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Malignant pheochromocytoma

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https://www.readbyqxmd.com/read/28337414/pheochromocytoma-with-negative-metanephrines-a-rarity-and-the-significance-of-dopamine-secreting-tumors
#1
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
#2
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
#3
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
#4
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
https://www.readbyqxmd.com/read/28264974/emerging-role-of-dopamine-in-neovascularization-of-pheochromocytoma-and-paraganglioma
#5
REVIEW
Thamara E Osinga, Thera P Links, Robin P F Dullaart, Karel Pacak, Anouk N A van der Horst-Schrivers, Michiel N Kerstens, Ido P Kema
Dopamine is a catecholamine that acts both as a neurotransmitter and as a hormone, exerting its functions via dopamine (DA) receptors that are present in a broad variety of organs and cells throughout the body. In the circulation, DA is primarily stored in and transported by blood platelets. Recently, the important contribution of DA in the regulation of angiogenesis has been recognized. In vitro and in vivo studies have shown that DA inhibits angiogenesis through activation of the DA receptor type 2. Overproduction of catecholamines is the biochemical hallmark of pheochromocytoma (PCC) and paraganglioma (PGL)...
March 6, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28255146/large-adrenal-leiomyoma-presented-as-adrenal-incidentaloma-in-an-aids-patient-a-rare-entity
#6
T J Huei, H T Lip, M S Rahman, A Sarojah
The literature on adrenal gland tumour in HIV-infected patients is scarce. We report a 46-year-old Malay man with HIV and Hepatitis C infection presenting with a large nonfunctioning adrenal tumour. Computed tomography showed a large right adrenal tumour with heterogeneous enhancement and central necrosis. A high index of suspicion of a malignant tumour or pheochromocytoma led us to surgical removal of the adrenal gland. In this case report, we highlight important features to look for during pre-op evaluation of a large adrenal mass...
February 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28229225/implications-of-sdhb-genetic-testing-in-patients-with-sporadic-pheochromocytoma
#7
Aurelie Maignan, Carole Guerin, Valentin Julliard, Nunzia-Cinzia Paladino, Edward Kim, Philippe Roche, Fréderic Castinetti, Wassim Essamet, Julien Mancini, Alessio Imperiale, Roderick Clifton-Bligh, Pauline Romanet, Anne Barlier, Karel Pacak, Fréderic Sebag, David Taïeb
PURPOSE: Succinate dehydrogenase B (SDHB) associated pheochromocytomas (PHEOs) are associated with a higher risk of tumor aggressiveness and malignancy. The aim of the present study was to evaluate (1) the frequency of germline SDHB mutations in apparently sporadic patients with PHEO who undergo preoperative genetic testing and (2) the ability to predict pathogenic mutations. METHODS: From 2012 to 2016, 82 patients underwent a PHEO surgical resection. Sixteen were operated in the context of hereditary PHEO and were excluded from analysis...
February 22, 2017: Langenbeck's Archives of Surgery
https://www.readbyqxmd.com/read/28225653/update-on-ct-and-mri-of-adrenal-nodules
#8
Nicola Schieda, Evan S Siegelman
OBJECTIVE: The objective of this article is to review the current role of CT and MRI for the characterization of adrenal nodules. CONCLUSION: Unenhanced CT and chemical-shift MRI have high specificity for lipid-rich adenomas. Dual-energy CT provides comparable to slightly lower sensitivity for the diagnosis of lipid-rich adenomas but may improve characterization of lipid-poor adenomas. Nonadenomas containing intracellular lipid pose an imaging challenge; however, nonadenomas that contain lipid may be potentially diagnosed using other imaging features...
