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Background: Reports on pituitary apoplexy in Korea are limited. There are two treatment options for pituitary apoplexy - surgical decompression and conservative management. In this study, we examined clinical presentation and outcomes following different therapies in Korean patients with pituitary apoplexy. Methods: Clinical data and treatment outcomes from 52 patients with pituitary apoplexy who visited Seoul National University Hospital between January 1992 and June 2008 were reviewed retrospectively. Patients were divided into 2 groups according to management options (a surgery group vs. a conservative management group) and their outcomes were compared. For patients who underwent surgery due to neurological symptoms, outcomes were compared between early and late surgery groups. Results: Headache (73.1%) was the most common presenting symptom, and more than half of the patients were reported to have visual symptoms. The most common type of pathology was a nonfunctioning pituitary adenoma (67.3%). Nine patients received conservative management and 43 underwent surgical decompression. Their recovery from neurological and endocrinological abnormalities were similar. Among patients who showed neurological symptoms, 9 underwent surgery within 1 week of symptom onset and 24 underwent surgery after 1 week. There was no significant difference between outcomes of early and late surgery groups except recovery from impaired visual deficit was greater in the late surgery group. Conclusion: Patients with pituitary apoplexy in Korea show similar clinical features as similar patients in other countries. In our study, there was no significant difference between clinical outcomes of the surgery group and the conservative treatment group. (J Korean Endocr Soc 23:247~253, 2009) ꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏꠏ

α-hydroxylase/17,20-lyase deficiency is a rare phenotype of congenital adrenal hyperplasia (CAH), and this is characterized by hyporeninemic hypertension, primary amenorrhea and abnormality of the secondary sexual characteristics (pseudohermaphroditism in men). This typ e of CAH is usually misdiagnosed at first as mineralocorticoid induced hypertension with primary aldosteroni sm, but primary amenorrhea with deficient sex hormone is a clue for making the correct diagnosis. The authors experienced 6 cases of 17α -hydroxylase/17,20-lyase deficiency in patients who ranged from 15 to 42 years of age. 4 cases were diagnosed according to the investigation of their mineralocorticoid-induced hypertension and 2 cases their primary amenorrhea and sexual infantilism. All of them had hypokalemia, hyporeninemic hypertension and an atrophied uterus and ovaries. In the genotypic male (46 XY), the testicles were atrophied in the abdominal cavity. The levels of cortisol, estrogen and dehydroepiandroste rone sulfate (DHEAS) were low, but the levels of progesterone and 11-deoxycorticosterone were high. Therefore, the diagnosis of 17α-hydroxylase/17,20-lyase deficiency should be considered in female patients who present with both sexual infantilism and mineralocorticoid hypertension. We report on these cases with a brief review of the literature. (J Korean

Background: This study was performed to evaluate the frequency and clinical characteristics of patients with active acromegaly and who show discordance of the growth hormone (GH) level and the insulin-like growth factor-I (IGF-I) level. Methods: We reviewed the medical records of the patients who were diagnosed with acromegaly between 01/01/1995 and 6/30/2007 at Seoul National University Hospital. We selected only the patients whose basal GH and IGF-I levels were available. We investigated the pre- and post-operative clinical characteristics, as well as the blood concentrations of GH and IGF-I. The concordance rate between the two hormones was examined. The patients were considered to have active disease on the basis of their IGF-I levels above the normal range, after adjustment for age and gender, and their mean basal GH value was ≥ 2.5 μg/L. The hormone levels and the clinical parameters were compared between the hormone concordant and discordant groups. Results: We reviewed the preoperative records of 103 acromegalic patients, and these patients met the above-mentioned criteria. 53 postoperative patients who were not cured by operation were monitored without them receiving radiation or medical therapy. Both the basal GH and IGF-I levels were above normal in 103 patients preoperatively, and the discordant rate was 0% (0/103 cases). Postoperatively, the discordant rate between the two hormones was increased to 30.2% (16/53 cases). Age, gender, body mass index and tumor size were insignificantly different between the concordant and discordant groups. However, postoperative residual tumors were less frequently observed in the discordant group (P = 0.006). Conclusion: For the patients with acromegaly, unlike the 0% discordance preoperatively, 30.2% of patients showed a discrepancy between their GH and IGF-I levels postoperatively. The patients who had hormonal discrepancy were less likely to have residual tumors after operation. Considering the frequency of this hormonal discrepancy, both hormone levels should be measured to evaluate the disease activity after treatment. Further, oral glucose tolerance testing should be performed and especially for the patients with an increased GH level, but who have a normal IGF-I concentration. (J Korean Endocr Soc 23:395~403, 2008)

