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

Pituitary tumors cause symptoms by secreting hormones (prolactin, PRL, responsible for amenorrhea-galactorrhea in women and decreased libido in men; growth hormone, GH, responsible for acromegaly; adrenocorticotropic hormone, ACTH, responsible for Cushing's syndrome; thyroid-stimulating hormone, TSH, responsible for hyperthyroidism), depressing the secretion of hormones (hypopituitarism), or by mass-related effects (headaches, visual field abnormalities...). All patients with pituitary tumors should be evaluated for gonadal, thyroid and adrenal function as well as PRL and GH secretion. Specific stimulation and suppression tests for pituitary hormones are performed in selected situations for detecting the type of hypersecretion or the response to treatment. Imaging procedures (mainly magnetic resonance imaging, MRI, nowadays) determine the presence, size and extent of the lesion. The classification of pituitary tumors is based on the staining properties of the cell cytoplasm viewed by light microscopy and immunocytochemistry revealing the secretory pattern of the adenoma. Treatment of pituitary adenomas consists of surgery (performed in more than 99% of cases via a transphenoidal route) and radiotherapy, generally fractionated or, in selected cases, using stereotactic techniques such as gamma-knife. The availability of medical treatment (dopamine, DA, agonists, somatostatin analogs, GH-receptor antagonists...) has profoundly modified the indications of radiotherapy, drugs being now generally used as a second-line treatment, after surgery (or even as first-line treatment). Based on the results of the different treatment modalities for each type of pituitary adenoma, recommendations will be proposed. They may be summarized as follows. For treatment of GH-secreting adenomas, trans-sphenoidal surgery is the first-line therapy except when the macroadenoma is giant or if surgery is contra-indicated; postoperative radiation therapy (fractionated, or by gamma-knife) is performed for partially resected tumors or when GH levels remain elevated (eventually after a trial of somatostatin analog). Somatostatin analogs, now available in slow release form, are proposed when surgery is contra-indicated, or has failed to normalize GH levels, or in waiting for the delayed effects of radiation therapy. If the probability of surgical cure is low (e.g. in patients with very large and/or invasive tumors), then somatostatin analogs may be reasonable primary therapeutic modality provided that the tumor does not threaten vision or neurological function. Pegvisomant, the new GH-receptor antagonist, is indicated in case of resistance to somatostatin analogs. Patients with PRL-secreting microadenomas may be treated either with trans-sphenoidal surgery or medically with DA agonists. In patients with macroadenomas, even in the presence of chiasmatic syndrome, DA agonists are now proposed as primary treatment. Indeed, effects on visual disturbances are often very rapid (within a few hours or days) and tumoral shrinkage is usually very significant. For patients with ACTH-secreting adenomas, primary therapy is generally trans-sphenoidal surgery by a skilled surgeon, whether or not a microadenoma is visible on MRI. Radiotherapy is reserved for patients who are subtotally resected or remain hyper-secretory after surgery. In waiting for the effects of radiotherapy, adrenal steroidogenesis inhibitors (mitotane, ketoconazole) may be indicated. If drugs are not available or not tolerated, bilateral adrenalectomy may be proposed. For patients with clinically non functioning adenomas (generally gonadotropin-secreting adenomas on immunocytochemistry), trans-sphenoidal surgery with or without postoperative radiation therapy is performed for almost all patients whether or not they have visual consequences of their tumor. Selected patients with small, incidentally discovered microadenomas may be carefully followed without immediate therapy.
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PMID:Diagnosis and treatment of pituitary adenomas. 1576 32

Concomitant thyroid cancer with pituitary tumor is uncommon. This study reports a case of advanced papillary thyroid carcinoma with pituitary adrenocorticotropic hormone (ACTH)-secreting tumor. A 58-year-old male patient had thyroid cancer in 1991 and presented with headache caused by pituitary tumor with apoplexy in 1993. Due to hypopituitarism, the patient underwent radioactive iodide ((131)I) for detection and treatment of metastatic thyroid cancer after the use of recombinant human thyroid-stimulating hormone (rhTSH) in 2000. During follow-up for thyroid cancer, (201)thallium scan proved to be an effective tool for detecting metastatic thyroid cancer in the patient without pituitary TSH reserve. Pituitary ACTH-secreting tumor was confirmed in 2001 based on the high serum ACTH level and positive immunohistochemical stain for ACTH. The patient had no Cushingoid features. Moreover, serum ACTH levels were 337 and 232 pg/mL with normal serum cortisol and urine-free cortisol. Although the patient underwent three operations and a total of 370 mCi (131)I therapy for recurrent thyroid cancer, the cancer continued to progress. Finally, the patient died of pneumonia with septic shock 12 years after the diagnosis of thyroid cancer.
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PMID:Concurrent papillary thyroid cancer with pituitary ACTH-secreting tumor. 1747 13