February 22, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28199934/mixed-corticomedullary-adrenal-carcinoma-case-report-comparison-in-features-treatment-and-prognosis-with-the-other-two-reported-cases
#9
Mhd Belal Alsabek, Riad Alhmaidi, Bader Ghazzawi, Ghiath Hamed, Alhadi Alseoudi
INTRODUCTION: Adrenal corticomedullary adenoma was reviewed in many cases in PubMed Library, While the coincidence corticomedullary adrenal carcinoma in the same gland was just described in two cases in the medical literature. Our case is the third to be reported and was treated with surgery and adjuvant chemotherapy and followed for two years. PRESENTATION OF CASE: A 50-year-old man suffered from a mass effect in the left abdominal side. While the laboratory showed a mild elevation in the levels of both serum cortisol and 24h urine cortisol, radiological images were highly suggested an adrenal malignant tumor without metastasis...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28197025/current-perioperative-management-of-pheochromocytomas
#10
REVIEW
Rashmi Ramachandran, Vimi Rewari
Neuroendocrine tumors which have the potential to secrete catecholamines are either associated with sympathetic adrenal (pheochromocytoma) or nonadrenal (paraganglioma) tissue. Surgical removal of these tumors is always indicated to cure and prevent cardiovascular and other organ system complications associated with catecholamine excess. Some of these tumors have malignant potential as well. The diagnosis, localization and anatomical delineation of these tumors involve measurement of catecholamines and their metabolic end products in plasma and urine, (123)I-metaiodobenzylguanidine scintigraphy, computed tomography, and/or magnetic resonance imaging...
January 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28196587/pheochromocytomas-and-paragangliomas-genetically-diverse-and-minimalist-all-at-once
#11
Patricia L M Dahia
Pheochromocytomas and paragangliomas are infrequent, genetically heterogeneous neuroendocrine tumors. In this issue of Cancer Cell, Fishbein et al. report novel driver genes, pathogenic mechanisms, and markers of malignancy. Notably, the new findings support the long-held view that these tumors distinctively arise from a single driver event, either inherited or acquired.
February 13, 2017: Cancer Cell
https://www.readbyqxmd.com/read/28187001/molecular-markers-of-paragangliomas-pheochromocytomas
#12
REVIEW
Svetlana O Zhikrivetskaya, Anastasiya V Snezhkina, Andrew R Zaretsky, Boris Y Alekseev, Anatoly V Pokrovsky, Alexander L Golovyuk, Nataliya V Melnikova, Oleg A Stepanov, Dmitry V Kalinin, Alexey A Moskalev, George S Krasnov, Alexey A Dmitriev, Anna V Kudryavtseva
Paragangliomas/pheochromocytomas comprise rare tumors that arise from the extra-adrenal paraganglia, with an incidence of about 2 to 8 per million people each year. Approximately 40% of cases are due to genetic mutations in at least one out of more than 30 causative genes. About 25-30% of pheochromocytomas/paragangliomas develop under the conditions of a hereditary tumor syndrome a third of which are caused by mutations in the VHL gene. Together, the gene mutations in this disorder have implicated multiple processes including signaling pathways, translation initiation, hypoxia regulation, protein synthesis, differentiation, survival, proliferation, and cell growth...
February 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28181818/recommendations-for-the-management-of-adrenal-incidentalomas-what-is-pertinent-for-radiologists
#13
Anju Sahdev
Adrenal incidentalomas are unsuspected, asymptomatic adrenal masses detected on imaging. Most are non-functioning benign adrenocortical adenomas but can represent other benign lesions or lesions requiring therapeutic intervention including adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma or metastasis. This review summarizes and highlights radiological recommendations within the recently issued guidelines for the management of adrenal incidentalomas from the European Society of Endocrinology Clinical Practice in collaboration with the European Network for Study of Adrenal Tumours...
April 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28134695/multiple-endocrine-neoplasia-type-2b-unmasked-by-18f-fdg-pet-ct-and-131i-mibg-spect-ct
#14
Xun Sun, Maher Mohamad Rajab Arnous, Xiaoli Lan
F-FDG PET/CT was performed to detect an occult malignancy in a 26-year-old woman with complicated medical history which included paroxysmal hypertension and significantly elevated tumor marker. The images revealed lesions in the thyroid, lymph nodes, and bilateral adrenal glands. Further I-MIBG SPECT/CT revealed intense activity in the lesion in the left adrenal gland, which was consistent with pheochromocytoma. The pathology examination after subsequent neck biopsy demonstrated medullary thyroid carcinoma...
