ABSTRACT Acromegaly is a devastating chronic slowly progressive disease. Its early diagnosis is a challenging issue that necessitates clinical suspicion of signs and symptoms as a first step. This report introduces an unusual early sign in the oral cavity that lead to the early diagnosis of an acromegaly case. A case of a healthy 40-year-old male patient presented with progressively growing multiple hard swellings in the upper and lower jaws. Clinical examination revealed bony hard multiple small spiky exostosis-like swellings, located at the maxillary and mandibular alveolar bones. An array of investigations revealed a 2-mm diameter pituitary tumour in MRI of sella. To the best of the author’s knowledge, this is the first report of spiky exostosis-like growths in the alveolar bone as an early sign of acromegaly. In this case, thorough examination of oral signs and symptoms was the first step for early diagnosis and hence, better prognosis for acromegaly.
Acromegaly--diagnosis

The primary causes of uncontrolled diabetes are poor life-style, infection, ischemic heart disease and inappropriate usage of oral anti-diabetic agents and insulin. Supplementary causes are stroke, acute pancreatitis and endocrine diseases. Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant syndrome characterized by primary hyperparathyroidism, pituitary neoplasia, and foregut lineage neuroendocrine tumors, and is associated with increased glucose levels. We present a case of a 69-year-old woman who had polyuria, polydipsia, weight loss and hyperglycemia over 6 months. She had hypertrophy of the face, hand, and foot, and active bleeding and large folds were observed in the stomach and duodenum upon esophagogastroduodenoscopy. She also had high levels of IGF-1 and gastrin and got the failure of growth hormone suppression after an oral glucose load (75 g). These findings suggested a diagnosis of acromegaly and gastrinoma, which was clinically diagnosed along with MEN 1. The patient improved glycemic control and symptoms after being treated with somatostatin analogues and insulin therapy over a 5-month follow-up period. Here, we report a case of MEN 1 in type 2 diabetes mellitus with a poorly controlled blood glucose level. Clinicians should consider endocrine disease in patients with poor glycemic control in diabetes.
Acromegaly ; Aged ; Blood Glucose ; Diabetes Mellitus* ; Diabetes Mellitus, Type 2 ; Diagnosis ; Duodenum ; Endocrine System Diseases ; Endoscopy, Digestive System ; Female ; Follow-Up Studies ; Foot ; Gastrinoma ; Gastrins ; Glucose ; Growth Hormone ; Hand ; Hemorrhage ; Humans ; Hyperglycemia ; Hyperparathyroidism, Primary ; Hypertrophy ; Insulin ; Insulin-Like Growth Factor I ; Multiple Endocrine Neoplasia Type 1* ; Multiple Endocrine Neoplasia* ; Myocardial Ischemia ; Neuroendocrine Tumors ; Pancreatitis ; Polydipsia ; Polyuria ; Somatostatin ; Stomach ; Stroke ; Weight Loss

A 10-year-old castrated male Korean shorthair cat weighing 4 kg was referred with signs of insulin-resistant diabetes mellitus based on clinical signs of polyuria, polydipsia, and polyphagia. Diagnosis of pituitary-dependent hyperadrenocorticism (PDH) was made based on results of an adrenocorticotropic hormone stimulation test and a dexamethasone screening test. In addition, plasma concentrations of insulin-like growth factor 1 (IGF-1) increased. Radiography, ultrasonography, and computed tomography (CT) revealed hepatomegaly, renomegaly, and adrenomegaly affecting both adrenal glands as well as multiple cysts in a generally enlarged pancreas. Magnetic resonance imaging (MRI) showed that the cat's pituitary gland was enlarged. The pituitary gland had a predominantly unilateral extension to the left. The signal intensity of the pituitary gland on precontrast T1 weighted images was hypointense compared to that of soft tissue and hyperintense compared to that of cerebrospinal fluid. On T2 weighted images, the pituitary gland was predominantly hypointense with a hyperintense rim. Contrast enhancement of the pituitary gland was not evident, and a mild degree of ring-like enhancement was seen. In addition, mild peritumoral edema was present. This is the first report of a cat with suspected double adenoma of the pituitary gland on the basis of compatible clinical signs, increased serum IGF-1 concentration, PDH, CT images, and MRI findings in diabetic cats with insulin resistance.
Acromegaly ; Adenoma* ; Adrenal Glands ; Adrenocortical Hyperfunction ; Adrenocorticotropic Hormone ; Animals ; Cats* ; Cerebrospinal Fluid ; Child ; Dexamethasone ; Diabetes Mellitus* ; Diagnosis ; Edema ; Hepatomegaly ; Humans ; Insulin Resistance ; Insulin-Like Growth Factor I ; Magnetic Resonance Imaging ; Male ; Mass Screening ; Pancreas ; Pituitary Gland* ; Plasma ; Polydipsia ; Polyuria ; Radiography ; Ultrasonography

