Ectopic Cushing’s syndrome caused by pheochromocytoma is rare. We reported a 15-year-old female patient who was admitted to hospital with typical Cushing’s syndrome. She had not started menstruation. Her plasma adrenocorticotropic hormone (ACTH) and 24-hour urinary free cortisol levels were extremely high. Gonadal and progestational hormone levels were also abnormal. Abdominal computed tomography scans and enhanced scans revealed multiple irregular tumors in the right adrenal. Pelvic echogram showed an infantile uterus, while the ovaries were at an immature stage of development. Retroperitoneal laparoscopic right adrenalectomy was performed without intraoperative complications. Histology and immunohistochemistry of the tumor were consistent with pheochromocytoma. Retroperitoneal laparoscopic adrenalectomy is a safe procedure with satisfactory outcomes and allows for rapid recovery.
Adolescent ; Adrenal Gland Neoplasms ; diagnosis ; secretion ; therapy ; Adrenocorticotropic Hormone ; secretion ; Female ; Humans ; Pheochromocytoma ; diagnosis ; secretion ; therapy

OBJECTIVETo evaluate the diagnosis and treatment of adrenal incidentalomas.

METHODSOne hundred and twenty-six patients were analyzed, in which 98 patients underwent operation.

RESULTSOf all types of the adrenal incidentalomas, 52 (41.3%) of them were adenomas and they were the most often; 43 (34.1%) of them were hypersecretory adrenal tumors, most of them were pheochromocytomas and Conn adenomas; all nonhypersecretory adrenal adenomas were under 6 cm, and all adrenal carcinomas were above 6 cm.

CONCLUSIONSIt is important to search for hypersecretory adrenal tumors and to detect malignant adrenal tumors. To nonhypersecretory adrenal adenomas, the sizes of tumors are the most important indexes in determining weather the tumors are benign or malignant and weather the tumors need to be treated with operation or not.

Adolescent ; Adrenal Gland Neoplasms ; classification ; diagnosis ; therapy ; Adrenocortical Adenoma ; diagnosis ; therapy ; Adult ; Aged ; Child ; Female ; Humans ; Hyperaldosteronism ; diagnosis ; therapy ; Male ; Middle Aged ; Pheochromocytoma ; diagnosis ; therapy

PURPOSE: There are no specific clinical and histopathologic characteristics of malignant pheochromocytoma and the optimal treatment modality has not been established yet. We analyzed the clinical and histopathologic features of malignant pheochromocytoma and treatment results. MATERIALS AND METHODS: We reviewed the clinical records of 10 patients with malignant pheochromocytoma diagnosed at Seoul National University Hospital from March 1987 to June 1998. RESULTS: Nine of 10 (90%) patients had functional tumors. The biochemical laboratory findings showed elevated 24-hour urine VMA level in nine patients available. The median size of the tumors was 11x11 cm. Six of 10 (60%) patients were initially diagnosed as malignant tumors because of direct invasions to adjacent tissues or distant metastases. On the other hand, remaining 4 patients were initially diagnosed as benign, but the distant metastases developed metachronously after resection of the primary lesion. The median duration between the initial operation and the detection of metastases was 57 months (range: 47~72 months) in these patients. The liver was the most common site of metastases (60%). With regards to the histopathological features, most of the tumors (87.5%) showed capsulation, necrosis and hemorrhage. The findings of lymphatic invasion, angio-invasion, and mitosis were found in 62.5% of the cases. All but 2 patients were initially treated with radical operation for the primary lesions. The disease recurrences or metastases occurred in 7 out of 10 patients. Of these, 4 patients were treated with chemotherapy or interferon- a after recurrences. Overall, the median survival for all patients was 82 months (range: 37~143 months). Two patients is alive and only one patient is alive without recurrence. CONCLUSION: The careful follow-up for at least 5 years and the aggressive multi-disciplinary therapy may be needed for the diagnosis and the management of malignant pheochromocytoma.
Diagnosis ; Drug Therapy ; Follow-Up Studies ; Hand ; Hemorrhage ; Humans ; Liver ; Mitosis ; Necrosis ; Neoplasm Metastasis ; Pheochromocytoma* ; Recurrence ; Seoul

