• The Korean Journal of Nuclear Medicine
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• v.24 no.2
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• pp.332-336
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• 1990

Pheochromocytoma occurs at all ages but is most common in young to midadult life. In adults 10 percent of pheochronocytoma are bilateral. And bilateral lesions are common in the familial syndromes; within Multiple Endocrine Neoplasia (MEN) kindreds over half with pheochromocytomas have bilateral lesions. We report a case of bilateral pheochromocytoma firstly detected by ultrasonogram. This 24-year-old male was normotensive and had symptoms of dilated cardiomyopathy and elevated VMA level. CT and MRI were performed to evaluate the tissue nature and associated abnormalities. $^{131}I-MIBG$ scan seems to be the imaging of choice in patients with suspected multicentric/bilateral or recurrent or metastatic pheochromocytomas.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.5
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• pp.1977-1980
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• 2015

Objective: The Von Hippel-Lindau syndrome (VHLD), an inherited neoplastic syndrome predisposing to central nervous system hemangioblastoma (CNS), pheochromocytoma (PCC), renal cell carcinoma(RCC), retinal hemangioma (RA) and renal cysts, is caused by mutations or deletions of the VHL tumor-suppressor gene. To assess VHL genotype-phenotype correlations with function of pVHL a gene mutation analysis of members in a Chinese family with non-syndromic PCCs and individuals with apparently sporadic pheochromocytoma (ASP) was performed. Materials and Methods: DNA samples of 20 members from the Chinese family with non-syndromic PCCs and 41 patients with ASP were analyzed by polymerase chain reaction and direct sequencing, confirmed by Taqman probe. Results: Three novel mutations (H125P, 623(^TTTGTtG) and R120T) were identified in the Chinese family and in 3 among 41 ASP patients. The mutations were all located in exon 2 of VHL gene encoding ${\beta}$-domain of pVHL. The tumor type in H125P carriers and R120T carriers was VHL type 2C. And 623(^TTTGTtG) carriers presented VHL type 2B or type 2C. Conclusions: VHL gene abnormalities were identified in the Chinese family with non-syndromic PCCs and patients with APS, resulting in dysfunction of pVHL. H125P and R120T could be associated with VHL type 2C, while 623(^TTTGTtG) might be linked with VHL type 2B or type 2C. Not only is the genetic analysis helpful for early diagnosis and treatment of patients with VHLD, it is also benefitial for research intoVHLD pathogenesis.

• Tuberculosis and Respiratory Diseases
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• v.70 no.2
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• pp.165-169
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• 2011

Pheochromocytomas are neuroendocrine tumors of chromaffin cell that originate in the paraganglia of the adrenal medulla. Approximately 10% of pheochromocytomas are found in the extra-adrenal paraganglia and are called paragangliomas. However, cases of middle mediastinal paragangliomas are very rare. In this case, the patient presented with a voice change and a headache. A middle mediastinal soft tissue mass with marked enhancement was detected on computed tomography of the chest. The 24-hour urine catecholamine level was markedly elevated. The middle mediastinal mass was biopsied via mediastinoscopy and the resulting immunohistochemical staining was compatible with a diagnosis of middle mediastinal paraganglioma. The mass was resected surgically and the symptoms were relieved.

• Journal of Chest Surgery
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• v.46 no.1
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• pp.88-91
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• 2013

A 36-year-old man visited Yeungnam University Hospital with a sudden onset of palpitation, headache, and was found to be hypertensive. Chest radiography showed a 6 cm sized mass lesion on the posterior mediastinum. A biochemical study showed elevated levels of catecholamines. An I-123 metaiodobenzylguanidine scan revealed a hot uptake lesion on the posterior mediastinum. The patient was prepared for surgery with ${\alpha}$ and ${\beta}$ blocking agents. Two months later, we removed the tumor successfully. A histological study proved that the resected tumor was mediastinal pheochromocytoma. Functional mediastinal pheochromocytomas are rare. Therefore, we reported the case with a literature review.

• The Korean Journal of Nuclear Medicine
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• v.26 no.2
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• pp.365-370
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• 1992

Pheochromocytomas are catecholamine producing tumors of neuroectoderrnal origin. Diagnosis of pheochromocytoma is significant due to potentially curable hypertension. But they have a significant associated morbidity due to uncontrolled hypertension and mortality since 10% are malignant. From Aug. 1989 to Jul. 1992, 12 patients of our institution had $^{131}I-MIBG$ scan during work up of suspected primary or recurrent pheochromocytoma. In our studies conclude that $^{131}I-MIBG$ scan is recommended as the initial localizing study of choice (especially for the detection of extraadrenal disease and postoperative recurrence) as a guide for CT and/or MR and specific functional confirmation of their findings.