BACKGROUND: The aim of this study was to assess the clinical differences between acromegalic patients with microadenoma and patients with macroadenoma, and to evaluate the predictive value of growth hormone (GH) levels for early detection of macroadenoma. METHODS: We performed a retrospective analysis of 215 patients diagnosed with a GH-secreting pituitary adenoma. The patients were divided into two groups: the microadenoma group and the macroadenoma group, and the clinical parameters were compared between these two groups. The most sensitive and specific GH values for predicting macroadenoma were selected using receiver operating characteristic (ROC) curves. RESULTS: Compared with the microadenoma group, the macroadenoma group had a significantly younger age, higher body mass index, higher prevalence of hyperprolactinemia and hypogonadism, and a lower proportion of positive suppression to octreotide. However, there were no significant differences in the gender or in the prevalence of diabetes between the two groups. The tumor diameter was positively correlated with all GH values during the oral glucose tolerance test (OGTT). All GH values were significantly higher in the macroadenoma group than the microadenoma group. Cut-off values for GH levels at 0, 30, 60, 90, and 120 minutes for optimal discrimination between macroadenoma and microadenoma were 5.6, 5.7, 6.3, 6.0, and 5.8 ng/mL, respectively. ROC curve analysis revealed that the GH value at 30 minutes had the highest area under the curve. CONCLUSION: The GH level of 5.7 ng/mL or higher at 30 minutes during OGTT could provide sufficient information to detect macroadenoma at the time of diagnosis.
Acromegaly* ; Body Mass Index ; Diagnosis ; Discrimination (Psychology) ; Glucose Tolerance Test ; Growth Hormone* ; Growth Hormone-Secreting Pituitary Adenoma ; Humans ; Hyperprolactinemia ; Hypogonadism ; Octreotide ; Prevalence ; Retrospective Studies ; ROC Curve

The aim of this study was to investigate the relationship between somatostatinergic tone (SST) and the size of growth hormone (GH)-producing pituitary tumors. GH levels of 29 patients with newly diagnosed acromegaly were measured using a 75-gram oral glucose tolerance test (OGTT), an insulin tolerance test (ITT), and an octreotide suppression test (OST). Differences between GH levels during the ITT and the OGTT (DeltaGH(IO)), and between the OGTT and the OST at the same time point (DeltaGH(OS)) were compared according to the size of the tumor and the response pattern to the OST. DeltaGH(IO) of macroadenomas (n=22) was non-significantly higher than those of microadenomas while DeltaGH(OS) of macroadenomas were significantly higher than those of microadenomas. According to further analyses of macroadenomas based on the response pattern to the OST, GH levels during the ITT were significantly higher in non-responders. DeltaGH(OS) showed near-significant differences between responders and non-responders. In conclusion, as the size of the pituitary tumor increases, the effect of glucose on SST appears to be attenuated. Macroadenomas that are non-responders to the OST possess a portion of GH secretion exceeding the range of regulation by SST.
Acromegaly/*diagnosis/*pathology ; Adenoma/drug therapy/*pathology ; Adult ; Aged ; Antineoplastic Agents, Hormonal/therapeutic use ; Female ; Glucose Tolerance Test ; Human Growth Hormone/*blood/secretion ; Humans ; Insulin/blood ; Insulin-Like Growth Factor I/analysis ; Male ; Middle Aged ; Octreotide/therapeutic use ; Pituitary Neoplasms/drug therapy/*pathology

