Cases reported "Craniopharyngioma"

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1/7. Radical surgery in a neonate with craniopharyngioma. report of a case.

    ultrasonography revealed a suprasellar tumor in a fetus at 28 weeks of gestation. The male newborn, delivered 10 weeks later, was operated at the age of 17 days, and a craniopharyngioma was completely removed. Intraoperatively, inappropriate secretion of antidiuretic hormone occurred and was followed by diabetes insipidus causing imbalance of fluid and electrolytes. The tumor recurred and was totally removed 1 year later. Further development was uneventful and, at the age of 8 years, the boy is in generally good mental and physical condition except for a left-sided hemiparesis. In contrast to the poor outcome of neonatal craniopharyngioma reviewed in the literature, this case may encourage radical surgery even in the very young.
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2/7. Extensive ossification in a craniopharyngioma.

    A rare extensive ossification occurred in a suprasellar craniopharyngioma of a man who died at 21 years of age. The tumor produced headache, retarded physical and mental development, visual disturbances, and increased intracranial pressure. The neoplasm recurred after surgical and roentgenological treatments. Differentiation of multipotential mesenchymal cells or mesenchymal type cells within the tumor has been suggested as the mode of occurrence of bone in this craniopharyngioma.
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3/7. craniopharyngioma and bardet-biedl syndrome. A case report.

    BACKGROUND: bardet-biedl syndrome is a rare disorder and associated with a variety of anomalies. CASE: An 18-year-old woman was referred with primary amenorrhea. Following physical, ophthalmologic, psychiatric, hormonal and radiologic examinations, the diagnosis of both craniopharyngioma and bardet-biedl syndrome was established. CONCLUSION: Although the pathogenesis of hypogonadism in a woman with bardet-biedl syndrome remains unclear, cranial structures, especially the hypothalamus and pituitary gland, should be investigated to reveal any possible abnormalities.
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4/7. Two tumors detected by thyroid assessment in two children.

    OBJECTIVE: To describe the early detection of two tumors in two children by recognition of unusual features in initial thyroid assessments. methods: We present the clinical findings and results of laboratory studies in two children. In addition, we describe RET proto-oncogene studies in one of them. RESULTS: A 14.5-year-old boy was referred for assessment because of short stature in conjunction with lack of physical growth and development. His physical examination was remarkable for height at the 50th percentile (height age, 11.5 years), weight at the 50th percentile (weight age, 13 years), and prepubertal male status. Pertinent laboratory findings were a normal thyroid-stimulating hormone (TSH) level but low free thyroxine (FT4) index. These findings prompted an immediate magnetic resonance imaging study of the head. A parasellar tumor was detected and removed; histopathologic examination revealed that it was a craniopharyngioma. The patient requires lifelong multihormonal therapy for his panhypopituitarism and has responded with physical growth. Our second patient, a 7.5-year-old girl, was referred because of a painless left thyroid nodule of 4 months' duration. Her physical examination was remarkable for (1) upper lip swelling, (2) intermittent rash, and (3) a goiter with painless mobile left and right nodules. Normal levels of TSH and FT4, serum calcitonin of 6,192 pg/mL, and a fine-needle biopsy specimen that stained strongly for calcitonin were obtained at her first clinic visit. A total thyroidectomy was performed and confirmed the presence of medullary thyroid carcinoma. Genetic studies showed that she was positive for the RET multiple endocrine neoplasia IIB mutation. After 4 years of follow-up, the patient had serum calcitonin levels that remained low (<2.2 pg/mL). CONCLUSION: attention to thyroid physical findings and laboratory studies can promptly lead to correct diagnoses and management of some rare and life-threatening tumors in children.
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5/7. craniopharyngioma in a female adolescent presenting with symptoms of anorexia nervosa.

    BACKGROUND: Tumours of the central nervous system mostly present with neurological symptoms and signs of intracranial hypertension. Several reports of cerebral tumors in adolescents outline initial symptoms of anorexia and emaciation, leading to the diagnosis of anorexia nervosa. PATIENT: We report on a 15.5-year-old girl with a craniopharyngioma. The girl had a 2 year history of weight loss, dystrophy, no onset of puberty, and a 6 year history of headache. These symptoms had led initially to the clinical diagnoses of migraine and anorexia nervosa, since unenhanced computed tomography of the brain was normal. At presentation, physical examination showed short stature (height SDS - 3.6) and Tanner stage I. Bone age delay was about four years. Laboratory analyses showed hypopituitarism. The diagnosis of craniopharyngioma was made by repeated imaging, depicting an intrasellar and parasellar mass, which was totally removed by neurosurgery. Hormonal substitution with hGH, L-thyroxine, hydrocortisone, and estrogens led to normal physiological development and final height within upper target height. CONCLUSIONS: The reported case illustrates that the diagnosis of craniopharyngioma is often delayed due to unspecific clinical symptoms. Careful evaluation of anthropometrics, ophthalmologic, and endocrine data in patients with suspected eating disorders may give additional clues to the diagnosis of a craniopharyngioma.
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6/7. Suprasellar and intrasellar paragangliomas.

    neoplasms of the sellar region are entities with a large differential diagnosis. Although paraganglionic cells have not been demonstrated in the pituitary or adjacent structures, the existence of sellar region paragangliomas is well-documented. To elucidate, in this area the nature of these unusual tumors is relatively difficult. Clinical history, physical examination, radiographic investigation as well as intraoperative gross observation are the same as those of sellar meningioma or pituitary adenoma. immunohistochemistry, using neuroendocrine markers and electron microscopy are the two definitive diagnostic methods to differentiate among these entities. The clinical management, the possible pathogenesis of the tumor, the importance of immunohistochemistry in making the diagnosis and the clinical outcome of these patients are discussed.
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7/7. Significant medical pathology uncovered by a comprehensive male infertility evaluation.

    OBJECTIVE: To determine if there was a specific screening regimen that could identify all patients with significant medical pathology found during a comprehensive male infertility evaluation. DESIGN: A retrospective study. SETTING: Two university-based male infertility clinics. patients: Thirteen patients with male factor infertility identified with significant medical pathology. MAIN OUTCOME MEASURES: Initial presentation, history, physical examination, semen analysis, and hormone profile. RESULTS: The identification of significant medical pathology was uncovered in 13 of 1,236 patients (1.1%) presenting to a male infertility clinic. The pathology was identified with a thorough history in 4 of 13 patients (30.8%) and by a complete physical examination in 8 of 13 patients (61.5%). Semen analyses were available on 12 patients, and 1 patient was anejaculatory. Two patients were azoospermic. Of the patients with sperm present, the mean sperm concentration was 8.6 x 10(6)/mL (range, 0.8 to 27), and the mean sperm motility was 32.0% (range, 0% to 65%). In 5 patients, endocrine abnormalities were specifically related to the subsequent pathology identified. A tumor was identified in 10 patients (6 testicular tumors, 3 brain tumors, and 1 spinal cord tumor). Two patients had ejaculatory dysfunction as a result of mesonephric duct anomalies affecting the ejaculatory duct or bladder neck closure. One patient had Klinefelter's syndrome. CONCLUSIONS: There was no pathognomonic finding on history, physical examination, semen analysis, or hormone profile that identified all patients with significant medical pathology. The significant medical pathology identified was represented in all semen quality groupings, that is, azoospermia, severe oligospermia, mild oligospermia, and normospermia. We recommend a comprehensive urologic evaluation for all male partners of infertile couples with a male factor or unexplained infertility in an attempt to identify significant and potentially treatable medical pathology before engaging in a series of therapies with assisted reproductive techniques.
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