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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Craniopharyngioma is a rare dysontogenetic benign tumor. Patients frequently suffer from endocrine deficiencies, sleep disturbances, and obesity due to pituitary and hypothalamic lesions. A self-assessment daytime sleepiness questionnaire (German version of the Epworth Sleepiness Scale) was used to evaluate 79 patients with childhood craniopharyngioma. Because hypothalamic lesions may explain daytime sleepiness in craniopharyngioma patients, salivary melatonin and cortisol concentrations were examined in obese and nonobese craniopharyngioma patients (n = 79), patients with hypothalamic pilocytic astrocytoma (n = 19), and control subjects (n = 30). Using a general linear model procedure analyzing the influence of body mass index (BMI) and tumor diagnosis on diurnal salivary melatonin, we found that morning salivary melatonin levels were related to BMI (by F test, P = 0.004) and tumor diagnosis (by F test, P = 0.032). Also for nighttime salivary melatonin levels significant relations with BMI (by F test, P < 0.001) and tumor diagnosis (by F test, P = 0.025) were detectable. Melatonin concentrations in saliva of craniopharyngioma patients collected at night or in the morning showed a negative correlation (night: Spearman's rho = -0.42; P = 0.001; morning: Spearman's rho = -0.31; P = 0.020) with the patient's Epworth Sleepiness Scale score. Severely obese craniopharyngioma patients and severely obese hypothalamic tumor patients had similar patterns of melatonin secretion. Differences in terms of diurnal salivary cortisol concentrations were not detectable when patient groups and controls were compared. We speculate that hypothalamic lesions might be responsible for both obesity and daytime sleepiness. As decreased nocturnal melatonin levels were associated with increased daytime sleepiness, BMI, and hypothalamic tumor diagnosis, further studies on the beneficial effects of melatonin substitution on daytime sleepiness and weight control in these patients are warranted.
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PMID:Melatonin secretion and increased daytime sleepiness in childhood craniopharyngioma patients. 1216 49

A family with coexistence of hypotension, recurrent respiratory infection, motor tics, obsessive compulsive disorder, major depressive disorder, early onset osteoporosis, low body mass index, bulimia nervosa and healthy aging with longevity is described. The family members had hyposexual behavior, less tendency for spirituality, had no insomnia but a tendency towards increased somnolence, no addictive behaviour, had more bonding and affectionate behavior and were less creative with an average IQ. There was no vascular thrombosis, systemic neoplasm and neuronal degeneration in the index family. All members of the family were left hemispheric dominant. The level of serum digoxin, HMG CoA reductase activity and dolichol was found to be decreased in all with a corresponding increase in RBC Na(+)-K(+) ATPase activity and serum ubiquinone magnesium level. There was increase in tyrosine catabolites and a reduction in tryptophan catabolites in serum. Total and individual glycosaminoglycan fractions, carbohydrate residues of glycoproteins, glycolipids, activity of GAG degrading enzymes and glycohydrolases were decreased in serum. The concentration of RBC membrane total GAG and carbohydrate residues of glycoproteins increased while cholesterol : phospholipid ratio of membrane decreased. The activity of free radical scavenging enzymes were increased while the concentration of free radicals decreased significantly. The same biochemical patterns were observed in left hemispheric dominance as opposed to right hemispheric dominance. The significance of these findings in the pathogenesis of these disorders is discussed.
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PMID:Familial hypodigoxinemic membrane Na(+)-K(+) ATPase upregulatory syndrome - relation between digoxin status and cerebral dominance. 1239 67

