Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Craniopharyngiomas are the third most common pediatric brain tumor and most common pediatric suprasellar tumor. Contemporary treatment of craniopharyngiomas uses limited surgery and radiation in an effort to minimize morbidity, but the long-term health status of patients treated in this fashion has not been well described. The purpose of this study was to analyze the health status of long-term survivors of pediatric craniopharyngioma treated primarily with radiation and conservative surgical resection. Medical records of all long-term survivors of craniopharyngioma treated at St. Jude Children's Research Hospital and then transferred to the long-term follow-up clinic were reviewed. The initial cohort comprised 55 patients. Of these, 51 (93%) were alive at the time of this analysis. The median age at diagnosis was 7.1 years (range, 1.2-17.6 years), and 29 (57%) were male. At the time of analysis, the median survival was 7.6 years (range, 5.0-21.3 years). Diagnosis and treatment included surgical biopsy, resection (n = 50), and radiation therapy (n=48). Only 1 patient received chemotherapy. Polyendocrinopathy was the most common morbidity, with hypothyroidism (96%), adrenocorticotropic hormone deficiency (84%), and diabetes insipidus (53%) occurring most frequently. Half of the patients were hypogonadal, and 33 (65%) were overweight or obese. The most common neurologic problems included shunt dependence (37%), seizures (28%), and headaches (39%). Psychological and educational deficits were also identified in a significant number of these individuals. Despite efforts to reduce morbidity in these patients, many survivors remain burdened with significant medical complications. In a small percentage of patients, complications may result in death even during extended remission of craniopharyngioma. Because of the broad spectrum or morbidities experienced, survivors of craniopharyngioma continue to benefit from multidisciplinary care.
 ...
PMID:Health status in long-term survivors of pediatric craniopharyngiomas. 2120 70

We report the case of a 31-year-old male who presented to the ER with a 1-week history of progressively worsening, throbbing, left retro-orbital headache, ptosis, and subjective worsening of short-term memory function. Initial review of systems and laboratory data were noncontributory. Non-contrasted CT demonstrated a large hyperdense mass centered in the suprasellar cistern without evidence of dissecting extra-axial hemorrhage. Though the initial appearance mimicked a basilar tip aneurysm or another primary extra-axial suprasellar pathology such as a hemorrhagic or proteinaceous craniopharyngioma, germinoma, or optic glioma, a second smaller, clearly intra-axial, hyperdense lesion was observed in the left periventricular forceps major white matter. Consideration for multiple cavernomas versus hypervascular metastatic disease such as renal malignancy, thyroid malignancy, or melanoma was raised. CTA confirmed normal intracranial vasculature. Subsequent MRI images showed an acutely hemorrhagic mass centered at the left paramedian hypothalamus and tuber cinereum with numerous secondary foci, demonstrating mature hemorrhagic elements and confirming the diagnosis of multiple cavernomas.
 ...
PMID:Exophytic hypothalamic cavernous malformation mimicking an extra-axial suprasellar mass. 2130 32

We present a case of a purely infrasellar craniopharyngioma that initially presented as a sphenoid sinus mass. Craniopharyngiomas are usually located within the sella. Purely infrasellar craniopharyngiomas have only rarely been reported in the literature. A 25-year-old woman presented with 6-month history of progressive headaches. Initial neuroimaging revealed the presence of a sphenoid sinus mass. Initially, she underwent an endoscopic biopsy of the mass by our ENT service. Pathology was consistent with craniopharyngioma and she was referred to neurosurgery for further surgical management. She then underwent an endoscopic transsphenoidal approach for complete resection of the purely extracranial, infrasellar craniopharyngioma. The Rathke pouch arises from the roof of the primitive mouth and grows toward the brain at the fourth week of gestation. Normally, it loses its attachment with the stomadeum completely by the eighth week of gestation. The craniopharyngeal canal (CPC) extends from the floor of the sella to the vomer and may rarely give rise to ectopic craniopharyngiomas. This case shows that such ectopic tumors may arise anywhere along the CPC. Endoscopic endonasal approach provides an excellent route for the resection of these tumors.
 ...
PMID:Extracranial infrasellar ectopic craniopharyngioma: a case report and review of the literature. 2177 8

Craniopharyngioma accounts for 2.5-4 percent of all intracranial tumors. The tumor is more observed in the chiasmatic region in adults and the intraventricular subtype is rare. We report an intraventricular craniopharyngioma in a 22-year-old woman presented with chronic headache. Magnetic Resonance Imaging showed hyperintense large mass on T(1)-weighted images and hypointense mass on T(2)-weighted images in third ventricle with pressure effect on both lateral ventricles and foramen of Monro. The diagnosis of craniopharyngioma was confirmed through histopathological examination of the resected tumor after surgery. After a follow-up period of nine months, neither tumor recurrence nor regrowth occurred. The early diagnosis of this relatively frequent tumor would help to prevent related sequelae.
 ...
PMID:Intrinsic third ventricular craniopharyngioma: A case report. 2181 61

A 9-year-old previously healthy girl presented with 3 weeks of intermittent emesis and headache to a community emergency department, where she had rapid decompensation due to increased intracranial pressure. Head computed tomography revealed a calcified suprasellar mass consistent with a craniopharyngioma. Despite medical and surgical intervention, the patient had progression of herniation with global cerebral infarction, and care was withdrawn. Although craniopharyngiomas are typically thought to be benign, slow-growing intracranial tumors, this case emphasizes the need for an expeditious diagnostic evaluation when symptoms that may be referable to intracranial hypertension are evident. Craniopharyngiomas and emergency management of intracranial hypertension are reviewed.
 ...
PMID:Diagnosis of a craniopharyngioma after acute brainstem herniation in an emergency department. 2182 87