Sporadic malignant pheochromocytoma, a rare disease with poor prognosis, is always difficult to treat due in part to lack of effective agents. We presented three patients with advanced malignant pheochromocytoma treated by sunitinib, which indicates that sunitinib is an effective agent for this malignancy.
Adrenal Gland Neoplasms ; drug therapy ; Adult ; Female ; Humans ; Indoles ; therapeutic use ; Male ; Middle Aged ; Pheochromocytoma ; drug therapy ; Protein Kinase Inhibitors ; therapeutic use ; Pyrroles ; therapeutic use

Pheochromocytoma in pregnancy is very rare but it is associated with very high maternal and fetal mortality. Therefore, it is important to include pheochromocytoma in the differential diagnosis of hypertension associated with pregnancy. It is difficult to make a diagnosis of pheochromocytoma in pregnancy before delivery. The characteristic symptoms of pheochromocytoma could be initiated during delivery because the process of delivery, general anesthesia, fetal movement, induce acute surge of catecholamine release, which could also induce cardiomyopathy. Early diagnosis and intensive care can affect the prognosis of cardiomyopathy induced by pheochromocytoma. Proper management with alpha-blockade, beta-blockade and angiotension converting enzyme inhibitor could acutely reverse the course of cardiomyopathy.
Adrenal Gland Neoplasms/surgery ; Adrenal Gland Neoplasms/diagnosis* ; Adrenal Gland Neoplasms/complications ; Adult ; Cardiovascular Agents/therapeutic use ; Disease-Free Survival ; Echocardiography ; Electrocardiography ; Female ; Human ; Myocardial Diseases/ultrasonography ; Myocardial Diseases/etiology* ; Myocardial Diseases/drug therapy ; Pheochromocytoma/surgery ; Pheochromocytoma/diagnosis* ; Pheochromocytoma/complications ; Pregnancy ; Pregnancy Complications, Cardiovascular/etiology* ; Pregnancy Complications, Neoplastic/surgery ; Pregnancy Complications, Neoplastic/diagnosis* ; Pregnancy Outcome* ; Puerperium ; Tomography, X-Ray Computed ; Substances: Cardiovascular Agents

Paragangliomas are relatively rare chromaffin cell tumors which may be cured through resection. Patients with paragangliomas may develop metastatic diseases. There is no consensus regarding refractory chemotherapy for treatment of metastatic disease. In this report, we presented a case of a 43-year-old woman who was admitted to the hospital with a history of episodic headaches, diaphoresis, and weakness. Elevated plasma catecholamine levels and a right paraaortic mass were observed on computed tomography. The mass was excised, and a diagnosis of paraganglioma was confirmed. After 20 months of follow-up, local recurrence and metastases were detected in the thorax, abdomen, and skeletal system. Plasma and urinary catecholamine levels were high. Chemotherapy was administered, and no improvement was observed. Therefore, following this palliative conventional chemotherapy, sorafenib was administered for three months, and, finally, positron emission tomography showed that the patient's lesions had completely regressed.
Abdomen ; Adult ; Chromaffin Cells ; Consensus ; Diagnosis ; Drug Therapy ; Female ; Follow-Up Studies ; Headache ; Humans ; Neoplasm Metastasis ; Paraganglioma* ; Paraganglioma, Extra-Adrenal ; Pheochromocytoma ; Plasma ; Positron-Emission Tomography ; Recurrence ; Thorax

Pheochromocytoma is a rare disorder and functioning tumor composed of chromaffin cells that secrete catecholamines. Patients with a pheochromocytoma 'crisis' have a high mortality in spite of aggressive therapy. We present a case with a severe acute catecholamine cardiomyopathy presenting ST segment elevation with cardiogenic shock after hemorrhage into a left suprarenal tumor. Intra-aortic balloon pump (IABP) support, combined with inotropic therapy, was performed. However, the patient deteriorated rapidly and was unresponsive to a full dose of inotropics and IABP. We decided to apply extracorporeal membrane oxygenation (ECMO) device for the patient. His clinical state began to improve 3 days after ECMO. After achieving hemodynamic stabilization, he underwent successful laparoscopic left adrenalectomy. He needed no further cardiac medication after discharge.
Adrenal Glands/pathology ; Adult ; Cardiomyopathies/*diagnosis/*etiology ; Catecholamines/*adverse effects ; Coronary Angiography/methods ; Diagnosis, Differential ; Electrocardiography/methods ; Extracorporeal Membrane Oxygenation/*methods ; Humans ; Intra-Aortic Balloon Pumping ; Male ; Myocardial Infarction/*diagnosis ; Pheochromocytoma/*therapy ; Time Factors ; Tomography, X-Ray Computed/methods

