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Malignant pheochromocytoma

Chrysanthi Aggeli, Alexander M Nixon, Christos Parianos, Georgios Vletsis, Labrini Papanastasiou, Theodora Kounadi, Georgrios Piaditis, Georgios N Zografos
OBJECTIVE: Resection of pheochromocytomas is a challenging procedure due to hemodynamic lability. Our aim was to evaluate surgical outcomes in 67 patients with pheochromocytoma and to validate the role of laparoscopic surgery in the treatment of these tumors. DESIGN: This study is a retrospective review. A total of 68 procedures for pheochromocytoma were performed between June 1997 and February 2017. All patients were investigated and operated on using an established departmental protocol...
October 2017: Hormones: International Journal of Endocrinology and Metabolism
F Oldhafer, K I Ringe, K Timrott, M Kleine, O Beetz, W Ramackers, S Cammann, J Klempnauer, F W R Vondran, H Bektas
PURPOSE: Treatment of malignancies invading the hepatic veins/inferior vena cava is a surgical challenge. An ante situm technique allows luxation of the liver in front of the situs to perform tumor resection. Usually, cold perfusion and veno-venous bypass are applied. Our experience with modified ante situm resection relying only on total vascular occlusion is reported. METHODS: Retrospective analysis on an almost 15-year experience with ante situm resection without application of cold perfusion or veno-venous bypass RESULTS: The ante situm technique was applied on eight patients...
February 22, 2018: Langenbeck's Archives of Surgery
Marie Marty, Delphine Gaye, Paul Perez, Claire Auder, Marie-Laure Nunes, Amandine Ferriere, Magalie Haissaguerre, Antoine Tabarin
CONTEXT: the recent recommendations of the European endocrine society states that the performance of computed tomography (CT) to characterize true adrenal incidentalomas (AIs) remains debatable. OBJECTIVE: to determine relevant thresholds for usual CT parameters for the diagnosis of benign tumors using robust reference standard amongst a large series of "true" AIs recruited in an endocrinological setting. DESIGN: retrospective study of 253 AIs in 233 consecutive patients explored in a single university hospital: 183 adenomas, 33 pheochromocytomas, 23 adrenocortical carcinomas, 5 other malignant tumors and 9 other benign tumors...
February 21, 2018: European Journal of Endocrinology
Rebeca Joya Vázquez, Cristina Vecino Bueno, José María Bengochea Cantos, Olga Gómez García, María Ángeles López López, Antonio Molina Sánchez, José Miguel Ruiz-Ayucar Imbert, Elizabeth Barrera Melgarejo
The giant cystic pheochromocytoma is a rare adrenal tumor in the predominantly asymptomatic course; so many cases are not diagnosed until the time of surgery. The simple mobilization of the tumor is associated with the passage to the blood of large amounts of catecholamines and high morbidity and mortality. So the surgery itself and perioperative management are a huge challenge. This article describes the case of a malignant giant pheochromocytoma (35 cm) which occupied the entire right abdomen. Even with the preoperative diagnosis of pheochromocytoma, pharmacological blockade preoperative and intraoperative measures, the patient died shortly before the end of surgery...
October 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
J Breza, J Breza
INTRODUCTION: In the MEN 2A syndrome, which is the most common of the three types of MEN, three endocrine systems are affected simultaneously or subsequently by the development of tumours manifested by medullary thyroid gland carcinoma, pheochromocytoma (often bilateral) and hyperparathyroidism. MATERIAL AND METHODS: 27 patients from 3 families affected by MEN 2A syndrome were examined clinically (by detecting the effects of catecholamine overproduction), biochemically (screening for metanephrine and normetanephrine in the serum), visualization (CT, MRI, MIBG, PET CT) and some of them also genetically (DNA fragment analysis obtained by PCR amplification)...
2018: Bratislavské Lekárske Listy
Fatemeh Khatami, Mahsa Mohammadamoli, Seyed Mohammad Tavangar
Pheochromocytomas and paragangliomas (PPGLs) are tumors arising from the adrenal medulla and sympathetic/parasympathetic paraganglia, respectively. According to Th e Cancer Genome Atlas (TCGA), approximately 40% of PPGLs are due to germ line mutations in one of 16 susceptibility genes, and a further 30% are due to somatic alterations in at least seven main genes (VHL, EPAS1, CSDE1, MAX, HRAS, NF1, RET, and possibly KIF1B). Th e diagnosis of malignant PPGL was straight forward in most cases as it was defined as presence of PPGL in non-chromaffin tissues...
January 1, 2018: Endocrine Regulations
Katarina Kluckova, Daniel A Tennant
Hypoxia is a critical driver of cancer pathogenesis, directly inducing malignant phenotypes such as epithelial-mesenchymal transition, stem cell-like characteristics and metabolic transformation. However, hypoxia-associated phenotypes are often observed in cancer in the absence of hypoxia, a phenotype known as pseudohypoxia, which is very well documented in specific tumour types, including in paraganglioma/pheochromocytoma (PPGL). Approximately 40% of the PPGL tumours carry a germ line mutation in one of a number of susceptibility genes of which those that are found in succinate dehydrogenase (SDH) or in von Hippel-Lindau (VHL) genes manifest a strong pseudohypoxic phenotype...