A 76-year-old woman suffered from somnolence while fasting for almost 2 years. Fasting plasma glucose (FPG) (40 mg/dl) and the immunoreactive insulin (IRI) level (8.8 microU/ml) were not compatible with Fajan's ratio or Turner's ratio observed in typical insulinoma. The secretin test showed no response to insulin secretion, suggesting the presence of insulinoma. Abdominal dynamic computed tomography (CT) revealed a 12-mm hypervascular lesion in the head of the pancreas. A selective arterial calcium infusion test (SACI) was performed, during which IRI in the hepatic venous blood was measured following selective intraarterial calcium infusion. An increase in IRI levels in the gastroduodenal and superior mesenteric arteries suggested the presence of a functional insulinoma in the head of the pancreas. Enucleation of the tumor improved FPG and IRI levels to 138 mg/dl and 3.8 microU/ml, respectively. After surgery, a secretin test showed a 5-fold increase in IRI levels, suggesting normal beta cell function. This case illustrates the value of the secretin test for the diagnosis of insulinoma and for the postoperative assessment of beta cell function. It further illustrates the value of the SACI for localizing an insulinoma.
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PMID:An insulinoma for which secretin test and selective arterial calcium injection test were useful. 1241 6

An open-label phase I trial of thalidomide (TL) in 20 patients with neurofibromatosis 1 (NF1) treated symptomatic plexiform neurofibroma (PNF). TL was well tolerated in doses up to 200 mg/d. Adverse reactions included transient somnolence in four, evanescent rash in two, and reversible mild peripheral neuropathy in two patients. Four patients showed less than 25% reduction in the tumor size. TL may have a role in the treatment of PNF and should be explored in a larger controlled study, possibly using higher doses of TL.
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PMID:Phase I study of thalidomide for the treatment of plexiform neurofibroma in neurofibromatosis 1. 1252 36

We report a 65-year-old Japanese lady who suffered from progressive loss of vision and visual field defect. She was well until her 61 years of the age in November of 1999, when she was found to have bitemporal hemianopsia. A small enhancing mass lesion was found in the chiasmatic region. She was treated with steroid and she noted marked improvement in her visual field defects. In August of 2000, she noted disturbance of gait. Cranial MRI revealed a mass in the right midbrain extending into the hypothalamic and thalamic regions. She was again treated with steroid with marked improvement. However, in November of 2001, she started to show somnolence and diabetes insipidus. She was treated with steroid, nasal desmopressin, and insulin for her steroid induced diabetes mellitus. Cranial CT scan showed a large enhancing lesion involving the entire midbrain, hypothalamus, and the thalamic regions. She developed respiratory arrest on July 15, 2001 and was pronounced dead. She was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had a primary malignant lymphoma of the brain. Clinical diagnosis in the early stage of her disease was neurosarcoidosis. Post-mortem examination revealed a mass continuously involving the pons, midbrain, hypothalamus, thalamus, and the putamen. The optic chiasm was enlarged. By histologic examination, the mass consisted of dense medium sized tumor cells. Immunohistologic observation revealed that the tumor cells were B-cell type malignant lymphoma. No tumor cells were found in the systemic organs.
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PMID:[A 65-year-old woman with progressive loss of vision and visual field defects]. 1264 5

We report a rare case of pilocytic astrocytoma and diencephalic syndrome occurring together in a 30-year-old woman with neurofibromatosis type 1 (NF-1). Diencephalic syndrome included emaciation and somnolence. Gadolinium-enhanced magnetic resonance imaging revealed a brain tumor in the optic chiasmal-hypothalamic region, which invaded the thalamus, brain stem and cerebellum. A biopsy specimen from the chiasmal tumor was compatible with pilocytic astrocytoma. Although she received radiation therapy, she died 22 months after the onset of the disease.
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PMID:[Pilocytic astrocytoma and diencephalic syndrome in an adult with neurofibromatosis type 1]. 1450 50

We report a case of new-onset seizures and narcolepsy in a previously healthy 40-year-old man. He developed severe daytime somnolence and cataplexy over the course of a few months. Brain MRI was normal, and polysomnography with multiple sleep latency testing confirmed a diagnosis of narcolepsy. His HLA haplotype is DQB1*0602 and cerebrospinal fluid analysis showed no detectable hypocretin. Approximately 18 months later, he developed complex partial seizures. Further MRI showed a progressively enlarging lesion involving the left frontotemporal and insular areas. Pathology from a partial resection was consistent with Rasmussen's syndrome. Evaluation for tumor, infectious, and paraneoplastic etiologies was negative. There was no further progression of the residual lesion on serial MRI. Although the pathophysiologic bases of narcolepsy and Rasmussen's syndrome are unknown, they may have an autoimmune basis. This unique case of both disorders in a single patient suggests the possibility of a common underlying disease process.
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PMID:Rasmussen's syndrome and new-onset narcolepsy, cataplexy, and epilepsy in an adult. 1469 22