Rathke's cleft cysts are rare benign lesions requiring surgical treatment when they become symptomatic. Transsphenoidal surgery is the recommended therapy due to its efficacy and safety. However, whether the optimal surgical strategy is simple drainage and biopsy or cyst wall resection remains controversial. We report a single center's experience of a series of 14 Rathke's cleft cysts treated with transsphenoidal resection of the cyst wall. Postoperatively, there was no cerebrospinal fluid rhinorrhea. The complications included permanent diabetes insipidus, hypocortisolism (including a patient with a coexisting adrenocorticotropic hormone-secreting adenoma), sinusitis and a case of meningitis and intrasellar abscess, one year post-surgery. Visual impairment and headache resolved in all cases. Pituitary dysfunction was restored only in patients with hyperprolactinemia and Cushing's disease. During the follow-up period (median 29 months) there was no recurrence requiring re-operation. According to our experience, the aggressive approach is associated with good surgical results and with low complication and recurrence rates.
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PMID:Treatment of Rathke's cleft cysts: experience at a single centre. 1936 83

The pituitary is an uncommon site for metastases. We report three cases of patients with a history of cancer (breast and lung) who presented with symptoms of headache, ophthalmoplegia, fatigue, diabetes insipidus, nausea, and vomiting. Cranial magnetic resonance imaging was performed, revealing sellar masses with infiltration of the adjacent tissues compatible with pituitary metastases in all three patients. In two of the patients, hormonal analyses were performed, which showed anterior pituitary insufficiency (thyroid-stimulating hormone and adrenocorticotropic hormone deficiency), symptoms which improved with hormone replacement therapy. Other treatments applied were surgery, radiotherapy and chemotherapy, which show no association with increased survival rates but are able to improve symptoms. The prognosis in all patients was poor. The patients developed further metastases and two died soon after diagnosis. Pituitary function study should be performed in patients with a previous neoplasm and symptoms compatible with hormonal dysfunction or local compressive symptoms.
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PMID:[Pituitary metastases in patients with prior neoplasms]. 1962 48

Two girls with central precocious puberty (CPP) associated with hypothalamic hamartoma (HH) and non classical form of congenital adrenal hyperplasia (NCAH), are reported. Case 1. The first patient, who showed at age around 4 years the onset of CPP, was submitted in view of some organic lesion to magnetic resonance (MRI) of the brain which documented the presence of HH. The remarkable acceleration of bone age (BA) advanced of 3 SD and some clinical signs of hyperandrogenism suggested the coexistence of NCAH, proved by adrenocorticotropic hormone (ACTH) test and molecular analysis. She resulted carrier of partial 21-hydroxylase deficiency. Case 2. In the second girl with CPP, aged 6.5 years, the remarkable advancement (4 SD) of bone age (BA) alerted to adrenal involvement. ACTH stimulation test and molecular analysis showed NACH due to 21-hydroxylase deficiency. Brain MRI, performed mainly for severe headache, showed the presence of HH. Yearly brain MRI to monitor HH dimensions and neurological examination with EEG, in order to exclude anomalies referable to gelastic epilepsy are advisable, in both cases. The authors' observation emphasizes the need to be careful in young patients with CPP, with fast progression of pubertal development and remarkable BA advancement. The association of CPP with HH and NCAH should be considered, performing not only MRI of the brain, but also ACTH test, beside LHRH test for the diagnosis of CPP. At the authors' knowledge this association has not been reported so far. Further observations are needed to understand if this rare combination is occasional or genetically determined.
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PMID:Precocious puberty with hypothalamic hamartoma and non classical form of congenital adrenal hyperplasia. Report of two cases. 1979 82

Pituitary metastasis occurs rarely in cancer patients and often remains undiagnosed. However, early detection and appropriate treatment can improve the patient's quality of life and possibly prolong survival. Herein, we describe the case of a 52-year-old woman with panhypopituitarism caused by metastases to the hypothalamus and pituitary from primary breast cancer. She had a 5-year history of breast cancer with metastases to the bones 1.5 years after initial diagnosis and mastectomy. She presented with severe headaches, generalized fatigue, dizziness, hypotension, difficulties with balance and coordination, polyuria, and polydipsia. Laboratory work-up revealed panhypopituitarism (central diabetes insipidus; hypothyroidism; and low prolactin, gonadotrophin, and adrenocorticotropic hormone levels), and magnetic resonance imaging confirmed the pituitary and hypothalamic involvement. She received hormone replacement therapy, radiation therapy of the sella turcica and suprasellar lesion, and chemotherapy, with significant improvement of her clinical status, but she died 15 months later.
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PMID:Panhypopituitarism due to metastases to the hypothalamus and the pituitary resulting from primary breast cancer: a case report and review of the literature. 1993 72

