treatment option, even in the patient who had been untreated. In contrast, melatonin secretion and its circadian rhythms were not affected in patients with a hypothalamo-neurohypophyseal germinoma. The circadian rhythms of growth hormone and adrenocorticotropic hormone were not dysregulated in patients with the melatonin deficiency. We conclude that germinoma cells originating the pineal gland impair the production of melatonin by pineocytes and consequently induce a permanent melatonin deficiency in those patients. Since melatonin exerts multiple physiological functions, once a clinical concept of "melatonin deficiency syndrome" is established, melatonin replacement therapy could be investigated in patients who have a pineal germinoma or who have undergone a neurosurgical pinealectomy.
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PMID:Twenty-four hour rhythm of melatonin in patients with a history of pineal and/or hypothalamo-neurohypophyseal germinoma. 974 84

Since neurohypophyseal germinomas occur at the pituitary and hypothalamic axis in children and adolescents, the endocrinopathy is one of the common and critical QOL determinants. We carried out a retrospective study on the outcome of endocrine function in patients with neurohypophyseal germinoma, in order to improve or preserve pituitary function after treatment. Sixteen patients (7 men and 9 women), aged 6 to 26 years were admitted and followed up for 95.3 (14-197) months. DI was noted in 12 patients in pretreatment and 16 in posttreatment regardless of tumor size. We carried out the replacement of GH in all 8 patients, presenting the symptoms under 15 years of age. Gonadal or gonadotropic, thyroid and adrenal hormones were replaced in 9, 12 and 15 patients, respectively. Patients with large tumor compressing chiasm or hypothalamus needed hormonal replacement such as gonadal or gonadotropic and thyroid hormones more frequently (<0.01) than those with small one. In addition, two patients with a small tumor at the pituitary stalk and the 3rd ventricle floor showed the improvement of secretion pattern in gonadotropins and ACTH after chemotherapy, although they later needed radiation therapy to control the tumor. Based on our study and review of literature, the endocrinological studies before and after treatment demonstrated that pituitary dysfunction present before treatment persisted or worsened even after tumor remission, except for patients with small and localized ones. The poor endocrine results is considered to be largely radiation-related. Chemotherapy alone seems to be insufficient to obtain complete response (CR). To avoid radiation related pituitary injury, combination of 24 Gy or less dosage of radiation and appropriate chemotherapy is essential. The earlier diagnosis by repeatedly using neuroimaging and serum and CSF tumor markers and earlier initiation of treatment, before irreversible pituitary-hypothalamic damage occurs, contributes to improvement of the outcome of pituitary functions in patients with neurohypophyseal germinomas.
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PMID:Long-term outcome of endocrine function in patients with neurohypophyseal germinomas. 1081 Dec 97

Germ cell tumours of the central nervous system (CNS) include many subtypes whose response to treatment varies, even though the symptoms and radiological appearances are similar. Five-year survival rates are 96% for germinomas, 100% for mature teratomas, 67% for immature teratomas and 69% for immature teratomas mixed with germinomas; for beta-HCG secreting germinomas the rate is only 38%. Patients with choriocarcinoma, embryonal carcinoma, or yolk sac tumour have the lowest survival rates; patients with germinoma or mature teratoma have longer survival rates. Although a wider resection is associated with a higher rate of survival for patients with non-germinomatous germ cell (NGGC) tumours, to date an aggressive surgical approach has been advocated only for pineal region tumours, but not for hypothalamic/neurohypophyseal tumours. Beside the delayed injury induced by radiotherapy, the late injury induced by chemotherapy is becoming increasingly evident. Cisplatin is considered an indispensable drug, but it may cause renal damage, ototoxicity, peripheral neuropathy and sterility, while etoposide is associated with an excess frequency of second neoplasms. Taking into account all of the published literature, the following therapeutic options are suggested: in pure germinoma tumours (GT) radiotherapy alone will usually ensure adequate control of the disease, and the long-term sequelae may be limited by reducing the dose delivered, as was proposed for germ cell testicular tumours, to 30 Gy to limited fields plus 25-30 Gy to the spinal axis if there is disseminated disease. In cases of recurrence, which should be uncommon, patients may be rescued with both radiotherapy and chemotherapy. In NGGC tumours, the prognosis is more unfavourable and there is often dissemination to the spine at diagnosis; however, the tumour's high chemosensitivity suggests neoadjuvant treatment chemotherapy with cisplatin and etoposide for three cycles followed by consolidation radiotherapy with 40 Gy to the limited fields plus 30 Gy to the spinal axis if disseminated. In our opinion, a higher dose of radiotherapy in cases in which chemotherapy does not achieve a radiological complete remission is not advisable, because very often the residual radiological abnormality does not represent biologically active tumour but differentiated forms such as mature teratoma. The challenge for 2000 is to both cure these patients, and avoid the late and permanent sequelae of radiation and/or chemotherapy that may subsequently impair quality of life.
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PMID:The treatment of cranial germ cell tumours. 1091 79

The application of gallium-67 (67Ga) citrate has not been reported for the detection of an intracranial germinoma. The purpose of this report is to further evaluate the effectiveness of 67Ga scintigraphy in neurohypophyseal germinomas. 67Ga studies in five male patients with histologically verified intracranial pure germinomas were evaluated. Two germinomas were located in the neurohypophysis, two in the pineal region, and one in both the neurohypophysis and pineal region. The control group included 36 patients with histologically verified pituitary macro-adenomas. 67Ga study at the time of original diagnosis showed an abnormal accumulation in two neurohypophyseal germinomas, and negative accumulation in the other germinomas. In the two patients with neurohypophyseal germinomas, 67Ga study showed an abnormal accumulation in the intracranial tumour region in accordance with magnetic resonance (MR) findings. After postoperative irradiation or chemoradiotherapy, MR imaging and 67Ga studies revealed the complete disappearance of this tumour and no metastatic spread. In one of these two patients, a whole-body 67Ga study demonstrated multiple bone metastases one year later, and the reduction of these metastatic regions after chemotherapy. 67Ga study disclosed negative accumulation in all 36 pituitary macroadenomas. Although 67Ga uptake by neurohypophyseal germinomas may not be specific for this condition, this approach may provide some clues for diagnosing patients with neurohypophyseal germinomas.
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PMID:Usefulness of gallium-67 scintigraphy in neurohypophyseal germinoma. 1094 40

Central diabetes insipidus (DI) can be the outcome of a number of diseases that affect the hypothalamic-neurohypophyseal axis. The causes of the condition can be classified as traumatic, inflammatory, or neoplastic. Traumatic causes include postoperative sella or transection of the pituitary stalk, while infectious or inflammatory causes include meningitis, lymphocytic hypophysitis, and granulomatous inflammations such as sarcoidosis and Wegener's granulomatosis. Various neoplastic conditions such as germinoma, Langerhans cell histiocytosis, metastasis, leukemic infiltration, lymphoma, teratoma, pituitary adenoma, craniopharyngioma, Rathke cleft cyst, hypothalamic glioma, and meningioma are also causes of central DI. In affected patients, careful analysis of these MR imaging features and correlation with the clinical manifestations can allow a more specific diagnosis, which is essential for treatment.
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PMID:MR imaging of central diabetes insipidus: a pictorial essay. 1175 30


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