• Kosin Medical Journal
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• v.33 no.3
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• pp.446-453
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• 2018

Pheochromocytomas might be sporadic or genetic. Genetic pheochromocytoma is associated with multiple endocrine neoplasia (MEN) type 2A, MEN type 2B, and von Hippel-Lindau (VHL) disease. RET mutations are identified in more than 90% of index cases of MEN2 and familial medullary thyroid cancer and in about 4-12% of apparent sporadic cases. Here, we report a 54-year-old man presenting with pheochromocytoma and renal cell carcinoma, who was identified as having a novel missense RET mutation.

• Nuclear Medicine and Molecular Imaging
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• v.42 no.sup1
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• pp.130-133
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• 2008

Adrenal tumors are increasingly detected by widespread use of anatomical imaging such as a, MRI, etc. For these adrenal tumors, differentiation between malignancy and benignancy is very important. In diagnostic assessment of adrenal tumor, $^{18}F-FDG$ PET and PET-CT have been reported to have high diagnostic performance, especially, very excellent performance in evaluation of adrenal metastasis in the oncologic patient. In cases of adrenal incidentalomas, $^{18}F-FDG$ PET or PET-CT is helpful if a or chemical-shift MRI is inconclusive. $^{18}F-FDG$ PET and PET-CT may be applied to the patients with MIBG-negative pheochromocytomas. In summary, $^{18}F-FDG$ PET and PET-CT are expected to be effective diagnostic tools in the management of adrenal tumor.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.4
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• pp.1751-1755
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• 2014

Background: In this study prognostic correlations of histopathologic parameters and the Ki-67 proliferation index and as well as the diagnostic value of immunohistochemical markers in pheochromocytomas were evaluated. Materials and Methods: A total of 22 patients diagnosed with a pheochromocytoma between 2000-2010 in Izmir Katip Celebi University Ataturk Training and Research Hospital were included. Diagnostic value of the PASS scoring system, and prognostic correlations of histopathologic parameters and Ki-67 proliferation index were investigated. SPSS for Windows 17.0 software was used for statistical analysis. Results: There was no statistically significant correlation between recurrence and clinicopathologic parameters or the PASS score (PASS>4). In addition, there were no statistically significant correlations between PASS score and clinicopathologic parameters, such as diameter (5 cm), weight (>100g), gender (female/male ratio) and age (25-45/45-55/>55). Besides, there were no significant correlation between diameter and clinicopathological parameters and also recurrence. However, there was a statistically significant correlation between Ki-67 proliferation index and capsule invasion (p=0.047). Conclusions: Some but not most of the findings in our study were concordant with the literature. To clarify relationships, investigations with standard scoring systems which are not affected by subjective factors and feature appropriate histopathological criteria should be made on larger study groups.

• Journal of Yeungnam Medical Science
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• v.40 no.4
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• pp.435-441
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• 2023

Pheochromocytomas and paragangliomas (PPGLs) may secrete hormones or bioactive neuropeptides such as interleukin-6 (IL-6), which can mask the clinical manifestations of catecholamine hypersecretion. We report the case of a patient with delayed diagnosis of paraganglioma due to the development of IL-6-mediated systemic inflammatory response syndrome (SIRS). A 58-year-old woman presented with dyspnea and flank pain accompanied by SIRS and acute cardiac, kidney, and liver injuries. A left paravertebral mass was incidentally observed on abdominal computed tomography (CT). Biochemical tests revealed increased 24-hour urinary metanephrine (2.12 mg/day), plasma norepinephrine (1,588 pg/mL), plasma normetanephrine (2.27 nmol/L), and IL-6 (16.5 pg/mL) levels. ¹⁸F-fluorodeoxyglucose (FDG) positron emission tomography/CT showed increased uptake of FDG in the left paravertebral mass without metastases. The patient was finally diagnosed with functional paraganglioma crisis. The precipitating factor was unclear, but phendimetrazine tartrate, a norepinephrine-dopamine release drug that the patient regularly took, might have stimulated the paraganglioma. The patient's body temperature and blood pressure were well controlled after alpha-blocker administration, and the retroperitoneal mass was surgically resected successfully. After surgery, the patient's inflammatory, cardiac, renal, and hepatic biomarkers and catecholamine levels improved. In conclusion, our report emphasizes the importance of IL-6-producing PPGLs in the differential diagnosis of SIRS.

• Journal of Yeungnam Medical Science
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• v.34 no.2
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• pp.247-253
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• 2017

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 $10.0{\times}9.5{\times}7.5cm$ 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.