Background: A pituitary incidentaloma is a pituitary mass discovered incidentally by computed tomography (CT) or magnetic resonance (MRI) of the brain performed for evaluation of an unrelated disease, and when the patient did not show neurological or endocrine signs and symptoms. Despite of increasing incidence, only a few studies have reported on characteristics, natural history, treatment, or follow-up strategy. Moreover no epidemiologic data exist on pituitary incidentaloma in Korean. Methods: We retrospectively enrolled subjects diagnosed as pituitary incidentaloma in Seoul National University Hospital from January 1992 to August 2006. We reviewed baseline demographic findings and the results of basal hormone tests, visual field test, and MRI (or CT) at baseline and during follow-up. Results: Among 1310 patients with pituitary adenoma, 161 subjects were diagnosed with incidentaloma. The subject age at diagnosis was 49.7 ± 15.5 years, with 84 males. Macroadenoma was more frequent (n = 110, 68.3%). with microadenomas and Rathke's cleft cysts found in 21 and 30 cases, respectively. The mean diameter of the tumor was 1.75 cm. Functional incidentalomas such as prolactinoma or acromegaly were found in 31 cases (19.3%) although there were no symptoms or visual field defects. Of 61 incidentaloma patients who did not undergo surgery or other treatment over one year, only 3 patient showed an increase in tumor size during the mean 38.0 ± 24.9 months follow-up. Conclusion: Pituitary incidentaloma generally showed a benign course despite the high prevalence of macroadenoma (68.3%). However, as about 20% of the patients had functioning tumors, pituitary hormonal evaluation at diagnosis is mandatory. Furthermore, as we experienced 3 cases with increased tumor size during follow up, including a case of pituitary microadenoma, long-term careful follow-up of pituitary incidentaloma seems necessary. A long-term, prospective study with more patients would reveal the characteristics and the natural history of pituitary incidentalomas in Korea. (J Korean Endocr Soc 23:111~116, 2008)

Background: Recently, the significance of primary aldosteronism is being recognized due to an increase in its incidence and its complications. However, it is difficult to differentiate primary aldosteronism based on radiological studies as the size of an aldosterone producing adenoma (APA) is small, and nonfunctioning adrenal incidentalomas are common. Adrenal vein sampling (AVS) has been considered as the gold standard for differentiating an aldosterone producing adenoma (APA) from bilateral idiopathic adrenal hyperplasia (BAH). The clinical implications and problems associated with AVS have not yet been reported in Korea. Method: Clinical data of 28 patients with primary aldosteronism who had undergone AVS in a hospital from 1995 to 2006 were retrospectively analyzed. Result: The study group comprised 13 males (46.4%) and 15 females (53. 5%), with a mean age of 44.5 ± 11.9 years. Clinical data indicated that 19 patients (67.9%) had APA and nine patients (32.1%) had BAH. AVS data demonstrated that 11 patients had APA and five patients had BAH. Two patients were not diagnosed despite successful AVS. AVS was not successful in 10 patients (37.5%); AV catheterization failed in two patients, was not selective in seven patients on the right side, and was not selective in one patient on the left side. Conclusion: AVS was helpful in primary aldosteronism patients with inconclusive CT and MRI findings. Selectivity of AVS should be appropriately assessed because of the technical problem of selective catheterization. (J Kor Endocr Soc 22:428~435, 2007)

Background: Recently, the detection rate for adrenal incidentaloma in Korea has been on the increase. We describe here the clinical characteristics of these tumors and describe appropriate guidelines of diagnosis and treatment. Methods: We analyzed age, sex, location, size, function, and the pathological findings for 132 patients with an adrenal mass by CT, USG, and MRI undertaken for health examinations or non-adrenal disease from January 2000 to March 2005. Results: Adrenal masses were most commonly found in patients in their sixties (31%). 62.1% of the patients were men and 37.9% were women. For the location of the masses, 53% were found in the left gland, 43.2% were found in the right gland and 3.8% were found in both glands. Of all of the masses analyzed, 66% were 1 cm to 4 cm in size, and an adenoma-like appearance was the most common finding (69.7%) seen in images. All of the pheochromocytomas and carcinomas were above 4 cm in size. Patients with a functional mass were seen in 18 cases (13.6%) and pheochromocytomas were seen in 12 cases (67%). Three patients were found with cancer (2.3%), two cases (1.5%) of a primary carcinoma and one case of a metastasis (0.8%). Conclusion: The frequency and characteristics of benign nonfunctional, functional and malignant masses that were found in our hospital were similar to those presented in studies conducted outside of Korea. Therefore, it may be possible to apply previously established guidelines to domestic patients.