Pheochromocytoma (PCC) is a neuroendocrine tumor that mainly arises from the medulla of the adrenal gland. Some PCCs become malignant and metastasize to other organs. For example, it typically involves skeletal system, liver, lung, and regional lymph nodes. However, only a few cases of PCC with brain metastasis have been reported worldwide. We report a case of metastatic brain tumor from PCC in South Korea in 2016. A 52-year-old man presented with headache, dizziness and motor aphasia. He had a medical history of PCC with multi-organ metastasis, previously underwent several operations, and was treated with chemotherapy and radiotherapy. Brain MRIs showed a brain tumor on the left parietal lobe. Postoperative pathology confirmed that the metastatic brain tumor derived from malignant PCC. This is the first report PCC with brain metastasis in South Korea.
Adrenal Glands ; Aphasia, Broca ; Brain Neoplasms ; Brain* ; Dizziness ; Drug Therapy ; Headache ; Humans ; Korea ; Liver ; Lung ; Lymph Nodes ; Magnetic Resonance Imaging ; Middle Aged ; Neoplasm Metastasis* ; Neuroendocrine Tumors ; Parietal Lobe ; Pathology ; Pheochromocytoma* ; Radiotherapy

BACKGROUND/AIMS: We investigated the clinical characteristics and follow-up findings of subjects with adrenal incidentalomas in a single, tertiary-care hospital in South Korea. METHODS: The study consisted of a retrospective analysis of 282 adrenal incidentaloma patients who underwent radiographic and endocrinological evaluations at Samsung Medical Center in Seoul, South Korea, between January 2004 and July 2011. RESULTS: Most (86.2%) of the subjects were found to have nonfunctioning tumors. Functioning tumors were seen in 39 patients (13.8%). Among them, 28 (9.9%) had subclinical Cushing syndrome (SCS), six (2.1%) had pheochromocytoma, and five (1.8%) had primary hyperaldosteronism. Malignant adrenal tumors were discovered in three cases: two (0.7%) were primary adrenal cancers, and one (0.4%) was a secondary metastasis from a lung cancer. Significant risk factors for functional tumors were female gender (odds ratio [OR], 3.386; 95% confidence interval [CI], 1.611 to 7.117; p = 0.0013) and a noncontrast attenuation value of > 10 Hounsfield units (OR, 2.806; 95% CI, 1.231 to 6.397; p = 0.0141). During follow-up (mean, 22.5 months) of 72 of the patients, three (4.2%) developed hormonal changes due to functional tumors. One was confirmed as pheochromocytoma by histopathology, and the others were diagnosed with SCS and followed routinely without surgical intervention. No malignant transformation was found in these patients. CONCLUSIONS: Based on these findings, initial hormonal and radiographic evaluations for adrenal incidentalomas appear to be more important than follow-up tests because functional or malignant changes are rare.
*Adrenal Gland Neoplasms/blood/diagnosis/epidemiology/therapy ; Aged ; *Cushing Syndrome/blood/diagnosis/epidemiology/therapy ; Disease Progression ; Female ; Hormones/blood ; Humans ; *Hyperaldosteronism/blood/diagnosis/epidemiology/therapy ; Logistic Models ; Male ; Middle Aged ; Odds Ratio ; *Pheochromocytoma/blood/diagnosis/epidemiology/therapy ; Predictive Value of Tests ; Prognosis ; Republic of Korea/epidemiology ; Retrospective Studies ; Risk Factors ; Tertiary Care Centers ; Time Factors ; Tomography, X-Ray Computed ; Tumor Markers, Biological/blood

