부신 우연종에서 발견된 갈색세포종의 특성 분석

김예안,황보율,김민주,최형진,서제현,이예나,곽수헌,구유정,오태정,노은,배재현,김정희,박경수,김성연 대한내분비학회 2012 Endocrinology and metabolism Vol.27 No.2

Background: In approach to an adrenal incidentaloma, early exclusion of pheochromocytoma is clinically important, due to the risk of catecholamine crisis. The aims of this study are to investigate the characteristics of incidentally detected pheochromocytomas,compared with that of the other adrenal incidentalomas, and to compare these characteristics with those of symptomatic pheochromocytomas. Methods: In this retrospective study, we reviewed the medical records of 198 patients with adrenal incidentaloma from 2001 to 2010. We analyzed the clinical, laboratory and radiological data of pheochromocytomas, in comparison with those of the other adrenal incidentalomas. We also compared the characteristics of these incidentally detected pheochromocytomas with the medical records of 28 pathologically proven pheochromocytomas, diagnosed based on typical symptoms. Results: Among the 198 patients with adrenal incidentaloma, nineteen patients were diagnosed with pheochromocytoma. Pheochromocytomas showed larger size and higher Hounsfield unit at precontrast computed tomography (CT) than did non-pheochromocytomas. All pheochromocytomas were larger than 2.0 cm, and the Hounsfield units were 19 or higher in precontrast CT. When both criteria of size > 2.0 cm and Hounsfield unit > 19 were met, the sensitivity and specificity for the diagnosis of pheochromocytoma were 100% and 79.3%, respectively. Compared with patients with pheochromocytoma, diagnosed based on typical symptoms,patients with incidentally detected pheochromocytoma were older, presented less often with hypertension, and showed lower levels of 24-hour urine metanephrine. Conclusion: Adrenal incidentaloma with < 2.0 cm in size or ≤ 19 Hounsfield units in precontrast CT imaging was less likely to be a pheochromocytoma. Patients with incidentally discovered pheochromocytoma showed lower catecholamine metabolites, compared with those patients with symptomatic pheochromocytoma.

Clinical Experiences of Pheochromocytoma in Korea

김광현,최영득,정재승,김원태,오철규,최윤병,유호송,함원식 연세대학교의과대학 2011 Yonsei medical journal Vol.52 No.1

Purpose: We report herein 119 patients with pheochromocytoma at our institute over the last 23 years. Materials and Methods: Between 1986 and 2009, 119 patients were diagnosed with pheochromocytoma at our institute. We reviewed the medical records of these patients. Results: Of 119 patients, 45 were male and 74 were female, and mean age was 43.83 ± 13.49 years. Forty-three patients (36.1%) were diagnosed incidentally, and 8 patients (6.7%) were found to have familial pheochromocytoma. The mean dimension of the tumors was 5.89 ± 3.18 cm. 4 patients had bilateral tumors; three of these patients were found to have familial pheochromocytoma and 1 patient was diagnosed with malignant pheochromocytoma. A total of eight patients (6.7%) were found to have malignant pheochromocytoma. In 1 patient, metastasis to a lymph node was found at the time of diagnosis. Metastases were found at a mean of 49 ± 25.83 (6-75) months after surgery in the other seven patients. 6 patients died of malignant pheochromocytoma at a mean of 31 ± 28.71 months (1-81) after diagnosis, and the other 2 patients survived for 15 and 24 months, respectively. Conclusion: Approximately 35% of patients with pheochromocytoma are diagnosed incidentally, and the number of detected cases is increasing. Although familial pheochromocytoma was found only in 6.7% of the patients, genetic testing should be considered in all patients, especially in patients with a family history, young age, or multifocal, bilateral, extra-adrenal, or malignant tumors. Given that malignant pheochromocytomas are frequently diagnosed during the follow-up period, long-term follow-up is necessary to confirm the absence of recurrence or metastasis.