The aim of this study was to assess the prevalence of diabetes and to study the effects of excess growth hormone (GH) on insulin sensitivity and beta-cell function in Korean acromegalic patients. One hundred and eighty-four acromegalic patients were analyzed to assess the prevalence of diabetes, and 52 naive acromegalic patients were enrolled in order to analyze insulin sensitivity and insulin secretion. Patients underwent a 75 g oral glucose tolerance test with measurements of GH, glucose, insulin, and C-peptide levels. The insulin sensitivity index and beta-cell function index were calculated and compared according to glucose status. Changes in the insulin sensitivity index and beta-cell function index were evaluated one to two months after surgery. Of the 184 patients, 17.4% were in the normal glucose tolerance (NGT) group, 45.1% were in the pre-diabetic group and 37.5% were in the diabetic group. The insulin sensitivity index (ISI0,120) was significantly higher and the HOMA-IR was lower in the NGT compared to the diabetic group (P = 0.001 and P = 0.037, respectively). The ISI0,120 and disposition index were significantly improved after tumor resection. Our findings suggest that both insulin sensitivity and beta-cell function are improved by tumor resection in acromegalic patients.
Acromegaly/*diagnosis/etiology/metabolism ; Adult ; Asian Continental Ancestry Group ; Blood Glucose/analysis ; C-Peptide/analysis ; Diabetes Mellitus/epidemiology ; Female ; Glucose Tolerance Test ; Human Growth Hormone/secretion ; Humans ; Insulin/blood/secretion ; *Insulin Resistance ; Insulin-Secreting Cells/cytology/*physiology ; Male ; Middle Aged ; Prediabetic State/epidemiology ; Republic of Korea

BACKGROUNDSubclinical apoplexy of pituitary functional adenoma can cause spontaneous remission of hormone hypersecretion. The typical presence of pituitary growth hormone (GH) adenoma is gigantism and/or acromegaly. We investigated the clinical characteristics of patients with spontaneous partial remission of acromegaly or gigantism due to subclinical apoplexy of GH adenoma.

METHODSSix patients with spontaneous remission of acromegaly or gigantism were enrolled. The clinical characteristics, endocrinological evaluation and imageological characteristics were retrospectively analyzed.

RESULTSIn these cases, the initial clinical presences were diabetes mellitus or hypogonadism. No abrupt headache, vomiting, visual function impairment, or conscious disturbance had ever been complained of. The base levels of GH and insulin growth factor-1 (IGF-1) were normal or higher, but nadir GH levels were all still > 1 µg/L in 75 g oral glucose tolerance test. Magnetic resonance imaging detected enlarged sella, partial empty sella and compressed pituitary. The transsphenoidal surgery was performed in 2 cases, and the other patients were conservatively managed. All the patients were in clinical remission.

CONCLUSIONSWhen the clinical presences, endocrine evaluation, biochemical examination and imageology indicate spontaneous remission of GH hypersecretion in patients with gigantism or acromegaly, the diagnosis of subclinical apoplexy of pituitary GH adenoma should be presumed. To these patients, conservative therapy may be appropriate.

Acromegaly ; diagnosis ; etiology ; Adolescent ; Adult ; Aged ; Female ; Gigantism ; diagnosis ; etiology ; Growth Hormone-Secreting Pituitary Adenoma ; complications ; Humans ; Immunohistochemistry ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pituitary Neoplasms ; complications ; Young Adult

