• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.93-97
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• 2008

Pheochromocytoma is a rare tumor of childhood, arising from adrenal medullary and chromaffin tissue. Because chronic hypoxia may induce pheochromocytoma, there have been several reports of pheochromocytoma development in cyanotic patients after corrective or palliative cardiac surgery. The variable clinical presentation of pheochromocytoma is obscured by both underlying heart disease and medications. If sudden hypertension, aggravation of a heart condition, or unusual symptoms such as diabetes mellitus develops in a cyanotic patient with congenital heart disease, pheochromocytoma must be ruled out. We report two patients presenting with cyanotic single-ventricle heart disease with pheochromocytoma.

• Advances in pediatric surgery
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• v.19 no.2
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• pp.108-121
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• 2013

목적 Pheochromocytoma of the Adrenal gland Scaled Score (PASS) 시스템과 면역화학염색 등을 통한 갈색세포종 및 부교감신경절종의 악성화 예측인자가 제시되고 있으나 명확한 병리학적 또는 분자생물학적 예측인자는 밝혀진 바 없다. 본 연구에서는 임상적, 병리학적 분석을 통해 갈색세포종 및 복강내 부교감신경절종의 악성화 예측인자를 확인하고자 하였다. 대상 및 방법 1990년 1월부터 2010년 12월까지 서울대학교어린이병원에서 수술적 절제 후 병리학적으로 갈색세포종 및 복강내 부교감신경절종으로 확진된 20명의 18세 이하 소아 환자를 대상으로 임상적 특징을 분석하였고, PASS 시스템에 따른 병리 슬라이드 판독하였다. 세포활성도를 반영한다고 알려진 유전자에 대한 항체 중 Ki-67, p53, bcl-2, mdm-2, cycline D1, p21, p27을 이용해 면역 화학검사를 한 후 결과를 확인하였다. 결과 20명의 환자 중 갈색세포종은 14명, 복강내 부교감신경절종은 6명이었다. 악성화는 각각 4명, 3명에서 관찰되었다. 혈관 침범, 주변부 지방조직 침습, 세포분열 증가가 통계적으로 유의한 악성화 예측인자였으며(각각 p=.007, .031, .031), 갈색세포종만 분석하였을 때도 통계적으로 유의하였다(각각 p=.033, .003, .019). PASS 시스템은 악성화를 예측하는데 있어 통계적으로 유의하지 않았으며, 혈관 침범, 주변부 지방조직 침습, 세포분열 증가를 항목으로 하여 새롭게 만든 병리 스코어 시스템은 악성 환자군과 양성 환자군 사이에 통계적으로 유의한 차이를 보였다(p< .001). 악성과 양성 질환 사이의 면역화학염색 결과에서 유의한 차이는 없었다. 결론 소아에서 갈색세포종 및 복강내 부교감신경절종의 악성화 예측인자로 혈관 침범, 주변부 지방조직 침습, 세포분열 증가를 이용할 수 있다. 소아에서 PASS시스템으로 악성화를 예측할 수 없었으나, 새로운 병리스코어 시스템으로 악성 환자군을 예측할 수 있었다. 면역화학검사 결과 세포 활성도를 반영하는 인자들은 악성화를 예측할 수 없었다.

• The Korean Journal of Nuclear Medicine
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• v.29 no.3
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• pp.319-327
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• 1995

$^{131}I$-metaiodobenzylguanidine(MIBG) has been used for the diagnosis and treatment of neural crest tumors. We report our experience with this agent in 8 patients[1 multiple endocrine neoplasia(MEN) type IIb; 2 malignant pheochromocytoma; 5 medullary thyroid carcinoma(MTC)]. The therapeutic procedure consisted of 30-200 mCi of $^{131}I-MIBG$ administered by slow I.V. infusion, given at 3-6 months intervals. Commutative activity ranged from 150 mCi to 410 mCi, in 1 to 4 courses. $^{131}I-MIBG$ therapy resulted in significant disease free interval in 1 malignant pheochromocytoma(no measurable lesion) after surgery; complete hormonal and tumoral response in 2 MTC(1 MEN IIb): stable disease in 1 recurred pheochromocytoma(MEN IIb): stable disease but symptomatic improvement in 1 MTC, progressive disease in 1 malignant pheochromocytoma and 2 MTC. The patients who showed progression appeared to have large inoperable tumors or postoperative remnant tumors.

