Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0001430 (adenoma)
 21,222 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Serial hormonal studies were carried out in a girl with a growth hormone-secreting pituitary adenoma and hyperprolactinemia diagnosed at 21 months of age, the youngest verified case of acromegaly. The child had progressive macrocephaly, noted at 6 months of age, which preceded the rapid acceleration of linear growth by nearly 1 year. At 21 months of age, the girl's head circumference measured 55 cm (+5.5 SD) and her height was 97.6 cm (+4.4 SD). Preoperative serum growth hormone level was 135 ng/mL, somatomedin C was 1,540 ng/mL (normal for bone age 18 to 97 ng/mL), and prolactin was 370 ng/mL (normal less than 20 ng/mL). Following total resection of a large adenoma, immunohistochemical staining of the tumor showed growth hormone but not prolactin. With longitudinal monitoring of the child for 2 years postoperatively, persistently low growth hormone levels were demonstrated and normal growth velocity (6 cm/yr). Peak serum growth hormone levels ranged from 2.8 to 4.1 ng/mL after stimulation tests with insulin, arginine, and L-dopa. Maximum sleep-entrained growth hormone level was 3.4 ng/mL. At the same time, serum somatomedin C levels measured serially were normal (29 to 111 ng/mL), whereas simultaneous prolactin levels were moderately increased (30 to 147 ng/mL). The data support the hypothesis that hyperprolactinemia may have contributed to stimulating somatomedin C and sustaining the normal growth rate in this child.
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PMID:Acromegaly in an infant. 265 29

Acromegaly is caused by GH-secreting pituitary adenomas and, in rare cases, by ectopic production of GRH with resultant hypersecretion of GH. Important systemic manifestations include acral enlargement, swelling, disfigurement, glucose intolerance and diabetes, hypertension, nerve entrapment, arthropathy, and cardiac disease. Tumor-related major manifestations are visual impairment, oculomotor paralysis, and hypopituitarism. Morbidity is substantial, and mortality is increased. Diagnosis should be made as early as possible by measuring plasma GH after an oral glucose load and plasma somatomedin C levels. Assessment of a pituitary lesion is best made by CT scanning in the coronal plane. Therapy is mandatory and consists of surgical removal of the pituitary adenoma (usually by the transsphenoidal route) or of the ectopic source of GRH (carcinoids or islet cell tumors). Adjunctive radiation and/or drug therapy is often necessary if complete surgical ablation of the adenoma is not possible. Radiation therapy can be administered as conventional supervoltage x-ray treatment or in the form of heavy particle beams. Drugs effective in partially lowering GH levels are bromocriptine and (not yet released) somatostatin analogues. Long-term follow-up of treated patients is important to guard against recurrence, progression, or development of hypopituitarism.
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PMID:Acromegaly. 331 99

A 21-yr-old woman with Turner's syndrome presented with signs and symptoms of acromegaly. The serum growth hormone (GH) (95+/-9.4 ng/ml; mean+/-SEM) and somatomedin C (11 U/ml) levels were elevated, and an increase in GH levels after glucose instead of normal suppression, increase after thyrotropin-releasing hormone (TRH) administration instead of no change, and decrease after dopamine administration instead of stimulation were observed. The pituitary fossa volume was greater than normal (1,440 mm(3)) and the presence of a pituitary tumor was assumed. After tissue removal at transsphenoidal surgery, histological study revealed somatotroph hyperplasia rather than a discrete adenoma. Postoperatively, she remained clinically acromegalic and continued to show increased GH and somatomedin levels. A search was made for ectopic source of a growth hormone-releasing factor (GRF). Computer tomographic scan revealed a 5-cm Diam tumor in the tail of the pancreas. Following removal of this tumor, serum GH fell from 70 to 3 ng/ml over 2 h, and remained low for the subsequent 5 mo. Serum somatomedin C levels fell from 7.2 to normal by 6 wk postoperatively. There were no longer paradoxical GH responses to glucose, TRH, and dopamine. Both the medium that held the tumor cells at surgery and extracts of the tumor contained a peptide with GRF activity. The GRF contained in the tumor extract coeluted on Sephadex G-50 chromatography with rat hypothalamic GH-releasing activity. Stimulation of GH from rat somatotrophs in vitro was achieved at the nanomolar range, using the tumor extract. The patient's course demonstrates the importance of careful interpretation of pituitary histology. Elevated serum GH and somatomedin C levels in a patient with an enlarged sella turcica and the characteristic responses seen in acromegaly to TRH, dopamine, and glucose do not occur exclusively in patients with discrete pituitary tumors and acromegaly. This condition can also occur with somatotroph hyperplasia and then revert to normal after removal of the GRF source. Thus, in patients with acromegaly a consideration of ectopic GRF secretion should be made, and therefore, careful pituitary histology is mandatory. Consideration for chest and abdominal computer tomographic scans before pituitary surgery, in spite of their low yield, may be justified.
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PMID:Somatotroph hyperplasia. Successful treatment of acromegaly by removal of a pancreatic islet tumor secreting a growth hormone-releasing factor. 629 May 40

