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 magnetic resonance images (MRI) of 30 patients with surgically or biochemically confirmed pituitary adenomas (20 macroadenomas, 10 microadenomas) were retrospectively evaluated. Ten patients had hyperprolactinemia, another eight had acromegaly, another eight had nonfunctioning adenoma and four had Cashing disease. The examination were performed at a 1.5-T superconducting MR system using a multisection spin-echo technique with 3mm-thick sections and a 256×224 matrix. TI weighted sagittal and coronal images were obtained before and within 30 minutes after the administration of Gd-DTPA (0.1mmol/kg). Analysis of the MRI was focused on the signal intensity and enhamcement patterns of the pituitary adenoma before and after Gd-DTPA administration. Compared with endocrinologic diagnosis, macroadenoma showed heterogeneous enhacement in 55%, rim enhancement in 35% and homogeneous enhancement in 10%, Conclusively, the enhancement patterns of the pituitary adenoma did not correlate with the subtypes made according to hormone production.
Acromegaly ; Adenoma ; Diagnosis ; Gadolinium DTPA ; Humans ; Hyperprolactinemia ; Magnetic Resonance Imaging* ; Pituitary Neoplasms* ; Retrospective Studies

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

Carney Complex is an autosomal dominant syndrome characterized by multiple neoplasias, including myxomas at various sites and endocrine tumors, spotty pigmentations and schwannomas. The criteria for diagnosis of the complex is the presence of two or more of the following conditions: 1) cardiac myxoma, 2) cutaneous myxoma, 3) mammary myxoma, 4) spotty mucocutaneous pigmentation, 5) primary pigmented nodular adrenal cortical disease (Cushing's syndrome), 6) testicular tumors (sexual precocity), 7) pituitary adenoma secreting growth hormone (acromegaly or gigantism). It is thought that the genetic defects which are responsible for Carney complex maps to the short arm of chromosome 2 (2p16). There are about 200 patients with Carney complex reported in the world. We encounted a patient who had a cardiac myxoma with a family history of cardiac myxoma, acromegaly, lentigosis, testicular mass with calcification and left adrenal nodule. This patient met the criteria for the diagnosis of the complex. Therefore, we think this patient represents clinical presentation of the Carney complex and we report this case with reviews of the literatures.
Acromegaly ; Arm ; Carney Complex* ; Chromosomes, Human, Pair 2 ; Diagnosis ; Growth Hormone ; Humans ; Myxoma ; Neurilemmoma ; Pigmentation ; Pituitary Neoplasms ; Testicular Neoplasms

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*

BACKGROUND: The primary goal of therapy for acmmegaly is to reduce excess growth hormone (GH) secretion through surgical excision of pituitary adenoma and, in patients with large tumors, to debulk tumor mass and decompress adjacent structures. For the patients in whom surgery is contraindicated or has failed, radiotherapy should be considered. However, there was no analysis of the outcome folIowing the treatment of acromegly in Korea. METHODS: We retrospectively reviewed 128 patients who underwent surgical excision or radiotherapy for acromegaly and followed at our hospital between January 1980 and July 1997 to investigate the outcome of surgny and radiotherapy for acromegaly and to analyze preoperative and preradiotherapeutic parameters that predict a successful outcome of therapy. RESULTS: Pituitary adenomectomy was underwent to the 113 patients with acromegaly, then 33.6% of them showed basa1 GH levels below 5 ug/L and basal GH levels were dropped below 2.5 ug/L in 22.1% of the 113 patients. Postoperative basal GH levels were significantly correlated with preoperative tumor size(r=0.54, p<0.05) and preoperative GH levels(r=0.44, p<0.05). A successful outcome of surgery was influenced by preoperative tumor size, preoperative GH level and extrasellar extension. Multivariate analysis indicated that preoperative tumor size was an independent significant factor affecting the postoperative outcome(OR=2.19, p<0.05). After radiotherapy, the median years of decrease GH<10ug/L and <5ug/L were 3.7 and 7.8, respectively and GH levels of <5ug/L occurred in 35 percent of the patients at 5 years and in 56 percent at 10 yeats. The outcome of radiotherapy depends on the GH levels in preradiotherapy. CONCLUSION: The most reliable prognostic preoperative parameter of successful outcome of surgery was preoperative tumor size and the rate of fall in serum GH after radiotherapy is comparable to the preradiotherapy GH levels in our study, so that early diagnosis and proper treatment can improve the outcome of therapy in the patients with acromegaly. In the cases of large GH-secreting pituitary adenoma, it is required a combination of surgery and radiotherapy to achieve maximal suppresssion of GH levels before radiotherapy.
Acromegaly* ; Early Diagnosis ; Growth Hormone ; Growth Hormone-Secreting Pituitary Adenoma ; Humans ; Korea ; Multivariate Analysis ; Pituitary Neoplasms ; Radiotherapy* ; Retrospective Studies