PURPOSE: Adrenocortical tumors are uncommon in children and comprise only a small proportion of primary adrenal neoplasms. The biologic behavior of these tumors may be very difficult to predict, and their rarity has hindered identification of clinical characteristics. Patients with functioning tumors have excessive steroid hormone production, and the clinical manifestation depends on the predominant hormone produced. The detection of nonfunctioning tumors is not easy and the diagnosis may be delayed. Benign tumors can be cured by complete surgical excision, but malignant cases have poor response to treatment and worse prognosis. Early diagnosis and proper management are very important because of the large proportion of functioning malignant tumors in children. We report clinical features of adrenocortical tumors in children that may be of help in the early detection, proper management, and assessment of prognosis of patients. METHODS: We reviewed the clinical characteristics of 14 cases of adrenocortical tumors, among 85 children diagnosed with adrenal tumors, who visited the Severance Hospital, College of Medicine, Yonsei University, from January 1970 to July 1996. RESULTS: 85 Patients were diagnosed with adrenal tumors. Among them, 71 cases 83.5%) were tumors of the adrenal medulla, neuroblastoma and pheochromocytoma, and 14 cases (16.5%) were adrenocortical tumors, consisting of 5 cases of adenoma, 7 cases of carcinoma, and 2 cases unspecified. The age distribution ranged from 16 months to 14 years of age, and the mean was 5 years & 11 months (median 4 years & 2 months). Sex distribution revealed a male to female ratio of 1:1.33. The left to right ratio was 3.7:1, showing a left side predominance. 13 Cases (92.9%) were functioning tumors: 12 cases (92.3%) had clinical evidence of androgen excess, among which 6 cases (46.2%) were associated with Cushing's syndrome, and 1 case was compatible with primary aldosteronism. Serum cortisol, urinary 17-ketosteroids and 17-hydroxycorticosteroids concentrations were measured in 11 cases and urinary concentrations of 17-ketosteroids were elevated in all 11 cases (100%), while 17-hydroxycorticosteroids were elevated in 4 cases (36.4%). Abnormalities of serum cortisol were found in all cases except 1: serum cortisol concentrations were abnormally elevated in 5 cases (45.5%), and the remainder (5 cases, 45.5%) showed loss of diurnal variation. Dexamethasone suppression test was performed in 9 cases, and all (100%) showed no suppression. Preoperative radiologic studies included abdominal sonograms, CT or MRI scans, and angiography. Histology showed carcinomas to be bigger and heavier than adenomas, and microscopically carcinomas had necrosis, calcifications, and invasions of vessels. Distant metastases were found in 4 cases (12.7%). Adrenalectomy with complete surgical excision was performed in 12 cases. Long-term follow-up was possible in 10 patients after operation: 3 patients initially diagnosed with adenoma survived without tumor recurrence over a year, and among 6 carcinoma patients, 4 expired within a year, and 2 survived, with one patient currently undergoing postoperative chemotherapy. Of the 10 patients currently under going follow-up, one patient was initially diagnosed with a histologically unspecified tumor, and has survived 4 years after operation. CONCLUSIONS: When adrenocortical neoplasms are suspected by clinical symptoms and laboratory findings, abdominal ultrasonogram, CT or MRI scans must be performed immediately. Early detection and proper management are important for better prognosis, but are often delayed in the majority of cases. Ultimately, pediatricians need to be familiar with clinical characteristics and laboratory findings of adrenocortical tumors, bearing in mind the possibility of diagnosis in children.
17-Hydroxycorticosteroids ; 17-Ketosteroids ; Adenoma ; Adrenal Gland Neoplasms ; Adrenal Medulla ; Adrenalectomy ; Age Distribution ; Angiography ; Child ; Cushing Syndrome ; Dexamethasone ; Diagnosis ; Drug Therapy ; Early Diagnosis ; Female ; Follow-Up Studies ; Humans ; Hydrocortisone ; Hyperaldosteronism ; Magnetic Resonance Imaging ; Male ; Necrosis ; Neoplasm Metastasis ; Neuroblastoma ; Pheochromocytoma ; Prognosis ; Recurrence ; Sex Distribution ; Ultrasonography