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.
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PMID:Health status in long-term survivors of pediatric craniopharyngiomas. 2120 70

A 57-year-old man was admitted with headache, vomiting, and bloody sputum. We diagnosed large cell lung cancer T4N2M1 (pituitary metastasis), Stage IV. When hospitalized, low values of cortisol and hyponatremia were found. A hormone stimulation test was performed, because we suspected hypopituitarism. The reaction of adrenocorticotropic hormone (ACTH) to the corticotropin-releasing hormone (CRH) loading test was good, but the reaction of serum cortisol was minimal. After corticosteroid administration, his serum sodium normalized. Limited ACTH reserve according to insufficient pituitary function was suggested as a cause of the hyponatremia. He received gamma-knife therapy, however his pituitary gland tumor did not decrease in size. Clinical symptoms such as visual field disturbance, oculomotor paresis, and visual impairment progressed, and he died about 5 months later. We report a case of hyponatremia in a patient with pituitary metastasis of lung cancer, as it is comparatively rare.
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PMID:[Hyponatremia caused by pituitary metastasis of lung cancer]. 2168 46

Pituitary apoplexy in patients with adrenocorticotropic hormone (ACTH) producing tumors is a rare occurrence. We report four patients with Cushing's disease harboring ACTH-secreting macroadenomas who presented with pituitary apoplexy. We report the endocrinologic and visual outcomes of these patients after emergent transsphenoidal surgery. A retrospective chart review was performed in 4 patients who presented with pituitary apoplexy from hemorrhage into an ACTH-secreting pituitary adenoma. The patient charts were reviewed for clinical presentation, neuroimaging findings, intraoperative surgical findings, pathologic findings, and postoperative endocrinologic and visual outcomes. All patients presented with acute headaches, nausea, vomiting, and visual loss from optic compression. MR imaging demonstrated a hemorrhagic macroadenoma that was confirmed at surgery. All patients underwent emergent transsphenoidal decompression (within 24 h of presentation). One of these underwent an additional craniotomy to resect residual tumor. Postoperatively, all patients showed significant improvement in visual acuity and visual fields with biochemical remission confirmed on laboratory testing. Significant weight loss as well as resolution of diabetes and hypertension was noted in all cases. All four patients remained in biochemical remission at their most recent follow-up visit (mean 40 months, range: 24-72 months). Excellent endocrine and visual outcomes can be achieved after emergent transsphenoidal surgery in patients with Cushing's disease presenting with pituitary apoplexy. Although the cure rates of non-apoplectic ACTH macroadenomas are generally poor, higher rates of remission can be achieved in cases of pituitary apoplexy. This may be partly due to the effects of tumor infarction.
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PMID:Pituitary tumor apoplexy in patients with Cushing's disease: endocrinologic and visual outcomes after transsphenoidal surgery. 2192 87

We reported a rare case of a dialysis patient coincident pituitary prolactinoma with calcification. A 55-year-old woman who had undergone hemodialysis for 8 years was admitted to the nephrology unit because of headache, blurred vision, and hypotension. Physical examination was normal; endocrinological examination demonstrated elevated serum levels of prolactin (> 4240 mIU/L), but other hormonal profiles, such as growth hormon, adrenocorticotropic hormone, thyroid stimulating hormone, free triiodothyronine, free thyroxine, follicle-stimulating hormone and luteinizing hormone, were absolutely or relatively lower. A cranial computed tomography (CT) suggested saddle area a high-density screenage with an anteroposterior diameter of 1.0 cm. A cerebral magnetic resonance scan confirmed the pituitary adenoma accompanied with calcification. Contrast-enhanced T1-weighted images revealed a less enhancing tumor, 14 mm wide round lesion with a high intensity signal. It enlarges the sella turcica, but the optic chiasma is not displaced. We suggest that in the differential diagnosis of any hemodialysis patient with severe headache, hypotension, and visual disturbances, this syndrome should be considered as prompt pituitary adenoma.
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PMID:One hemodialysis patient with headache, blurred vision, and hypotension induced by pituitary prolactinoma. 2293 96


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