Higher sympathetic activity as a risk factor for skeletal deterioration in pheochromocytoma

Kim, Beom-Jun,Kwak, Mi Kyung,Kim, Jae Seung,Lee, Seung Hun,Koh, Jung-Min Elsevier 2018 Bone Vol.116 No.-

<P><B>Abstract</B></P> <P>Despite the potential biological importance of sympathetic activity in human bone metabolism, its effects on bone microarchitecture, a key determinant of bone quality, has not been thoroughly studied. In the present study, we investigated the lumbar spine trabecular bone score (TBS) as an indicator of skeletal deterioration in pheochromocytoma. Among 620 consecutive patients with newly diagnosed adrenal incidentaloma, 29 with histologically confirmed pheochromocytoma (a catecholamine-secreting neuroendocrine tumor) and 266 with nonfunctional adrenal incidentaloma were defined as cases and controls, respectively. After adjustment for confounders, subjects with pheochromocytoma had 2.9% lower lumbar spine TBS than those without pheochromocytoma (<I>P</I> = 0.038). Moreover, urinary normetanephrine level, but not urinary metanephrine level, was inversely correlated with lumbar spine TBS (<I>P</I> = 0.009). Subjects in the highest urinary normetanephrine quartile showed markedly lower lumbar spine TBS than those in the lowest quartile (<I>P</I> = 0.018), in a dose-response manner across increasing urinary normetanephrine quartile categories (<I>P</I> for trend = 0.021). Consistent with the results of previous studies, subjects with pheochromocytoma had significantly lower bone mass at the lumbar spine and higher serum level of C-terminal telopeptide of type I collagen than controls (<I>P</I> = 0.013 and 0.002, respectively). These findings provide clinical evidence that catecholamine excess and the resultant sympathetic overstimulation in pheochromocytoma may contribute to bone fragility, especially in the trabecular bone, through a weak microarchitecture in addition to a lower bone mass and increased bone resorption, and support the possibility of pheochromocytoma as a secondary cause of osteoporosis.</P> <P><B>Highlights</B></P> <P> <UL> <LI> Subjects with pheochromocytoma had a significantly lower lumbar spine trabecular bone score (TBS). </LI> <LI> Urinary normetanephrine level, but not urinary metanephrine level, was inversely correlated with lumbar spine TBS. </LI> <LI> Pheochromocytoma was associated with lower bone mass at the lumbar spine and with higher bone resorption rate. </LI> <LI> Sympathetic overstimulation can adversely affect both bone mass and bone quality in humans. </LI> </UL> </P>

척추와 대동맥주위 림프절로 전이한 거대 갈색세포종

박연원 ( Yeon Won Park ),문한주 ( Han Ju Moon ),한정석 ( Jung Suk Han ),한지민 ( Ji Min Han ),박종욱 ( Jong Wook Park ),구윤희 ( Yun Hyi Ku ) 영남대학교 의과대학 2017 Yeungnam University Journal of Medicine Vol.34 No.2

Approximately 10-15% of pheochromocytomas are malignant. There are insufficient histologic criteria for the diagnosis of malignant pheochromocytoma. Thus, the term malignant pheochromocytoma is restricted to tumors with local invasion or distant metastases. We experienced a case of malignant pheochromocytoma recurred with spinal metastasis 4 years after the surgery for huge benign pheochromocytoma. A 68-year-old female was admitted for trunk and back pain. The patient had a history of surgery 4 years ago for a 10.0× 9.5×7.5 cm sized benign pheochromocytoma at the left adrenal gland. A thoracolumbar magnetic resonance imaging showed a tumor in the 7th thoracic vertebral body and a 24-hour urinary norepinephrine increased, suggesting metastatic recurrence of malignant pheochromocytoma. After metastasectomy in the 7th thoracic vertebral body, urine catecholamine was normalized and pain also disappeared. However, a metastatic lesion was found in the paraaortic area on a follow-up abdominal computed tomography scan and an additional metastasectomy was performed. The pathology confirmed the diagnosis of metastatic pheochromocytoma in the paraaortic lymph nodes. She is supposed to be treated with adjuvant iodine 131-meta-iodobenzylguanidine therapy. In our experience, a close follow-up should be considered in patients who had a huge benign pheochromocytoma due to the possibility of malignant metastases.

