131I-MIBG treatment in malignant pheochromocytoma: A case report

Author(s):  
Lucia Almeida ◽  
Helena Alves ◽  
Hugo Duarte
2013 ◽  
Vol 3 (6) ◽  
pp. 89 ◽  
Author(s):  
Cristina Torres Arcos ◽  
Virgilio Ruiz Luque ◽  
José Aguilar Luque ◽  
Pablo Martínez García ◽  
Antonia Brox Jiménez ◽  
...  

Malignant pheochromocytoma is a rare disease and surgical resectionis the only curative treatment. There are no definitive histologicalor cytological criteria of malignancy, as it is impossible todetermine this condition in the absence of advanced locoregionaldisease or metastases. We report a case of a patient with a giantretroperitoneal tumour, the second largest to be published, whichwas diagnosed as a malignant pheochromocytoma; it was treatedwith surgery. The literature is reviewed to evaluate tumour featuresand criteria to distinguish between benign and malignantpheochromocytomas.


1989 ◽  
Vol 78 (10) ◽  
pp. 1484-1485
Author(s):  
Toru IWAMA ◽  
Natsuko SATOU ◽  
Yuuji SATOU ◽  
Akira SHIBUYA ◽  
Yamao ADACHI ◽  
...  

2019 ◽  
Vol 72 (2) ◽  
pp. 251-253
Author(s):  
Mustafa Gürbüz ◽  
Neslihan Özyurt ◽  
Akın Fırat Kocaay ◽  
Çiğdem Soydal ◽  
Filiz Çay Şenler

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A159-A159
Author(s):  
Charles Ma ◽  
Noah Bloomgarden ◽  
Simona Stefan

Abstract Introduction: In the US, Pheochromocytoma/paraganglioma incidence is estimated to be 2–8 per 1 million people each year with around 100–200 of these cases being malignant. Malignant pheochromocytoma is defined by documented presence of metastases or evidence of extensive local invasion. There are certain genetic syndromes which are also associated with renal cell carcinoma, including SDHB mutations type 4, VHL disease and familial pheochromocytoma. These syndromes are important to recognize as they may signify a worse prognosis. Here, we describe a case of co-occurrence of malignant PC with renal cell carcinoma. Case Report: A 53-year-old Hispanic male with history of HTN and recently diagnosed metastatic pheochromocytoma was admitted for surgical debulking of the left retroperitoneal/adrenal and renal masses. Symptoms began five months prior after he presented with an ischemic stroke in the setting of labile hypertension. He was diagnosed with a 6.3 x 4.6 x 6.8 cm incidental left retroperitoneal mass and suspicious left renal mass on CT imaging but also noted several lytic bony lesions concerning for bone metastasis. A spinal biopsy was obtained which was consistent with a well-differentiated metastatic neuroendocrine tumor. Laboratory evaluation was notable for Chromogranin A level of 6959ng/mL (25–140). He was started on Lanreotide. Given persistently difficult to control HTN he underwent work up for secondary hypertension. Hormonal evaluation was notable for plasma free metanephrine of 534pg/mL (<57pg/mL), normetanephrine 6155pg/mL(<148pg/mL), and total metanephrine of 6689pg/mL (205pg/mL) consistent with metastatic Pheochromocytoma. After appropriate alpha blockade he underwent left adrenalectomy, nephrectomy and liver tumor microwave ablation. Pathology was consistent with an 8.7cm pheochromocytoma with extensive retroperitoneal soft tissue invasion and PASS score of 9 as well as a 3.6 cm renal cell (clear cell-papillary type) carcinoma. On follow up, Plasma metanephrine decreased significantly postoperatively to a free metanephrine of 28pg/ml, normetanephrine 1153pg/ml, and total metanephrine of 1181pg/mL. He was referred for genetic testing but unfortunately, he was readmitted one month later with cerebral hemorrhage and expired. Conclusion: Advancements in genetics have led to improved understanding of the molecular etiologies of pheochromocytomas. A number of genetic defects are associated with PC and RCC, including SDHB mutations type 4, VHL and familial pheochromocytoma. Our case underscores the high morbidity and mortality in patients with metastatic PC with RCC and perhaps the catastrophic outcomes in such patients. Assessing patient’s genetics in these cases is now the standard of care, however further research studies are warranted to better understand the significance of tumor genetics on prognosis and management.


2021 ◽  
Vol 12 ◽  
Author(s):  
Hideaki Kaneto ◽  
Shinji Kamei ◽  
Fuminori Tatsumi ◽  
Masashi Shimoda ◽  
Tomohiko Kimura ◽  
...  

IntroductionPheochromocytoma is a catecholamine-producing tumor in the adrenal medulla and is often accompanied by hypertension, hyperglycemia, hypermetabolism, headache, and hyperhidrosis, and it is classified as benign and malignant pheochromocytoma. In addition, persistent hypertension is often observed in subjects with malignant pheochromocytoma.Case PresentationA 52-year-old Japanese male was referred and hospitalized in our institution. He had a health check every year and no abnormalities had been pointed out. In addition, he had no past history of hypertension. In endocrinology markers, noradrenaline level was as high as 7,693 pg/ml, whereas adrenaline level was within normal range. Abdominal contrast-enhanced computed tomography revealed a 50-mm hyper-vascularized tumor with calcification in the right adrenal gland and multiple hyper-vascularized tumors in the liver. In 131I MIBG scintigraphy, there was high accumulation in the right adrenal gland and multiple accumulation in the liver and bone. In echocardiography, left ventricular ejection fraction was as low as 14.3%. In coronary angiography, however, there was no significant stenosis in the coronary arteries. Based on these findings, we finally diagnosed him as malignant pheochromocytoma accompanied by multiple liver and bone metastases and catecholamine cardiomyopathy. However, blood pressure was continuously within normal range without any anti-hypertensive drugs. Right adrenal tumor resection was performed together with left hepatic lobectomy and cholecystectomy. Furthermore, serum levels of vascular endothelial growth factor (VEGF) and parathyroid (PTH)-related protein were very high before the operation but they were markedly reduced after the operation.ConclusionsThis is the first report showing the time course of serum VEGF level in a subject with malignant pheochromocytoma, clearly showing that malignant pheochromocytoma actually secreted VEGF. In addition, this case report clearly shows that we should bear in mind once again that malignant pheochromocytoma is not necessarily accompanied by hypertension.


2021 ◽  
Vol 100 (3) ◽  
pp. 258-266
Author(s):  
D.T. Utalieva ◽  
◽  
S.B. Babakhanova ◽  
E.Yu. Ilyina ◽  
N.A. Andreeva ◽  
...  

In recent decades, there has been marked progress in understanding the biology of the most common extracranial solid tumor of childhood – neuroblastoma (NB), which led to a significant improvement in treatment outcomes due to stratification of patients into risk groups, intensification of treatment of patients with metastatic disease and the presence of unfavorable molecular genetic markers. Survivors who have received multimodal therapies, including chemotherapy, high-dose therapy and autologous peripheral stem-cell transplantation, 131I-metaiodobenzylguanidine (131I-MIBG) therapy, radiation therapy, and immunotherapy have a high risk of developing long-term side effects of treatment (LT SE). The study of the nature and frequency of LT SE after completion of therapy in patients with NB is important to ensure the quality of life and minimize severe health disorders. This article presents a literature review and description of a clinical case report of primary hypothyroidism in a high-risk patient with NB who received multicomponent treatment, including 131I-metaiodobenzylguanidine therapy due to the persistence of MIBG-positive foci after the induction chemotherapy.


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