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Query: UMLS:C0018681 (headache)
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Pituitary macroadenomas are rare in children and adolescents, and when encountered are usually hormone secreting. Symptomatic pituitary non-secreting macroadenoma apoplexy in an adolescent is rare and potentially life-threatening. A 15 year-old patient is described, hospitalized due to headache, fever and photophobia 4 days prior to admission. A meningeal syndrome was postulated, based on clinical examination and cerebrospinal fluid testing. However, clinical examination and hormone testing revealed partial failure of the anterior pituitary. Computed tomography of the brain demonstrated a space-occupying lesion of the pituitary. Magnetic nuclear resonance imaging suggested the presence of a pituitary macroadenoma. Hypophysectomy was performed. Histological examination revealed an extensive infarction of a pituitary adenoma. Hormonal substitution with thyroxine and corticosteroids was administered. This report emphasizes that pituitary non-secreting macroadenoma apoplexy may rarely be the cause of headache and fever in an adolescent, thus causing difficulties in differential diagnosis from acute meningitis.
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PMID:Pituitary non-secreting macroadenoma apoplexy in an adolescent. patient report and review of the literature. 1250 64

Clinical and pathological findings in 100 cases of acromegaly were reviewed. Great individual variation in manifestations of the disease was apparent. The chronic course was evidenced by 30 patients presenting symptoms unrelated to acromegaly. Headache, visual disturbances, arthritis and elevated blood pressure were common. The incidence of diabetes mellitus, thyroid and adrenal dysfunction was similar to that reported in other series. Gonadal disturbances were frequent. Two patients had galactorrhea and two had hyperparathyroidism. Radiologically, pituitary fossae were normal in 24. Pituitary histology in 29 cases revealed four eosinophilic adenomas, but most were mixed eosinophilic-chromophobic. Fifty-two patients received radiation therapy. Rhinorrhea and optic nerve fibrosis were seen in this group. Twenty-four patients were operated upon, with no operative deaths since 1945 and no morbidity since 1952. Visual failure was the clearest indication for surgery. Treatment will be difficult to evaluate until an index of growth hormone secretion is generally available.
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PMID:Acromegaly: a review of 100 cases. 1394 86

A thirty-eight year-old lady with a history of bilateral adrenalectomy for Cushing's disease seven years previously, presented with sudden onset of severe headache, nausea, vomiting and loss of consciousness. She was somnolent and confused. She had neck stiffness, sixth nerve palsy and mydriasis on the left side. Computerized tomography (CT) and magnetic resonance imaging (MRI) studies revealed a non-homogeneous, grade IV D pituitary mass lesion associated with hemorrhage in the chiasmatic, interhemispheric, cerebellopontine, perimesencephalic cisterns and a hematoma within the frontal lobe. Angiography showed only bilateral elevation of horizontal segments of the anterior cerebral arteries. According to this angiographic evidence, it was presumed that the subarachnoid hemorrhage and the intracerebral hematoma were linked to pituitary adenoma apoplexy. ACTH level was 450 pg/ml. The hemorrhagic lesion with suprasellar extension was totally removed by left pterional craniotomy. Histological examination revealed a necrotic, ACTH-secreting pituitary adenoma. Even though apoplexy is a well known complication of pituitary adenomas, to our knowledge subarachnoid hemorrhage and intracerebral hematoma as a result of pituitary apoplexy in the context of Nelson's syndrome has not previously been reported.
Pituitary 2002
PMID:An unusual presentation of Nelson's syndrome with apoplexy and subarachnoid hemorrhage. 1455 76

