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Malignant pheochromocytoma

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https://www.readbyqxmd.com/read/28540670/open-adrenalectomy-in-the-era-of-laparoscopic-surgery-a-review
#1
REVIEW
Giovanni Taffurelli, Claudio Ricci, Riccardo Casadei, Saverio Selva, Francesco Minni
Open adrenalectomy still plays an important role in adrenal surgery. A review of the current literature has been carried out to discuss the main indication to open adrenalectomy with regards to the main adrenal pathologies. The authors underlined the role of open adrenalectomy on the basis of personal experience and a literature review. Indication to open adrenalectomy for adrenal cysts, myelolipomas, pheochromocytomas, metastases, adrenocortical carcinomas and tumour recurrences were analysed and discussed...
May 24, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28499761/cystadenoma-of-the-rete-testis-in-a-patient-with-von-hippel-lindau-disease
#2
Jacob Lewis, David Thiel, Gregory Broderick, Candice Bolan
Von-Hippel Lindau (VHL) disease is a rare, inherited multicystic disorder that is characterized by several benign and malignant neoplasms (1). Classically, the disease manifests itself in a broad spectrum including: renal cell carcinomas, intracranial and spinal hemangioblastomas, endolymphatic sac tumors, renal and pancreatic cysts, and pheochromocytomas. Another important, but commonly forgotten manifestation is the cystadenoma of the rete testis.
May 9, 2017: Urology
https://www.readbyqxmd.com/read/28497219/robot-assisted-adrenalectomy-indications-and-drawbacks
#3
C Nomine-Criqui, A Germain, A Ayav, L Bresler, L Brunaud
Adrenal tumors can vary from a benign adrenocortical adenoma with no hormonal secretion to a secretory adrenocortical malignancy (adrenocortical carcinoma) or a hormone-secreting tumor of the adrenal medulla (pheochromocytoma). Currently, laparoscopic adrenalectomy is regarded as the preferred surgical approach for the management of most adrenal surgical disorders, although there are no prospective randomized trials comparing this technique with open adrenalectomy. However, widespread adoption of robotic technology has positioned robotic adrenalectomy as an option in some medical centers...
May 12, 2017: Updates in Surgery
https://www.readbyqxmd.com/read/28491185/extra-adrenal-pheochromocytoma-at-the-organ-of-zuckerkandl-a-case-report-and-literature-review
#4
Tarana Gill, Kalie Adler, Alicia Schrader, Keyur Desai, Joshua Wermers, Nebiyu Beteselassie
Pheochromocytomas and paragangliomas are tumors that occur in characteristic locations and are commonly detected on imaging studies. A correct diagnosis is important because of differences in associated neoplasms, risk for malignancy, and need for genetic testing. In addition, associated complications, including death, can be avoided if appropriately recognized and treated. Here, we report a rare case of an extra-adrenal paraganglioma of the organ of Zuckerkandl.
June 2017: Radiology case reports
https://www.readbyqxmd.com/read/28477311/update-on-adrenal-tumours-in-2017-world-health-organization-who-of-endocrine-tumours
#5
REVIEW
Alfred King-Yin Lam
The fourth edition of the World Health Organization (WHO) classification of endocrine tumours contains substantial new findings for the adrenal tumours. The tumours are presented in two chapters labelled as "Tumours of the adrenal cortex" and "Tumours of the adrenal medulla and extra-adrenal paraganglia." Tumours of the adrenal cortex are classified as cortical carcinoma, cortical adenoma, sex cord stromal tumours, adenomatoid tumour, mesenchymal and stromal tumours (myelolipoma and schwannoma), haematological tumours, and secondary tumours...
May 6, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28477304/routine-genetic-screening-with-a-multi-gene-panel-in-patients-with-pheochromocytomas
#6
Emilia Sbardella, Treena Cranston, Andrea M Isidori, Brian Shine, Aparna Pal, Bahram Jafar-Mohammadi, Greg Sadler, Radu Mihai, Ashley B Grossman
PURPOSE: Several new gene mutations have been reported in recent years to be associated with a risk of familial pheochromocytoma. However, it is unclear as to whether extensive genetic testing is required in all patients. METHODS: The clinical data of consecutive patients operated for pheochromocytoma over a decade in a tertiary referral center were reviewed. Genetic screening was performed using a 10-gene panel: RET, VHL, SDHB, SDHD, SDHA, SDHC, SDHAF2, MAX, TMEM127 and FH...