Tumor-induced osteomalacia (TIO), a paraneoplastic disease, is characterized by hypophosphatemia, and caused by renal phosphate wasting inappropriately, normal or decreased 1, 25(OH)2D3 production, and defective calcification of cartilage and bone. Because the removal of the responsible tumor normalizes phosphate metabolism, unidentified humoral phosphaturic factors (phosphatonin) are believed to be responsible for this syndrome. These factors include fibroblast growth factor (FGF)-23, secreted frizzled-related protein-4 and matrix extracellular phosphoglycoprotein. However, no case of TIO producing FGF-23 has been clearly reported in Korea. Herein, a case of TIO producing FGF-23 in a 45-year-old woman is reported. The patient presented with a large tumor on her buttock, with severe bone and muscle pain. A histological examination of the tumor revealed a mixed connective tissue tumor, consisting of deposition of calcified materials and surrounding primitive spindle cells, with prominent vascularity. Whether FGF-23 is a secreted factor, as well as its levels of expression in tumors were investigated. An immunohistochemical study showed the tumor cells to be FGF-23 positive. Furthermore, the levels of serum FGF-23 were extremely high and an RT-PCR analysis, using total RNA from the tumor, revealed the abundant expression of FGF-23 mRNA. After removal of the tumor, all the biochemical and hormonal abnormalities disappeared, with marked symptomatic improvement. (J Kor Endocrine Soc 22:142~148, 2007)

1950년대 처음으로 성장호르몬 치료가 시작되었을 때 성 장호르몬은 사람 사체의 뇌하수체로부터 추출하였으며, 한 사람에서 추출된 성장호르몬의 양이 무척 적었기 때문에 공 급이 제한되었다[1]. 추출된 성장호르몬제제는 성장호르몬 결핍증 소아에서 사용되었으며, 1985년에 이 추출물이 Creutzfeldt-Jacob 질환의 발생과의 관련성 때문에 사용이 중단되었다. 1985년 이후에 임상에 사용된 모든 성장호르몬 은 재조합 DNA에서 유래된 합성 사람 성장호르몬으로서 Creutzfeldt-Jacob 질환의 원인 물질이 함유되어 있지 않으 며 여러 제약회사로부터 제조되었다. 최근의 제제는 WHO 기준 제제를 사용하여 1 mg당 3 IU의 강도를 함유하고 있 다(3 IU/mg). 이러한 생합성 성장호르몬이 가능하기 전에는 성장호르몬 치료는 성장호르몬 결핍증에 의한 저신장증을 가진 소아에 국한되었다. 현재 미국을 포함한 여러 나라에서 성장호르몬 치료는 성장호르몬 결핍증 외에도 터너증후군, 신부전증에 의한 성장지연, 자궁 내 성장 지연, Prader -Willi 증후군, 그리고 최근에는 특발성 저신장증(idiopathic short stature) 등에 의한 저신장증의 치료에 허용된다. 성장 호르몬은 인슐린 다음으로 허용된 유일한 두 번째의 재조합 DNA제제이므로, 미국 식품의약국은 모든 제조회사가 시판 후 안전성 연구를 시행하도록 요구하고 있다. 소아 성장호르 몬 치료는 대부분 경우에 성장판이 닫히고 최종 신장에 도 달한 후에 중단하였다. 성장호르몬 결핍 소아에서 성인이 된 후 성장호르몬 치료를 계속할 것인지에 대한 문제는 앞으로 논의해야 할 것이다. 뇌하수체질환과 뇌하수체기능저하증을 가진 성인에서는 보통 성장호르몬 결핍증이 동반된다. 성인 성장호르몬 결핍 증은 체구성과 대사 이상뿐 아니라 육체적 능력과 정신 기 능 장애와 관련이 있다. 이들 이상이 성장호르몬 결핍증에 고유한 소견은 아니나 특징적인 임상 증후군을 구성하고 있 다. 1996년 미국과 대부분의 유럽 국가에서 성장호르몬 결 핍증 성인에서 성장호르몬 치료가 허용되었으며, 그 후 성장 호르몬 결핍증 성인의 치료에 대한 많은 임상 연구가 진행 되어 왔다. 성장호르몬 결핍증 성인에서 성장호르몬 치료의 좋은 효과는 체구성, 뼈의 건강, 심혈관 위험인자 및 삶의 질 등에서 증명되었다. 그러나 실제 심혈관질환 빈도와 사망 률이 감소하는 지는 앞으로 확인되어야 하며, 아직도 치료비 용이 비싸다는 문제점이 있다. 전체적으로 치료는 안전한 것 으로 판단되나, 당불내인성, 뇌하수체/시상하부 종양 재발 및 암 발생의 위험과 같은 문제에 대해서는 장기간의 관찰 이 필요하다[2]. 국내에서는 1999년부터 기질성 왜소증, 터너증후군 등과