Octreotide is a somatostatin analog that inhibits growth hormone release showing higher potency than natural somatostatin so it has proved to be effective in acromegaly treatment. The objective of present study was to establish the effects of octreotide LAR (long acting release) preparation in patients with active acromegaly. The following parameters were assessed: clinical response, safety of medication, GH and IGF-1 serum concentrations and pituitary tumor size. Eleven patients (6 men and 5 women) range 41.4 years old with diagnosis of active acromegaly were included. Octreotide was administered at 0.1 mg subcutaneusly dose three times daily for four weeks to test the drug tolerability. Afterwards patients received octreotide LAR 20 mg intramuscularly separated by 28 days periods with an option to continue for 8 months. Basal average GH serum concentrations was 27.6 ng/mL. After 6 months treatment reduction to 5.03 +/- 5.38 ng/mL in 9 patients (p < 0.001) was observed. Basal IGF-1 average serum concentration was 889.55 +/- 167.29 ng/mL with a reduction value to 483.00 +/- 239.71 ng/mL in 9 of 11 patients after 6 months treatment (p < 0.005). The drug was well tolerated with few adverse effects Diarrhea, flatulence and steatorrhea were observed during the administration of subcutaneous octreotide in 18.2% of patients. Two patients had symptomatic biliary lithiasis that was successfully removed by surgery. Clinical symptoms improved and some of them dissapeared such as headaches and sweatings. Tumor shrinkage was observed in 66.7% of cases. Monthly injections of 20 mg of octreotide LAR were effective to reduce GH and IGF-1 levels in patients with active acromegaly accompanied by improvement of clinical symptoms and significant tumor size reduction.
Pituitary 2003
PMID:Improvement of acromegaly after octreotide LAR treatment. 1467 21

We described a 39-yr-old asian female who was initially diagnosed with prolactinoma and presented with increase nervousness and weight loss. Laboratory evaluation revealed an inappropriately normal TSH level with elevated free T4, total T3, alpha-subunit and prolactin level. The alpha-subunit/TSH molar ratio was 4. MRI showed a macroadenoma extending to the suprasellar cistern. Treatment was begun with propylthiouracil and bromocriptine. After 5 months of therapy, she became pregnant. At 27 weeks of gestation, she developed headache and decreased visual acuity in her left eye. MRI showed a slightly increase in tumor size compressing the optic chaiasm. Transphenoid macroadenectomy was performed with immediate relief of the visual field abnormality. At 39 weeks gestation a baby with no malformations was delivered. This is the second case report of TSH secreting pituitary adenoma which was exarcerbated during pregnancy. In contrast to the first case, our case was managed with both surgical and medical approach. The judicious use of both medical and surgical therapy can result in a successful outcome to mother and fetus in a patient with TSH secreting pituitary adenoma.
Pituitary 2003 Sep
PMID:Successful management of a pregnant woman with a TSH secreting pituitary adenoma with surgical and medical therapy. 1470 21

We describe two young men with cystic pituitary enlargement on magnetic resonance imaging (MRI) causing hypopituitarism. The first patient presented acutely unwell with headache and vomiting associated with anterior and posterior pituitary dysfunction. The second patient presented with hypopituitarism after a long history of hypogonadism. In both cases yellow/brown fluid was found at surgery and histological examination revealed inflammatory infiltrate with foamy histiocytes, lymphocytes and multinucleated giant cells containing cholesterol clefts. Full recovery of pituitary function occurred after surgery in the first but not the second patient. The first case is the first documented case of xanthomatous hypophysitis with recovery of pituitary function following surgery. The cases differed in duration of disease, as indicated by the long history of symptoms, the histological finding of marked fibrosis and the lack of recovery of pituitary function in the second. Xanthomatous pituitary lesions categorized in the literature as xanthomatous hypophysitis, xanthogranulomatous hypophysitis and xanthogranuloma of the sellar region have overlapping histological features. Our two cases revealed histological features that do not fit completely into any of the categories but share features of all three. These findings suggest that the various xanthomatous lesions of the sellar region may be a spectrum of a common inflammatory process rather than distinct pathological entities.
Pituitary 2003
PMID:Xanthomatous pituitary lesions: a report of two cases and review of the literature. 1497 40