Laparoscopically Resected Composite Pheochromocytoma-Ganglioneuroma

김나래,김태은,이정남,엄영실,정동해,박상희,조현이 대한내분비학회 2011 Endocrinology and metabolism Vol.26 No.4

Composite pheochromocytoma-ganglioneuroma is one of the mixed neuroendocrine-neural tumors composed of pheochromocytoma and other neural crest derivatives. To date, less than 50 cases of composite pheochromocytoma have been reported, and about 70% of the accompanying tumors were ganglioneuromas. Here, we describe six cases of composite pheochromocytoma-ganglioneuromas in five men and one woman, aged 33 to 64. The size of the tumors ranged from 3.0 to 11.0 cm, and four out of the six presented with intermittent onset of hypertension, palpitation, or dizziness. Microscopically, each tumor was composed of large pleomorphic shaped chromaffin cells arranged in the Zellballen patterns characteristic of pheochromocytoma, and they were mixed with clusters of mature ganglion cells and bundles of spindle-shaped Schwann cells characteristic of ganglioneuroma of variable proportions. All were successfully treated laparoscopically, and none were associated with multiple endocrine neoplasm syndrome or neurofibromatosis. Preoperative diagnosis of a composite pheochromocytoma-ganglioneuroma is impossible because of the low incidence rate, and the radiological findings and symptoms are similar to those of typical pheochromocytomas. Although the significance of microscopic detection of the nonpheochromocytoma component from pheochromocytoma has not yet been clarified, microscopic identification of the composite pheochromocytoma-ganglioneuroma is important because cumulative cases are used in an effort to predict the behavior of this composite tumor.

무증상 갈색 세포종

박재호 ( Jae Ho Park ),최수윤 ( Su Yun Choi ),소의영 ( Euy Young Soh ) 대한임상종양학회 2010 Korean Journal of Clinical Oncology Vol.6 No.2