May 5, 2017: Endocrine
https://www.readbyqxmd.com/read/28469506/genotype-phenotype-correlation-in-patients-with-germline-mutations-of-vhl-ret-sdhb-and-sdhd-genes-thai-experience
#7
Chutintorn Sriphrapradang, Kitjapong Choopun, Atchara Tunteeratum, Thanyachai Sura
Mutations in the VHL, RET, SDHB, and SDHD genes are responsible for von Hippel-Lindau (VHL) disease, multiple endocrine neoplasia type 2 (MEN2), and familial paraganglioma, respectively. However, genotype-phenotype correlation data are lacking in Southeast Asia. A retrospective medical chart review was performed on patients referred to the genetics service. We found 35 patients diagnosed with clinical syndromes (16 VHL, 9 MEN2, 9 paragangliomas, and 1 neurofibromatosis type 1). In patients with VHL, 5 known VHL mutations were identified: p...
2017: Clinical Medicine Insights. Endocrinology and Diabetes
https://www.readbyqxmd.com/read/28458869/an-unusual-cause-of-back-pain-a-case-of-large-nonfunctioning-retroperitoneal-paraganglioma-presented-as-a-large-cystic-lesion-a-case-report-and-review-of-literature
#8
Siu Yan Amy Kok, Chung Ying Leung, Ki Yau Chow
Pheochromocytoma arising from outside the adrenal glands is also called paraganglioma. When it occurs below the diaphragm, in the organ of Zuckerkandl or retroperitoneum, it is also called extra-adrenal pheochromocytoma. Paragangliomas are rare tumors which arise from neuroendocrine cells and extra-adrenal paragangliomas (EAPs) account for only 10-15% if all paragangliomas and may present incidentally as a symptomless mass. Typical triad of sweating, headache and fluctuating hypertension if not present makes preoperative diagnosis difficult...
April 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28458565/nvp-auy922-a-novel-hsp90-inhibitor-inhibits-the-progression-of-malignant-pheochromocytoma-in-vitro-and-in-vivo
#9
Jianpo Lian, Dengqiang Lin, Xing Xie, Yunze Xu, Lieyu Xu, Li Meng, Yu Zhu
PURPOSE: Malignant pheochromocytoma (PCC) is a rare tumor with a very poor prognosis and no effective treatments. The aim of this study was to assess the efficacy of a novel second-generation synthetic heat-shock protein 90 (HSP90) inhibitor, NVP-AUY922, to treat malignant PCC in vitro and in vivo. MATERIALS AND METHODS: Cell Counting Kit-8 (CCK-8) and Transwell assays were used to assess the effects of NVP-AUY922 on the proliferation and migration of the PCC cell line PC12...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28449504/malignant-pheochromocytoma-in-the-anterior-mediastinum-with-sternal-invasion-a-case-report
#10
Mingzhi Song, Kebin Sun, Tian Xia, Lei Zhou, Yangyang Li, Zhe Sun, Yuchi Zhang, Xianbin Zhang, Ran Sun, Bo Chen, Qingwei Tan
Pheochromocytomas are catecholamine-producing neuroendocrine tumors that usually occur in the adrenal medulla or sympathetic paraganglia. Anterior mediastinum involvement with pheochromocytoma is rare and may not present with typical symptoms. Its clinical manifestation may be unclear and a high index of suspicion is required for accurate diagnosis. We report a rare case of pheochromocytoma of the anterior mediastinum in a 51-year-old female. A painful hard mass on the sternum was the only clinical manifestation...
March 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28429074/ultrastructure-of-pheochromocytoma-undescribed-morphologic-features
#11
A Beiras Fernandez, A Kornberger, M Fraga, C F Vahl, A Beiras
We examined samples of human pheochromocytoma from 11 patients aged 30-70 years including one case of malignant pheochromocytoma with a view to identifying previously unreported ultrastructural details.We identified two types of nuclear inclusions consisting of irregularly shaped singular or multiple granulofibrillar formations with a typical concentric halo, on the one hand, and accumulations of egg-shaped structures consisting of granules and microfilaments, on the other. In some of the tumor cells, membrane-covered inclusions containing parallel laminar elements arranged in a paracrystalline, periodic fashion, or mega-mitrochondriae characterized by increased electrodensity of their matrix, and fibrillary material in the spaces between the cristae were present...