BACKGROUND: Cardiac myxomas are rare benign tumors of the heart. Although cardiac myxomas are histologically benign, they may be lethal because of their strategic position. Most cases are sporadic, but rare familial occurrence has been described. PATIENTS AND RESULTS: The left atrial myxoma with cerebral embolism was diagnosed in the 21 year old female and the left atrial myxoma with acromegaly due to pituirary adenoma was subsequently diagnosed in her 19 year old male sibling. The myxoma in the male patient was successfully excised. CONCLUSION: Echocardiography can be used effectively in the diagnosis of atrial myxoma, detection of its possible recurrence, and screening other members of the family.
Acromegaly ; Adenoma ; Diagnosis ; Echocardiography ; Female ; Heart ; Humans ; Intracranial Embolism ; Male ; Mass Screening ; Myxoma* ; Recurrence ; Siblings ; Young Adult

BACKGROUND: Thyrotropin-secreting pituitary adenoma is an uncommon disease and about 150 cases has been reported in the world literature. In Korea, only seven cases were reported as yet. The authors recently experienced four cases of TSH secreting pituitary tumor and analyzed the clinical characteristics and treatment outcomes of TSH-secreting tumors in Korean. METHODS: We analyzed clinical records of the four cases who had been recently treated at Asan Medical Center and the Korean literature which deals with the previously reported seven cases of TSH-secreting pituitary tumor. RESULTS: The average age at diagnosis was 37 years (ranging from 11 to 55 years). Four were men and seven were women. After the detection of hyperthyroidism, TSH-secreting pituitary adenoma was diagnosed 3.6 years later on the average. Ten patients presented with hyperthyroidism, but one had primary hypothyroidism. Typical features of acromegaly were observed in two patients. Visual disturbance was present in three cases, and galactorrhea was present in one case. Serum TSH concentrations ranged from 1.5 to 42.5uIU/mL showing mildly elevated or unsup-pressed TSH levels despite of elevated serum thyroid hormone concentrations. Among six cases in whom a-subunit level was measured, five showed elevated a-subunit level and a-subunit/TSH molar ratio. Two of 11 cases had microadenoma and the remainder had macroadeno#ma. Immunohisto-cheical studies were done in eight cases and revealed that three were positive for TSH only and five patients were positive for multiple hormones. Eight patients underwent transsphenoidal pituitary surgery and seven (88%) of them were cured. External irradiation or octreotide was used as adjunctive treatment in three cases. After treatment, TSH levels decreased in all six patients studied, hyperthyroidism was eliminated in all eight patients studied and visual disturbance was improved in two patients. CONCLUSION: Clinical characteristics of TSH-secreting pituitary adenoma in Koreans were similar with world literature, but were more common in women, had less visual disturbance and better surgical results. Diagnosis was commonly delayed for several years. TSH-secreting pituitary adenoma may be diagnosed more frequently and earlier with widespread use of sensitive TSH assay and early and proper diagnosis would lead proper treatments with improved outcome.
Acromegaly ; Asian Continental Ancestry Group ; Chungcheongnam-do ; Diagnosis ; Female ; Galactorrhea ; Humans ; Hyperthyroidism ; Hypothyroidism ; Korea ; Male ; Molar ; Octreotide ; Pituitary Neoplasms ; Pregnancy ; Thyroid Gland

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

A non-isotopic in situ hybridization method with biotin-labelled oligonucleotide probes was used to examine growth hormone(GH) and prolactin(PRL) gene expression in 32 patients with pituitary adenomas; 13 were prolactinomas, 8 GH secreting adenomas, and 11 mixed GH and PRL secreting adenomas.Positive immunostaining for GH was found in all patients with GH secreting adenomas, and mixed GH and PRL secreting adenomas. Positive immunostaining for PRL was found in all patients with prolactinomas and 9(81.8%) of 11 mixed GH and PRL secreting adenomas, 5(62.5%) of 8 GH secreting adenomas. Immunohistochemistry revealed that 13 were lactotrope adenomas, 5 somatotrope adenomas, and 14 GH and PRL cell adenomas.In situ hybridization revealed that GH mRNA expression was found in all the patients with somatotrope adenomas and GH and PRL cell adenomas, and 6(46.1%) of 13 lactotrope adenomas. PRL mRNA expression was 100% in lactotrope and GH and PRL cell adenomas, and 4(80.0%) of 5 somatotrope adenomas.The patients with a clinical diagnosis of acromegaly had detectable PRL mRNA in their neoplasm and it is suggested that the PRL cells in the adenomas did not result from dedifferentiation, but from the neoplastic stimulus for some mixed tumors probably occurred in cells previously committed to produce PRL and GH. In lactotrope adenomas, the PRL cells of the patients without expression of GH mRNA may be arised from cells programmed to secrete PRL or precussor PRL cells rather than from mixed GH-PRL cells. The finding that some patients produced mRNA detectable by in situ hybridization, but no hormone detectable by immunohistochemistry within tumor was suggested of a silent adenoma.These observations indicated that in situ hybridization studies may improve the classification of pituitary adenomas and may provide a precise knowledge of the biology of these neoplasms.
Acromegaly ; Adenoma ; Biology ; Classification ; Diagnosis ; Gene Expression ; Human Growth Hormone ; Humans ; Immunohistochemistry ; In Situ Hybridization ; Methods ; Oligonucleotide Probes ; Pituitary Neoplasms ; Prolactin ; Prolactinoma ; RNA, Messenger