목적: 무증상 갈색세포종은 전체 갈색세포종의 약 1.6-30%를 차지하며 방사선적 검사에서 우연히 발견된다. 본 저자들은 갈색세포종으로 진단된 환자들을 대상으로 무증상 갈색세포종과 증상이 있는 갈색세포종을 비교하여 임상 양상의 특징을 알아보고자 하였다. 대상 및 방법: 본 연구는1988년 4월 1일부터 2009년 9월 30일까지 부신내 종양 및 후복부 종양으로 수술을 시행 후 조직검사상 갈색세포종으로 진단된 57명의 환자를 대상으로 하였다. 무증상 갈색세포종 12예와 전형적 갈색세포종 45예를 비교하여 임상 양상 및 치료 결과에 차이가 있는지 분석하였다. 결과: 무증상 갈색세포종 환자 중 8명(66.7%)에서 cathecolamine 과다분비가 관찰되었다. 전형적 갈색세포종 군과 무증상 갈색세포종 군을 비교하였을 때 catecholamine 및 그 대사물의 평균 수치는 무증상 군에서 모두 낮게 측정되었다. 전체 57명의 환자 중 47명의 환자에게 수술 전 처치를 했음에도 불구하고, 수술 중 수축기 혈압이 160이상 되는 경우가 38명의 환자에서 관찰되었다. 무증상 갈색세포종에서도 수술 중 수축기 혈압이 160mmHg이상 상승한 경우는 6예였다. 양군을 비교하였을 때 고혈압 발작 양상은 전형적 갈색세포종과 의미있는 차이는 없었다. 무증상 갈색세포종 중 1예, 전형적 갈색세포종 중 4예에서 추적 관찰하는 동안 재발되었으며 전형적 갈색세포종 중 1예는 악성 갈색 세포종이었다. 결론: 부신 종양이 진단되었을 경우 catecholamine에 대한 생화학적 검사는 필수적이다. 무증상 갈색세포종이라 하더라도 catecholamine의 과다 분비가 진행되고 있으므로 수술적 치료가 일차적으로 시행되어야 할 것이며 적절한 수술 전 처치도 필요하겠다. 수술 후에도 재발 및 전이 여부를 확인하기 위한 정기적인 추적관찰을 고려해야 할 것이다. Purpose: Approximately 1.6-30% of histologically proven pheochromocytomas are discovered by radiologic examination in the absence of symptoms. The aim of this study was to analyze the asymptomatic pheochromocytoma, to compare with typical pheochromocytoma. Meterials and Methods: Fifty seven patients were reviewed who diagnosed pheochromocytoma at from 1988 to 2009. In the current study, we evaluated the characteristics of asymptomatic pheochromocytoma(AP) in 12 patients and compared with other 45 patients with typical pheochromocytoma(TP). Results: Catecholamine overproduction was observed in 8 (66.7%) of AP patients. In particular, the mean values of biochemical parameters were lower in AP patients compared to TP patients, and single catecholamine overproduction was frequently observed. Despite preoperative preparation in forty seven patients, intraoperative hypertension occurred in thirty eight patients with spiking of >160 mmHg. In the asymptomatic group, there were 6 cases of spiking blood pressure higher then 160 mmHg. Intraoperative hemodynamic changes were not significantly different between AP and TP. During follow-up, there were five cases of recurrent disease (1 in the asymptomatic group and 4 in the typical group), and 1 case of recurrent disease was malignant. Conclusion: Catecholamine screening in all patients with adrenal tumors is essential. Even in asymptomatic pheochromocytoma patients, there exists a likelihood of catecholamine overproduction, the surgical extirpation may offer an advantage. Such patients should be appropriately prepared before surgical intervention and regular and long term follow-up should also be considered to detect recurrent disease.

내분비-대사 : 부신 크롬친화세포종의 임상 및 병리조직학적 고찰

홍석호 ( Suk Ho Hong ),강구흠 ( Gu Hyum Kang ),강대영 ( Dae Young Kang ) 대한내과학회 2012 대한내과학회지 Vol.82 No.1

The diagnosis and prognosis of malignant pheochromocytoma are important. Other than distant metastasis, the pathology is insufficient to diagnose malignant versus benign pheochromocytoma. We analyzed 33 pheochromocytoma cases using the histological grade on the Thompson and Kimura scales. We compared the Thompson and Kimura scores with the degree of immunohistochemical staining for Ki-67 and heat shock protein 90 (Hsp90). The most frequent symptom was hypertension (17 cases; 51.5%) and four patients had accompanying tumors: one case each of multiple endocrine neoplasm, papillary carcinoma of the thyroid, gastric adenocarcinoma, and neurofibroma. The mean Thompson score was 3.67 in benign pheochromocytoma and 10 in malignant pheochromocytoma, and the Kimura score was 2.17 and 5, respectively. The average immunohistochemical staining for Ki-67 was 5.77 and 10.44 in benign and malignant pheochromocytoma, respectively, while Hsp90 immunohistochemical staining was positive only in the cytoplasm of malignant heochromocytoma. Immunohistochemical staining for Ki-67 and Hsp90 was seen in malignant pheochromocytoma. The Thompson and Kimura scales and immunohistochemical results could help to differentiate benign and malignant pheochromocytoma. (Korean JMed 2012;82:60-66)