• 대한핵의학회:학술대회논문집
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• 1990.04a
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• pp.190.1-190.1
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• 1990

• 대한핵의학회:학술대회논문집
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• 1995.05a
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• pp.210-210
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• 1995

• The Korean Journal of Nuclear Medicine
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• v.33 no.4
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• pp.422-429
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• 1999

Purpose: The measurement of radiation absorbed dose is useful to predict the response after I-131 labeled metaiodobenzylguanidine (MIBG) therapy and determine therapy dose in patients with unresectable or malignant pheochromocytoma. We estimated the absorbed dose in tumor tissue after high dose I-131 MIBG in a patient with pheochromocytoma using a gamma camera and Medical Internal Radiation Dose (MIRD) formula. Materials and Methods: A 64-year old female patient with pheochromocytoma who had multiple metastases of mediastinum, right kidney and periaortic lymph nodes, received 74 GBq (200 mCi) of I-131 MIBG. We obtained anterior and posterior images at 0.5, 16, 24, 64 and 145 hours after treatment. Two standard sources of 37 and 74 MBq of I-131 were imaged simultaneously. Cummulated I-131 MIBG uptake in tumor tissue was calculated after the correction of background activity, attenuation, system sensitivity and count loss at a high count rate. Results: The calculated absorbed radiation dose was 32-63 Gy/ 74 GBq, which was lower than the known dose for tumor remission (150-200 Gy). follow-up studies at 1 month showed minimally reduced tumor size on computed tomography, and mildly reduced I-131 MIBG uptake. Conclusion: We estimated radiation absorbed dose after therapeutic I-131 MIBG using a gamma camera and MIRD formula, which can be peformed in a clinical nuclear medicine laboratory. Our results suggest that the measurement of radiation absorbed dose in I-131 MIBG therapy is feasible as a routine clinical practice that can guide further treatment plan. The accuracy of dose measurement and correlation with clinical outcome should be evaluated further.

• The Korean Journal of Nuclear Medicine
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• v.28 no.3
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• pp.402-406
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• 1994

Pheochromocytomas are catecholamine-producing tumors of neuroectodermal origin, and may arise wherever chromaffin cells are located. They are rare but potentially lethal and amenable to surgical cure. Once a clinical diagnosis has been established, localization becomes of importance to direct surgical approach. MIBG scintigraphy is the initial localizing procedure of choice, due to its ability to screen the entire body, especially in the detection of extraadrenal and recurrent pheochromocytoma. A case of recurrent extraadrenal pheochromocytoma diagnosed by $^{131}I$-MIBG scintigraphy is presented with review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.64 no.3
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• pp.219-223
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• 2008

Pheochromocytoma is derived from the chromaffin tissue. The typical finding of pheochromocytoma is paroxysmal hypertension accompanied with various signs and symptoms that are due to the excess of catecholamines or other bioactive substances. Yet the diagnosis is sometimes difficult to make because its clinical presentation is quite variable. Especially, hemoptysis is a very rare symptom, so the diagnosis is often missed or delayed. Without making the correct diagnosis and then subsequently administering treatment, the condition may be fatal. We herein report on a 68 year-old woman who was admitted because of abdominal pain and hemoptysis. The initial radiologic findings suggested pulmonary edema with alveolar hemorrhage. The urine catecholamine levels were elevated and she developed catecholamine-induced cardiomyopathy. We performed bronchial arterial embolization and we administered alpha blocker medication for controlling the hemoptysis and hypertension. After the temporary symptomatic improvement, her clinical course was aggravated by pneumonia and pulmonary edema. In spite of performing definitive surgery for pheochromocytoma, she died of postoperative hemodynamic instability.

• Nuclear Medicine and Molecular Imaging
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• v.43 no.5
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• pp.436-442
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• 2009

Purpose: We underwent this study to evaluate the diagnostic potential of I-123/I-131 metaiodobenzylguanidine (MIBG) scintigraphy alone in the initial diagnosis of pheochromocytoma, compared with biochemical test and anatomic imaging. Materials & Methods: Twenty two patients (M:F=13:9, Age: $44.3{\pm}\;19.3$ years) having the clinical evaluation due to suspicious pheochromocytoma received the biochemical test, anatomic imaging modality (CT and/or MRI) and I-123/I-131 MIBG scan for diagnosis of pheochromocytoma, prior to histopathological confirmation. MIBG scans were independently reviewed by 2 nuclear medicine physicians. Results: All patients were confirmed histopathologically by operation or biopsy (incisional or excisonal). In comparison of final diagnosis and findings of each diagnostic modality, the sensitivities of the biochemical test, anatomic imaging, and MIBG scan were 88.9%, 55.6%, and 88.9%, respectively. And the specificities of the biochemical test, anatomic imaging, and MIBG scan also were 69.2%, 69.2%, and 92.3%, respectively. MIBG scan showed one false positive (neuroblastoma) and one false negative finding. There was one patient with positive MIBG scan and negative findings of the biochemical test, anatomic imaging. Conclusion: Our data suggest that I-123/I-131 MIBG scan has higher sensitivity, specificity, positive predictive value, negative predictive value and accuracy than those of biochemical test and anatomic imaging. Thus, we expect that MIBG scan is e tectively used for initial diagnosis of pheochromocytoma alone as well as biochemical test and anatomic imaging.

• The Korean Journal of Nuclear Medicine
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• v.31 no.3
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• pp.395-398
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• 1997

Whole body $^{99m}Tc-MIBI$ scan in conjunction with parathyroid scan is an effective method in detecting parathyroid lesions in patients with bone pain and possible bone lesions such as brown tumors.