February 15, 2018: Cell and Tissue Research
L L Jiang, H H Wu, N Bu, J Q Zhang, L Q Zhou, X H Guo
Objective: To summarize the clinical characteristics of paraganglioma. Methods: A total of 215 pheochromocytoma/ paraganglioma cases in Peking University First Hospital between January 1996 and December 2014 were retrospectively analyzed. The patients were divided into two groups according to the tumor position: pheochromacytoma group (173 patients) and paraganglioma group (42 patients). Then the clinical characteristics between the groups were compared. Results: There were 42 paraganglioma cases in the study, accounting for 19...
January 23, 2018: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Joseph M Pappachan, Nyo Nyo Tun, Ganesan Arunagirinathan, Ravinder Sodi, Fahmy W F Hanna
PURPOSE OF REVIEW: Pheochromocytomas and paragangliomas (PPGLs) are uncommon catecholamine-producing neuroendocrine neoplasms that usually present with secondary hypertension. This review is to update the current knowledge about these neoplasms, the pathophysiology, genetic aspects and diagnostic and therapeutic algorithms based on scientific literature mostly within the past 3 years. RECENT FINDINGS: Eighty to eighty-five percent of PPGLs arise from the adrenal medulla (pheochromocytomas; PCCs) and the remainder from the autonomic neural ganglia (paragangliomas; PGLs)...
January 22, 2018: Current Hypertension Reports
Yasuhiro Ohshima, Hitomi Sudo, Shigeki Watanabe, Kotaro Nagatsu, Atsushi B Tsuji, Tetsuya Sakashita, Yoichi M Ito, Keiichiro Yoshinaga, Tatsuya Higashi, Noriko S Ishioka
PURPOSE: Therapeutic options for patients with malignant pheochromocytoma are currently limited, and therefore new treatment approaches are being sought. Targeted radionuclide therapy provides tumor-specific systemic treatments. The β-emitting radiopharmaceutical meta-131I-iodo-benzylguanidine (131I-MIBG) provides limited survival benefits and has adverse effects. A new generation of radionuclides for therapy using α-particles including meta-211At-astato-benzylguanidine (211At-MABG) are expected to have strong therapeutic effects with minimal side effects...
January 19, 2018: European Journal of Nuclear Medicine and Molecular Imaging
Guillaume Gravel, Sophie Leboulleux, Lambros Tselikas, Flora Fassio, Mohamed Berraf, Amandine Berdelou, Bakar Ba, Segolene Hescot, Julien Hadoux, Martin Schlumberger, Abir Al Ghuzlan, France Nguyen, Mathieu Faron, Thierry de Baere, Eric Baudin, Frederic Deschamps
PURPOSE: Bone metastases (BM) and skeletal-related events (SRE) are frequent in patients with malignant pheochromocytoma and paraganglioma (PPM) and the best modality of prevention unknown. The role of interventional radiology (IR) techniques for the prevention of SRE in the multidisciplinary management of malignant PPM has not been evaluated in that setting. METHODS: Single referral center retrospective review of all patients with malignant PPM with BM from 2000 to 2016...
January 5, 2018: Endocrine
P Meignan, Q Ballouhey, J Lejeune, K Braik, B Longis, A R Cook, H Lardy, L Fourcade, Aurélien Binet
Mini-invasive surgery is more and more integrated in pediatric surgery. The robotic-assisted surgery brought new advantages from which the patient and the surgeon could benefit compared to laparoscopy. Its use in oncological surgery is still controversial. 12 robotic-assisted tumor resections with the da Vinci Surgical Robot (Intuitive Surgical, Sunnyvale, CA) were attempted in 11 children (mean age 7.65 years; age range 0.75-16.75 years; mean weight 30.3 kg; weight range 8.6-62 kg) in two centers. Mean total operative time was 145 min (range 72-263 min)...
December 29, 2017: Journal of Robotic Surgery
Masahiro Shibata, Takahiro Inaishi, Noriyuki Miyajima, Yayoi Adachi, Yuko Takano, Kenichi Nakanishi, Dai Takeuchi, Sumiyo Noda, Yuichi Aita, Kazuhiro Takekoshi, Yasuhiro Kodera, Toyone Kikumori
BACKGROUND: Recent advance of genetic testing has contributed to the diagnosis of hereditary pheochromocytoma and paraganglioma (PPGL). The clinical characteristics of hereditary PPGL are varying among the types of mutational genes. It is still difficult to specify the pathognomonic symptoms in the case of rare genetic mutations. Here, we report the case of synchronous bilateral pheochromocytomas and paraganglioma with novel MYC associated factor X (MAX) gene mutation. CASE PRESENTATION: A 24-year-old female had hyperhidrosis and hypertension...