Cushing's disease caused by a microadenoma located near the pituitary stalk is infrequent and spontaneous remission caused by necrosis of a corticotropinoma in such location has not been reported. A 42-year-old woman with ACTH-dependent Cushing's syndrome presented on magnetic resonance imaging (MRI) a 3-mm microadenoma attached to the pituitary stalk. Treatment with ketoconazole normalized urinary free cortisol (UFC) from 433.0 to 66.0 microg/day, although it failed to reduce elevated serum androgen levels (DHEAS 4770 ng/ml). After one year, treatment was stopped and UFC rose again to 936.0 microg/day but one month later the patient presented acute headache and signs of steroid withdrawal syndrome. Endocrine evaluation showed glucocorticoid and androgen deficiency (UFC 5.0 microg/day; DHEAS < 300 ng/ml); control MRI revealed disappearance of the microadenoma. Cushingoid signs subsided and steroid replacement was initiated, proving still necessary over two years after the episode. Infarction or hemorrhage of a corticotrope adenoma could be a probable underlying mechanism although its precipitating factor is unclear. Ketoconazole withdrawal, through abrupt increase in cortisol production and/or the interruption of a hypothetical inhibitory action on cell replication followed by tumor growth and compromise of vascular supply, may be considered as possible triggering factors. To the best of our knowledge, this is the first report of spontaneous remission of Cushing's disease caused by presumed infarction of a microadenoma, unusually located in the superior rim of the pituitary, attached to the stalk.
Pituitary 2004
PMID:Spontaneous remission of Cushing's disease after disappearance of a microadenoma attached to the pituitary stalk. 1563 98

Although intrinsic pituitary lesions are the most common cause of hypopituitarism, suprasellar masses can produce similar symptoms. The differential diagnosis of a suprasellar mass includes cystic lesions, tumors, granulomatous disease, and infection. The etiology is not always obvious, and despite extensive work-up, may remain elusive. A 28-year-old Mexican man presented with complaints of headache and weakness for two weeks duration. He became increasingly lethargic and an MRI revealed a two centimeter suprasellar mass. Testing of the hypothalamic-pituitary axis suggested panhypopituitarism. He was prescribed treatment with hydrocortisone, DDAVP, and levothyroxine. Open craniotomy and biopsy of the hypothalamus revealed marked inflammation with plasma cells, histiocytes, and small lymphocytes. Light microscopy revealed macrophage-contained leishmania-like organisms although results were not immediately available. Pathological data was consistent with acute infection by Trypanasoma cruzi. Despite supportive efforts, the patient expired two months after presentation. This case illustrates the difficulty of diagnosing and the potential rapid mortality of a suprasellar mass. Because of the wide consideration of etiologies, a tissue diagnosis is needed. However, as this case illustrates, a definitive tissue diagnosis is not always possible, even following biopsy during open craniotomy.
Pituitary 2004
PMID:Acute chagas' disease presenting with a suprasellar mass and panhypopituitarism. 1576 61

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

Lymphocytic hypophysitis (LYH) is a pituitary disease which can cause headache, changes in visual field and pituitary dysfunction. The clinical, histopathological and morphological findings and its association with other autoimmune disorders allow LYH to be included among the autoimmune diseases. Pituitary trans-sphenoidal biopsy is thought to be the diagnostic gold standard for LYH, even if some morphological findings on hypothalamic-pituitary magnetic resonance imaging (MRI) can suggest the occurrence of this disease. Despite the fact that organ-specific antibodies are good markers of many autoimmune endocrine diseases, the pathogenetic and diagnostic roles of anti-pituitary antibodies (APAs) in LYH are still under discussion. In fact, several methods have been used to detect APAs, but the conflicting results from different methods have impaired the clinical relevance of these antibodies. Recently, APAs have been detected by an immunofluorescence method in patients with selective idiopathic hypopituitarism (particularly in those with growth-hormone deficiency) and in adults with autoimmune endocrine diseases. The results suggest that only when they are present at high titres may they be considered a good marker of pituitary involvement, and in particular of growth-hormone-producing cells.
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PMID:Pituitary antibodies and lymphocytic hypophysitis. 1582 23


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