Rhabdoid tumors of the brain are rare with an invariable dismal prognosis despite treatment. This is the case of a 3 year old boy who presented lethargy, somnolence, nausea, vomiting, and headaches one week prior to hospitalization. A posterior fossa tumor with hydrocephalus was noted on a head computed tomography (CT) scan. A ventriculoperitoneal shunt was placed with subsequent gross total tumor resection. Pathology findings were those of a rhabdoid tumor. The histopathology, immunohistochemistry and ultrastructure of this unusual pediatric cerebral neoplasia is discussed.
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PMID:Rhabdoid tumor: an unusual pediatric brain tumor. 1476 7

Childhood craniopharyngiomas are histologically benign tumours arising from remnants of Rathke's pouch in the hypothalamic-pituitary region. The two common treatment approaches are primary total resection or limited resection followed by radiotherapy. To study the outcome after a primary surgical approach, we followed 25 consecutive patients (10 females, 15 males) under 16 years of age who were treated in a single institution with a management policy of radical tumour excision (mean age at diagnosis 9 years 2 months, SD 4 years 3 months; range 2 years 9 months to 15 years 11 months). Mean follow-up after primary surgery was 11 years 3 months (SD 7 years 7 months). Tumour control, and neurological, endocrine, and hypothalamic complications and their impact on health-related quality of life were assessed (medical follow-up, semi-structured interview, and questionnaires). Results of tumour control were generally good, however, local failure was observed in 6 of 25 patients, and severe late-treatment complications decreased quality of life for many long-time survivors. Endocrine deficiency occurred in 24/25, visual complications in 16/24, neurological complications in 8/24, obesity in 14/23, increased daytime sleepiness in 6/21, and significant school problems in 10/20. Patients with craniopharyngioma rated their health-related quality of life as considerably lower than healthy controls; the domains of social and emotional functioning were particularly affected. Parents' ratings were considerably lower than those of the patients. Poor functional outcome was associated with large tumours infiltrating or displacing the hypothalamus, the occurrence of hydrocephalus, and young age at diagnosis, but also with multiple operations due to tumour recurrence. Alternative treatment strategies should be considered, especially in very young patients with large tumours.
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PMID:Outcome of craniopharyngioma in children: long-term complications and quality of life. 1507 99

To characterize a new, one-stop multidisciplinary palliative care (MD) clinic which offers standardized multidisciplinary assessment, specific care recommendations, patient and family education, and on-site counseling, we retrospectively compared the assessments of 138 consecutive patients with advanced cancer referred to the MD clinic and 77 patients referred to a traditional pain and symptom management (PSM) clinic. The two groups were similar in tumor type, demographics, and symptom distress. The MD clinic team (physicians; nurses; pharmacists; physical, speech, and occupational therapists; social workers; chaplains; nutritionists; psychiatric nurse practitioner) delivered 1,066 non-physician recommendations (median 4 per patient, range 0-37). The PSM clinic team made no non-physician recommendations, but referred 14 patients to other medical specialists. In 80 (58%) MD-clinic patients with follow-up 9 days (median) after assessment, significant improvement was observed in pain, nausea, depression, anxiety, sleep, dyspnea, and well-being, but not in fatigue, anorexia, or drowsiness. In 83 patients interviewed after the MD clinic, satisfaction was rated as excellent (5 out of 5) in 86-97% of seven areas. Assessment at an MD clinic results in a high number of patient care recommendations, improved symptoms, and high levels of patient satisfaction.
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PMID:Impact of a half-day multidisciplinary symptom control and palliative care outpatient clinic in a comprehensive cancer center on recommendations, symptom intensity, and patient satisfaction: a retrospective descriptive study. 1516 46


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