We encountered a 33-year-old female patient with a pituitary growth hormone (GH)-secreting macroadenoma. The patient was treated with somatostatin analogue (Octreotide) in combination with bromocriptine for 2 months before a transsphenoidal adenomectomy was carried out. Octreotide (300-800 micrograms/day) in combination with bromocriptine was effective in reducing the size of the adenoma by 36%, but produced only a marginal decrease in serum GH. After the operation, bromocriptine alone (15 mg/day) did not lower the level of GH which was produced by residual adenoma tissue. When octreotide (200 micrograms/day) was resumed along with the bromocriptine one year after the operation, it effectively lowered serum GH for 6 months. Thereafter, octreotide therapy became ineffective with a concomitant rise in serum GH and somatomedin C, which was not accompanied by an increase in tumor size. This was a rare case of acromegaly that showed desensitization to octreotide after long-term treatment.
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PMID:Desensitization to somatostatin analogue (Octreotide) observed in a patient with acromegaly. 762 75

A man had left-sided atypical clusterlike headache for 9 years before he developed symptoms and signs consistent with acromegaly. Preoperative evaluation revealed raised levels of somatomedin C and growth hormone. An MR indicated a left-sided intrasellar mass measuring 8 x 7.5 x 10 mm. He underwent surgery and microscopy confirmed the diagnosis of a benign hypophyseal adenoma. Postoperatively, the acromegalic features regressed, and for the last 4 years the patient has been completely free from headache attacks. On pharmacological testing of the pupillary response to 1% and 5% phenylephrine and 2% tyramine solutions, there was no convincing evidence of persistent sympathetic dysfunction on the earlier symptomatic side.
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PMID:Clusterlike headache in a patient with a pituitary adenoma. With a review of the literature. 898 93

We report a successfully treated case of invasive TSH-secreting pituitary adenoma associated with an unruptured internal carotid artery aneurysm by two-stage operation and gamma-knife radiosurgery. A 64-year-old woman was admitted to our department with a 3-year history of general fatigue and 1-year history of anxiety, palpitation and hyperhydrosis. Endocrinological examination revealed hyperthyroidism with elevated TSH, GH and somatomedin C. Magnetic resonance images demonstrated a tumor in the sella turcica which extended into the left cavernous sinus, furthermore, indicated aneurysm-like flow void at the ventral part of the left internal carotid artery. The aneurysm was confirmed by conventional angiography. Neck clipping of the aneurysm was performed through pterional approach as the first operation. One month later, at the second operation, the pituitary adenoma except for cavernous sinus portion was resected via the transsphenoidal approach. Immunohistological examination revealed positive for TSH and GH. Gamma-knife radiosurgery with a central dose of 33.3 Gy and peripheral dose of 17 Gy was carried out for residual tumor at the cavernous sinus under both MRI and CT guidance. Posttreatment course was uneventful with normalization of thyroid function at 16 months after gamma-knife. Two-stage operation and gamma-knife radiosurgery is effective for TSH-secreting adenoma extending into the cavernous sinus associated with an unruptured aneurysm.
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PMID:[A case of TSH-secreting pituitary adenoma associated with an unruptured aneurysm: successful treatment by two-stage operation and gamma-knife]. 1055 92

A pituitary acidophil adenoma in a domestic shorthaired cat with diabetes mellitus and elevated serum somatomedin C level is described. Growth hormone production by the neoplasm was confirmed by an appropriate reaction using an immunoperoxidase technic.
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PMID:Growth Hormone-producing Pituitary Adenoma, Elevated Serum Somatomedin C Concentration and Diabetes Mellitus in a Cat. 1742 32