Case report of pseudo-pheochromocytoma in a dog

Jiwoon Jung,Eunbin Chung,Sungho Lee,Jungnok Ryu,Gunha Hwang,Tae Sung Hwang,Hee Chun Lee,이재훈,이동빈 한국예방수의학회 2022 예방수의학회지 Vol.46 No.4

Pseudo-pheochromocytoma is defined as an adrenal tumor that has the typical characteristics of pheochromocytoma likely hypertension but is histopathologically diagnosed to other types of adrenal tumors. 11-year-old, castrated male Maltese presented for a left adrenal mass with hypertension. According to clinical signs and radiological features, a temporary diagnosis was made of pheochromocytoma. The adrenalectomy was performed and the histopathological examination of the resected adrenal gland was diagnosed as cortical adenocarcinoma. This report describes the case of pseudo-pheochromocytoma in a dog and implies the salience that even if pheochromocytoma is diagnosed based on all test results, pseudo-pheochromocytoma should not be excluded.

Prevalence and Patterns of Left Ventricular Dysfunction in Patients with Pheochromocytoma

박재형,김규섭,김준형,이재환,최시완,정진옥,성인환,설지영 한국심초음파학회 2011 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.19 No.2

Background: Excessive catecholamine release in pheochromocytoma is known to cause transient reversible left ventricular (LV) dysfunction, such as in the case of pheochromocytoma-associated catecholamine cardiomyopathy. We investigated patterns of clinical presentation and incidence of LV dysfunction in patients with pheochromocytoma. Methods: From January 2004 to April 2011, consecutive patients with pheochromocytoma were retrospectively studied with clinical symptoms, serum catecholamine profiles, and radiologic findings. Patterns of electrocardiography and echocardiography were also analyzed. Results: During the study period, a total of 36 patients (21 males, 49.8 ± 15.8 years, range 14-81 years) with pheochromocytoma were included. In the electrocardiographic examinations, normal findings were the most common findings (19, 52.8%). LV hypertrophy in 12 cases (33.3%), sinus tachycardia in 3 (8.3%), ischemic pattern in 1 (2.8%) and supraventricular tachycardia in 1(2.8%). Echocardiographic exam was done in 29 patients (80.6%). Eighteen patients (62.1%) showed normal finding, 8 (27.6%)revealed concentric LV hypertrophy with normal LV systolic function, and 3 (10.3%) demonstrate LV systolic dysfunction (LV ejection fraction < 50%). Three showed transient LV dysfunction (2 with inverted Takotsubo-type cardiomyopathy and 1 with a diffuse hypokinesia pattern). Common presenting symptoms in the 3 cases were new onset chest discomfort and dyspnea which were not common in the other patients. Their echocardiographic abnormalities were normalized with conventional treatment within 3 days. Conclusion: Out of total 36 patients with pheochromocytoma, 3 showed transient LV systolic dysfunction (catecholamine cardiomyopathy). Pheochromocytoma should be included as one of possible causes of transient LV systolic dysfunction.

Recurrent Catecholamine-Induced Cardiomyopathy in a Patient With a Pheochromocytoma

장세영,양동헌,이상혁,김재희,박선희,조용근,채성철,전재은,박의현 대한심장학회 2009 Korean Circulation Journal Vol.39 No.6

Pheochromocytomas presents with variable clinical manifestations. Cardiomyopathy caused by a pheochromocytoma is well known. We report the case of a 62-year-old woman with recurrent left ventricular dysfunction, who was subsequently found to have a pheochromocytoma. The patient had two different patterns of cardiomyopathy. Patients with a cardiomyopathy, of non-specific origin, should have a pheochromocytoma ruled out. Pheochromocytomas presents with variable clinical manifestations. Cardiomyopathy caused by a pheochromocytoma is well known. We report the case of a 62-year-old woman with recurrent left ventricular dysfunction, who was subsequently found to have a pheochromocytoma. The patient had two different patterns of cardiomyopathy. Patients with a cardiomyopathy, of non-specific origin, should have a pheochromocytoma ruled out.