April 20, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/28413794/comparative-evaluation-of-iodine-131-metaiodobenzylguanidine-and-18-fluorodeoxyglucose-positron-emission-tomography-in-assessing-neural-crest-tumors-will-they-play-a-complementary-role
#12
Soumyakanti Kundu, Purushottam Kand, Sandip Basu
BACKGROUND: 18-Fluorodeoxyglucose positron emission tomography (FDG-PET) has established a role in the evaluation of several malignancies. However, its precise clinical role in the neural crest cell tumors continues to evolve. PURPOSE: The purpose of this study was to compare iodine-131 metaiodobenzylguanidine ((131)I-MIBG) and FDG-PET of head to head in patients with neural crest tumors both qualitatively and semiquantitatively and to determine their clinical utility in disease status evaluation and further management...
January 2017: South Asian Journal of Cancer
https://www.readbyqxmd.com/read/28396811/giant-cystic-pheochromocytoma-with-low-risk-of-malignancy-a-case-report-and-literature-review
#13
Ravi Maharaj, Sangeeta Parbhu, Wesley Ramcharan, Shanta Baijoo, Wesley Greaves, Dave Harnanan, Wayne A Warner
Giant pheochromocytomas are rare silent entities that do not present with the classical symptoms commonly seen in catecholamine-secreting tumors. In many cases they are accidentally discovered. The algorithm to diagnose a pheochromocytoma consists of biochemical evaluation and imaging of a retroperitoneal mass. The female patient in this case report presented with a palpable abdominal mass and was cured with surgical resection. She suffered no recurrence or complications on follow-up. The left retroperitoneal mass measured 27 × 18 × 12 cm and weighed 3,315 grams...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/28386672/malignancy-in-pheochromocytoma-or-paraganglioma-integrative-analysis-of-176-cases-in-tcga
#14
Yong Joon Suh, Ji-Young Choe, Hyo Jin Park
Methods of diagnosing malignant pheochromocytoma (PCC) or paraganglioma (PGL) are needed. However, there are no reliable histopathologic criteria to distinguish malignant PCC/PGLs. The recent genomic analysis of The Cancer Genome Atlas (TCGA) provides in-depth information enabling more accurate diagnosis of disease entities. Therefore, we investigated genomic expression differences and mutational differences of malignant PCC/PGLs with TCGA. As of December 2014, TCGA had acquired multigenomic analysis of 176 PCC/PGL samples...
June 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28384794/clinical-characterization-of-the-pheochromocytoma-and-paraganglioma-susceptibility-genes-sdha-tmem127-max-and-sdhaf2-for-gene-informed-prevention
#15
Birke Bausch, Francesca Schiavi, Ying Ni, Jenny Welander, Attila Patocs, Joanne Ngeow, Ulrich Wellner, Angelica Malinoc, Elisa Taschin, Giovanni Barbon, Virginia Lanza, Peter Söderkvist, Adam Stenman, Catharina Larsson, Fredrika Svahn, Jin-Lian Chen, Jessica Marquard, Merav Fraenkel, Martin A Walter, Mariola Peczkowska, Aleksander Prejbisz, Barbara Jarzab, Kornelia Hasse-Lazar, Stephan Petersenn, Lars C Moeller, Almuth Meyer, Nicole Reisch, Arnold Trupka, Christoph Brase, Matthias Galiano, Simon F Preuss, Pingling Kwok, Nikoletta Lendvai, Gani Berisha, Özer Makay, Carsten C Boedeker, Georges Weryha, Karoly Racz, Andrzej Januszewicz, Martin K Walz, Oliver Gimm, Giuseppe Opocher, Charis Eng, Hartmut P H Neumann
Importance: Effective cancer prevention is based on accurate molecular diagnosis and results of genetic family screening, genotype-informed risk assessment, and tailored strategies for early diagnosis. The expanding etiology for hereditary pheochromocytomas and paragangliomas has recently included SDHA, TMEM127, MAX, and SDHAF2 as susceptibility genes. Clinical management guidelines for patients with germline mutations in these 4 newly included genes are lacking. Objective: To study the clinical spectra and age-related penetrance of individuals with mutations in the SDHA, TMEM127, MAX, and SDHAF2 genes...