The objective of this study was to determine the epidemiology of primary tumors of the central nervous system (CNS) in pediatric patients from a Brazilian oncology institute. We retrospectively analyzed 741 charts (415 males and 326 females) of patients under 21 years of age who were diagnosed with a CNS tumor. The analysis included patients from 1989 to 2009 and was performed using the World Health Organization criteria. We evaluated the distribution of age, sex, topography, clinical symptoms, symptom intervals, and classification of the tumors. Patients with clinical/radiologic diagnoses were included. Seven hundred forty-one patients with tumors in the CNS were reviewed, and 83% of the patients presented a histologic diagnosis. Males (56%) were more prevalent than females. In children under the age of 1 year, the supratentorial compartment was the predominant region involved (62.0%). Astrocytoma was the most frequent tumor type (37.0%), followed by medulloblastoma (13.6%), craniopharyngioma (10.5%), and ependymoma (6.8%). Headaches were the most common symptom, and the symptom intervals varied from 1 to 5010 days. Approximately 4% of the patients had associated genetic syndromes. Although it was not a population study and selection bias may have occurred, this study supplies important epidemiologic data from an emerging country in which population studies are rare.
 ...
PMID:Pediatric central nervous system tumors: a single-center experience from 1989 to 2009. 2203 Nov 23

We reviewed four surgical cases of purely third ventricle craniopharyngioma, focusing on surgical outcomes and adjuvant treatments. From 2002 to 2008, we performed surgical treatments, via a transcallosal transforaminal approach, on four patients. All were males, with a median age of 42 (36-45) years. Most patients complained of headaches, while two (50%) patients presented with visual disturbances, and one (25%) presented with an endocrinological disturbance. Patients' follow-up periods ranged from 1.6 to 8.6 years. We totally removed the tumor in each of the four patients. The tumors originated in the infundibulum of the third ventricular floor. The pituitary stalk was anatomically preserved. The histopathological findings showed the adamantinomatous type of craniopharyngioma in all patients. Postoperatively, two patients who had experienced visual disturbances showed improvement, and there was no aggravation. Two patients had intact pituitary functioning, while two needed complete hormone replacement. The patients experienced no surgery-related complications. Two patients experienced recurrences 4.5 and 1.6 years later. One patient received gamma knife surgery for the recurred lesion, which controlled the lesion well. Purely third ventricle craniopharyngioma showed good visual and endocrinological outcomes after surgery. Gamma knife surgery could be of help in the event of a recurred lesion.
 ...
PMID:Operative outcomes and adjuvant treatment of purely third ventricle craniopharyngioma after a transcallosal approach. 2347 22

A 23-year-old non-diabetic woman presented to our emergency room with progressive headache. She was diagnosed with craniopharyngioma and received tumor resection. Her blood glucose level was within the normal limit before surgery, and she had no family history of diabetes. Three months after the surgery, acute hyperosmolar hyperglycemic state developed. After 4 months of follow-up, her diabetes persisted but improved with oral antidiabetic drugs. This is the third case report of diabetes developing several months after craniopharyngioma tumor resection. The possible mechanisms of cranipharyngioma or its surgery inducing diabetes mellitus are hypothalamic obesity or hypothalamic damage. The degree of hypothalamic damage before the operation is predictive of diabetes development, and blood glucose level monitoring is important for these patients.
 ...
PMID:Craniopharyngioma or its surgery induces diabetes mellitus. Case report. 2216 33

We report the clinical significance of anterior cerebral artery (ACA) notching on the optic nerve and chiasm in a 3.5-year-old girl with a craniopharyngioma and progressive blindness. She presented with a headache and vomiting, followed by binocular blindness. Magnetic resonance imaging (MRI) studies demonstrated severely distended A1 segments and ill-depicted ACAs. Surgical decompression via a right subfrontal approach was performed to reverse blindness. Postoperative MRI studies showed good ACA visualization. A second operation via a right pterional approach revealed ACA notching, which appeared as a transverse groove on the right optic nerve and chiasm. ACA notching should be considered as a possible cause of progressive visual disturbance and a potential risk of ACA infarction in a child with a craniopharyngioma.
 ...
PMID:Anterior cerebral artery notching on anterior optic pathways in a child with craniopharyngioma and progressive blindness. 2257 4

Craniopharyngiomas are biologically benign lesions (WHO Grade 1) of the sellar and suprasellar region, associated with a serious morbidity. About 50% of these tumors become clinically apparent during childhood. Clinical symptoms include headaches, chiasm syndrome, hydrocephalus, pituitary insufficiencies, and obesity. Growth arrest is a typical symptom in children. The treatment of craniopharyngiomas includes surgery as well as radiotherapy. The goal of surgery varies according to the tumor location and extension and may range from complete resection to biopsy. Surgical complications are well known and cause constant evaluation of surgical strategies. Diencephalic obesity is related to surgical manipulation of hypothalamic tissue. Therefore, a classification system for craniopharyngiomas based on preoperative MRI is suggested by the authors. Recurrences are frequent in craniopharyngiomas, even after complete or gross-total resection. Radiotherapy is therefore recommended to patients with incomplete resections. However, the ideal time for radiotherapy after surgery is under discussion. The treatment of craniopharyngiomas requires an interdisciplinary and multimodal approach. Each patient should receive an individually tailored treatment. Surgically, different approaches as well as different degrees of resection can be considered, depending on tumor location and tumor extension.
 ...
PMID:Surgical strategies in childhood craniopharyngioma. 2264 14


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>