December 28, 2017: Surgical Case Reports
Doğuş Vurallı, Nurgün Kandemir, Graeme Clark, Diclehan Orhan, Ayfer Alikaşifoğlu, Nazlı Gönç, Saniye Ekinci, Alev Özön
Vurallı D, Kandemir N, Clark G, Orhan D, Alikaşifoğlu A, Gönç N, Ekinci S, Özön A. A pheochromocytoma case diagnosed as adrenal incidentaloma. Turk J Pediatr 2017; 59: 200-206. There are two problems that needs to be addressed in cases of an adrenal incidentaloma. The first is to decide whether the adrenal mass is benign or malignant, and the second is to determine whether the mass is hormonally active or not. A 17-year-old male was admitted with the complaint of progressive weight gain. Abdominal ultrasonography was performed for elevation in transaminases which revealed a hypoechoic mass located in the left adrenal gland...
2017: Turkish Journal of Pediatrics
Erdal Uysal, Türkay Kırdak, Ahmet Orhan Gürer, Mehmet Ali İkidağ
Pheochromocytoma is a rare tumor originating from the embryonic neural crest and secreting high levels of catecholamines. The average tumor size is approximately 7 cm, and the average weight is approximately 200 g in the previous publications. Sometimes these tumors may be bigger. In this report, a case of multicystic malignant pheochromocytoma with a huge size is presented, which is seldom reported in the past. A 37-year-old male patient was referred to our hospital for etiological investigation of his recently diagnosed hypertension...
2017: Turkish Journal of Surgery
Teruaki Sugino, Ryosuke Ando, Rei Unno, Keitaro Iida, Taku Naiki, Shuzo Hamamoto, Kentaro Mizuno, Atsushi Okada, Yukihiro Umemoto, Noriyasu Kawai, Keiichi Tozawa, Yutaro Hayashi, Anri Inaki, Daiki Kayano, Seigo Kinuya, Takahiro Yasui
BACKGROUND: Pheochromocytomas are rare neuroendocrine tumors, with a malignancy frequency of approximately 10%. The treatment of malignant pheochromocytoma is palliative, and the traditional management strategy has limited efficacy. Furthermore, no clear criteria exist for the treatment of metastatic pheochromocytoma, especially for unresectable lesions. We report a case of complete remission of metastatic pheochromocytoma in 123I-metaiodobenzylguanidine (MIBG) scintigraphy after a single session of 131I-MIBG therapy...
December 19, 2017: BMC Research Notes
Xia Gong, Yifei Yu, Weiwei Zhan
OBJECTIVES: To evaluate the features of adrenal masses on ultrasonography and correlate the findings with the pathologic diagnoses to help distinguish benign from malignant adrenal lesions. METHODS: Ultrasonography was performed in 1363 patients with adrenal lesions. The following ultrasonographic parameters were recorded: size, shape, margin, echogenicity, echo texture, cystic necrosis, calcifications, and blood supply. The sensitivity and specificity of aggressive features for predicting malignancy were calculated...
December 1, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
Maki Yokomoto-Umakoshi, Hironobu Umakoshi, Mika Tsuiki, Tatsuki Ogasawara, Kaho Hamanaka-Hiroshima, Akiyo Tanabe, Tetsuya Tagami, Akira Shimatsu, Mitsuhide Naruse
Malignant pheochromocytoma (PHEO) and paraganglioma (PGL) (PHEO and PGL: PPGL) are frequently associated with bone metastasis. Bone metastasis requires long-term management and may lead to skeletal-related events (SREs) that remarkably reduce patients' quality of life (QOL). The aim of this study was to elucidate the risk factors for developing bone metastasis in patients with PPGL. The medical records of 40 consecutive adult patients with malignant PPGL at the National Hospital Organization Kyoto Medical Center between 2006 and 2016 were reviewed...
November 29, 2017: Endocrine Journal
A Ferriere, V Kerlan, A Tabarin
The 2017 Endocrine Society annual meeting included several communications and debates on the conservative adrenal surgery in bilateral hereditary pheochromocytomas (BHP), bilateral adrenal macronodular hyperplasia (BAMH) and primary hyperaldosteronism (PHA). The general principle is to preserve a part of the adrenal cortex to prevent the occurrence of a definitive adrenal insufficiency. In BHP, cortical sparing surgery allows more than 50% of patients to maintain normal corticotropic function at 10 years with a low recurrence rate (~ 10%)...
October 2017: Annales D'endocrinologie
L L Li, L Zhao, J T Dou, G Q Yang, W J Gu, Z H Lü, J M Ba, Y M Mu, J M Lu, C Y Pan
Objective: To investigate the clinical value of tumor size in the evaluation of endocrinological and histological natures regarding adrenal incidentaloma (AI) patients. Methods: A total of 1 941 AI patients who were hospitalized in Department of Endocrinology, Chinese PLA General Hospital between January 1997 and December 2016 were retrospectively reviewed. The demographics of patients, imaging features, functional status and histological results were analyzed. Results: Of 1 941 patients, 984 (50.70%) were males, and 957 (49...
November 14, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
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