April 6, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28374498/utility-of-gata3-in-the-differential-diagnosis-of-pheochromocytoma
#16
Carmen M Perrino, Alex Ho, Christopher P Dall, Debra L Zynger
AIMS: GATA3 is a relatively new immunohistochemical marker which shows consistent nuclear expression in a variety of tumors, including breast and urothelial carcinoma. The staining pattern of GATA3 in adrenal lesions is not well established. We aim to describe the expression of GATA3 in adrenal tumors and determine if there is differential staining between pheochromocytoma and adrenal cortical carcinoma. METHODS AND RESULTS: A retrospective search was performed to identify 74 adrenal lesions which were immunohistochemically tested for GATA3 expression...
April 4, 2017: Histopathology
https://www.readbyqxmd.com/read/28337414/pheochromocytoma-with-negative-metanephrines-a-rarity-and-the-significance-of-dopamine-secreting-tumors
#17
Michael Bozin, Alastair Lamb, Lydia Johns Putra
We describe a case of a 25-year-old female with a dopamine secreting PPGL diagnosed retrospectively with biochemical analysis. This finding resulted in change in approach to investigation and management, given their important clinical implications. There are important differences in management of dopamine secreting PPGL compared to classical noradrenaline and adrenaline-secreting PPGL. This includes the risk of peri-operative cardiovascular collapse peri-operatively with alpha/beta blockade, risk of malignancy/recurrence, and associated genetic abnormalities...
May 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28331462/a-case-of-malignant-pheochromocytoma-presenting-7-years-after-the-initial-surgery
#18
Larsa Al-Omaishi, Jonathan Babin, Ralph L Corsetti
BACKGROUND: Pheochromocytoma (PHEO) is a rare tumor of the adrenal medulla and sympathetic ganglion that produces the catecholamines norepinephrine and epinephrine. Traditionally, approximately 10% of PHEOs were thought to be malignant, but recent developments in PHEO research have noted that specific genetic mutations are associated with higher risk of metastatic spread. CASE REPORT: We report the case of a 71-year-old female who presented with abdominal pain in September 2009 when she was 64 years old...
2017: Ochsner Journal
https://www.readbyqxmd.com/read/28327598/global-dna-methylation-analysis-identifies-two-discrete-clusters-of-pheochromocytoma-with-distinct-genomic-and-genetic-alterations
#19
Samuel Backman, Rajani Maharjan, Alberto Falk-Delgado, Joakim Crona, Kenko Cupisti, Peter Stålberg, Per Hellman, Peyman Björklund
Pheochromocytomas and paragangliomas (PPGLs) are rare and frequently heritable neural-crest derived tumours arising from the adrenal medulla or extra-adrenal chromaffin cells respectively. The majority of PPGL tumours are benign and do not recur with distant metastases. However, a sizeable fraction of these tumours secrete vasoactive catecholamines into the circulation causing a variety of symptoms including hypertension, palpitations and diaphoresis. The genetic landscape of PPGL has been well characterized and more than a dozen genes have been described as recurrently mutated...
March 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28291009/a-family-with-von-hippel-lindau-syndrome-the-findings-of-indium-111-somatostatin-receptor-scintigraphy-iodine-123-metaiodobenzylguanidine-scintigraphy-and-single-photon-emission-computerized-tomography
#20
Pelin Arıcan, Berna Okudan Tekin, Seniha Naldöken, Rıza Şefizade, Dilek Berker
Von Hippel-Lindau syndrome (VHLS) is an autosomal dominant hereditary familial disorder characterized by development of malignant and benign neoplasms. Differential diagnosis of the adrenal and pancreatic masses are difficult in patients with VHLS. Iodine-123 metaiodobenzylguanidine (I-123 MIBG) and indium-111 somatostatin receptor scintigraphies (In-111 SRS) have important roles in the differential diagnosis of adrenal and pancreatic masses in those patients. In this case report, we present the findings of I-123 MIBG single-photon emission computerized tomography (SPECT/CT) and In-111 SRS SPECT/CT in three members of a family with VHLS...
February 5, 2017: Molecular Imaging and Radionuclide Therapy
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