McCune-Albright syndrome (MAS) is a rare disorder that develops from an activating mutation in the Gs gene. It is characterized by an association with Polyostotic fibrous dysplasia, and precocious puberty, Caf-au-lait pigmentation, and other endocrinopathies that result from the hyperactivity of a variety of endocrine glands. Recently we encountered a patient with MAS with fibrous dysplasia, skin pigmentation, acromegaly, hyperprolactinemia and a thyroid nodule. A 23-year-old male presented for an evaluation of a change in his facial structures. Fibrous dysplasia was diagnosed by a bone biopsy and radiographic studies. The GH level increased paradoxically after an oral glucose load. The plasma prolactin, IGF-1 and alkaline phosphatase were high. Thyroid ultrasonography revealed multiple nodules. The brain MRI demonstrated a mass in the left pituitary gland. Genetic analysis identified a change from Arg (CGT) at codon 201 to Cys (TGT).
Thyroid Diseases/etiology/genetics ; Puberty, Precocious/etiology/genetics ; Mutation ; Male ; Hyperprolactinemia/etiology/genetics ; Humans ; GTP-Binding Protein alpha Subunits, Gs/*genetics ; Fibrous Dysplasia, Polyostotic/*diagnosis/genetics/pathology ; Cafe-au-Lait Spots/etiology/genetics ; Adult ; Acromegaly/*diagnosis/etiology

BACKGROUND: It has been suggested that acromegalic patients are at increased risk of developing colonic neoplasia. We evaluated the prevalence of colonic neoplasia and the relationships of clinical parameters of acromegalic activity with presence of colonic neoplasia in Korean acromegalic patients. METHODS: In 30 patients with active acromegaly, colonoscopy was performed at the time of diagnosis. According to the endoscopic findings and histological classifications, the patients were grouped, and a number of clinical parameters of acromegalic activity were compared between those with and without colonic polyps. RESULTS: Colonic polyps were detected in 17 (56.7%) in the 30 acromegalic patients with colonoscopic examinations; 7 (23.3%) hyperplastic and 10 (33.3%) adenomatous polyps. Those with colonic polyps were significantly older (52.2+/-9.46 years vs. 42.2+/-12.97 years, p=0.02) and also had significantly longer disease duration (11.8+/-7.52 years vs. 5+/-2.20 years, p=0.004) than those without ones. In 21 patients who were more than 40 years old, significant differences were found in disease duration (12.7+/-7.54 years vs. 5.0+/-2.68 years, p=0.026) and serum IGF-1 level (1311.9+/-500.14 ng/mL vs. 715.9+/-330.58 ng/mL, p=0.015) between those with and without colonic polyps. CONCLUSION: These results suggest that screening colonoscopy should be recommended in acromegalic patients who are more than 40 year-old, have long disease duration or have high serum IGF-1 level.
Acromegaly ; Adenomatous Polyps ; Adult ; Classification ; Colon* ; Colonic Polyps* ; Colonoscopy ; Diagnosis ; Humans ; Insulin-Like Growth Factor I ; Mass Screening ; Prevalence*

OBJECTIVE: The authors present a retrospective clinical analysis of transsphenoidal surgery for acromegaly including preoperative factors determining the therapeutic outcome. METHODS: Forty-three patients(24 females, mean age 45.3 years) harboring growth hormone(GH)-secreting adenomas(13 microadenomas and 30 macroadenomas) were treated between the years 1987 and 2001. The mean duration of follow-up was 69 months. RESULTS: The average estimated duration of symptoms prior to diagnosis was 8 years. The control rate following surgery was 51%(22 out of 43 cases). Of the patients with postoperative persistent disease, 11 patients had had additional bromocriptine therapy with or without irradiation, and consequently 7 patients achieved biochemical remission. The overall remission rate of multimodality treatment was 67%. The preoperative GH value, tumor size, extrasellar extension of tumor, the number of surgeons, and the extent of the surgical removal were significant univariative predictors of outcome(p<0.05). CONCLUSION: This study suggests that surgical outcome for acromegaly could be achieved by a experienced neurosurgeon. In the group of the patients of large or invasive adenomas, with the less probability of surgical control, adjunctive medical or radiation therapy to control GH hypersecretion should be considered.
Acromegaly* ; Adenoma ; Bromocriptine ; Diagnosis ; Female ; Follow-Up Studies ; Growth Hormone ; Humans ; Microsurgery* ; Pituitary Neoplasms ; Retrospective Studies ; Treatment Outcome