Patients with adrenocortical carcinoma (ACC) present with evidence of excessive adrenal steroid hormone in approximately 60% of cases, in which rapidly progressing Cushing's syndrome with or without virilization is the most frequent presentation. Some patients experience an increase or a decline in cortisol production through the progression of their ACC. We report on an unusual case of a cortisol-producing ACC, and the patient presented with a decline in cortisol production, followed by an increase in cortisol production, through the progression of the tumor. A 65-year-old woman who manifested with facial edema and weight gain was diagnosed with Cushing's syndrome, caused by cortisol producing ACC. The patient was treated with adrenalectomy. However, 8 months later, a metastatic hepatic tumor of recurred ACC was detected. At that time, the hormonal evaluation revealed that the liver mass did not produce any hormones. The patient was treated with metastatectomy. Four months later, a relapsed tumor was detected. Increased cortisol production was observed at that time. We speculate there was a change in the clonal dominance within the ACC and this change might cause such a difference. This is the first case report of ACC that showed variable hormone production during progression. (J Kor Endocrinol Soc 21:419~423, 2006)

Cystic islet cell neoplasms are among the rarest entities in the differential diagnosis of cystic tumor of the pancreas, and this malady raises difficult clinical problems. The diagnosis of insulinoma could be difficult if the functional activity is incomplete, which possibly leads to blunted symptoms of hypoglycemia and failure in the laboratory to provide evidence of hyperinsulinemia. Furthermore, if the imaging shows a smaller tumor than usual or an unusual morphology like cyctic tumor, then physicians can become somewhat confused. We report here on a clinical case of cystic insulinoma with the typical neuroglycopenic symptoms and laboratoryconfirmed hyperinsulinemia. At resection, a 2-cm cavitary mass without central necrosis was excised and this was confirmed histologically as a purely cystic insulioma. This is the first report of a functional cystic insulinoma of the pancreas in Korea. We suggest that the differential diagnosis of endocrine tumor must be considered for any pancreatic cyst, and especially when it is discovered in a patient who is clinically suggestive of having the associated syndrome. (J Kor Endocrinol Soc 21:552~555, 2006)

Background: Idiopathic central diabetes insipidus (CDI) can be diagnosed when it occurs in the absence of a genetic or secondary cause known to be responsible for diabetes insipidus (DI). Some studies have reported that idiopathic CDI in adults shows a more benign clinical course than in children and young patients. However, the clinical characteristics and progress of this disorder have not been fully described. Therefore, we investigated the clinical courses of adult patients over the age of sixteen years with idiopathic central DI. Methods: We reviewed the medical records of all patients who had documented cases of idiopathic CDI from 1989 to 2005, and studied clinical features, hormone data, and imaging studies at diagnosis and during at least 1-year of follow-up. Results: There were 9 male (30.0%) and 21 female (70.0%) patients with a mean age of 39.3 years at diagnosis and a mean follow-up duration of 6.9 years. At diagnosis, deficits in anterior pituitary hormones were documented in 6 patients (20%), hyperprolactinemia in 4, and hypogonadism in 2. Two patients had an anterior pituitary hormone deficiency that was newly detected at a mean 3.4 years after the onset of DI. On initial MRI, the posterior pituitary was not hyperintense in 7 of the 30 patients (23.3%), but pituitary stalk thickening was observed in 15 (50.0%). After a mean follow-up of 6.9 years (range: 1 to 18), follow-up pituitary MRI showed improvement or no changes in patients with initial MRI findings of a pituitary abnormality, and no development of new lesions in 7 patients with a normal pituitary finding on initial MRI. Conclusion: Two of the 30 patients with idiopathic CDI developed an anterior pituitary hormone deficiency during follow-up, but no subject showed any aggravation on follow-up MRI. No patient showed a newly developed pituitary abnormality on follow-up MRI after a negative finding on the initial MRI (J Kor Endocrinol Soc 21:482~489, 2006)

Herein is reported the case of a 43-year-old woman, who experienced an acute aortic dissection associated with underlying Cushing's syndrome. The patient had central obe sity and a moon face of ten years duration, but had never sought medical consultation. On the day of her presentation, she experienced a severe chest pain radiating to her back. Computed tomography revealed a Stanford type B acute aortic dissection and a left adrenal mass. From her hormonal study results, clinical symptoms and signs, she was diagnosed with Cushing's syndrome, which was due to a left adrenal adenoma. After medical treatment to stabilize the aortic dissection, she underwent left adrenalectomy. The aortic lesion of the present patient suggests that hypercortisolemia arising from Cushing's syndrome might be related to the development of acute aortic dissection. (J Kor Endocrinol Soc 21:556~559, 2006)