January 27, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28121933/successful-treatment-of-metastatic-pheochromocytoma-in-the-spine-with-cement-augmentation
#15
Siyi Cai, Xiangyi Kong, Chengrui Yan, Yong Liu, Xi Zhou, Guixing Qiu
Metastatic pheochromocytoma in the spine is rare, and there is no standard curative management. Treatment via open surgery is often risky in the perioperative period, while osteoplasty by cement augmentation is a less invasive option.We describe 2 patients with recurrence of pheochromocytoma involving the spine and the pelvis who were successfully treated with osteoplasty by cement augmentation. A 31-year-old female underwent cement augmentation for a pelvic lesion 6 months after the resection and screw fixation of an L3 lesion...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28108930/controlling-tumor-progression-with-cyclophosphamide-vincristine-and-dacarbazine-treatment-improves-survival-in-patients-with-metastatic-and-unresectable-malignant-pheochromocytomas-paragangliomas
#16
Shiko Asai, Takuyuki Katabami, Mika Tsuiki, Yasushi Tanaka, Mitsuhide Naruse
Evidence has not been established to support that combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) improves survival in patients with malignant pheochromocytoma and paraganglioma (M-PPGL). To investigate the efficacy of CVD for this disease, we retrospectively analyzed data of 23 patients with metastatic and unresectable M-PPGL (mean age, 41.7 ± 15.4 years) who received at least 2 cycles of this regimen. The follow-up period after initiation of CVD ranged from 0.3 to 13...
January 20, 2017: Hormones & Cancer
https://www.readbyqxmd.com/read/28081522/adrenal-giant-cystic-pheochromocytoma-treated-by-posterior-retroperitoneoscopic-adrenalectomy
#17
Carlos E Costa Almeida, Marta Silva, Luis Carvalho, Carlos Manuel Costa Almeida
INTRODUCTION: Giant cystic pheochromocytoma (>10cm) is rare with only a few cases described in the literature. Preoperative diagnosis is very difficult because clinical, biochemical and radiologic finds are usually not consistent with a pheochromocytoma. Open surgery is traditionally the gold standard. CASE PRESENTATION: A 51 year-old male patient resorted to surgery department with an adrenal cystic incidentaloma. He was asymptomatic, mild hypertension easily controlled, with increased plasma fractionated metanephrines...
December 27, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28063406/contemporary-imaging-of-incidentally-discovered-adrenal-masses
#18
REVIEW
Daniel J Wale, Ka Kit Wong, Benjamin L Viglianti, Domenico Rubello, Milton D Gross
Adrenal lesions are routinely encountered incidentally in clinical practice. Although most of these lesions are benign, malignancy needs to be excluded. Therefore, the initial clinical workup is to exclude aggressive characteristics suggesting malignancy and to identify characteristics predictive of the most common benign lesion, an adrenal adenoma. Predicting a benign adenoma using a variety of imaging modalities has been widely studied using unenhanced computed tomography (CT), contrast enhanced CT, and magnetic resonance (MR) imaging...
March 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28058556/surgical-treatment-of-malignant-pheochromocytoma-and-paraganglioma-retrospective-case-series
#19
Veljko Strajina, Benzon M Dy, David R Farley, Melanie L Richards, Travis J McKenzie, Keith C Bible, Florencia G Que, David M Nagorney, William F Young, Geoffrey B Thompson
INTRODUCTION: Pheochromocytoma and paraganglioma (PPGL) are rare neoplasms; about 10% are malignant. Literature regarding possible benefit from resection is extremely limited. METHODS: A 20 year review of all patients undergoing surgery for malignant PPGL at the Mayo Clinic Rochester Campus between 1994 and June 2014 was performed. RESULTS: We identified 34 patients undergoing surgery for malignant PPGL. Median follow up was 6 and 5 years survival was 90% (median 11 years)...
January 5, 2017: Annals of Surgical Oncology
https://www.readbyqxmd.com/read/28006088/case-of-parotid-mucoepidermoid-carcinoma-expanding-the-spectrum-of-von-hippel-lindau-related-neoplasms
#20
Michael H Berger, Darcy A Kerr, Artur E Rangel Filho, Zoukaa B Sargi
BACKGROUND: von Hippel-Lindau (VHL)-related tumors occurring outside the spectrum of VHL-defining tumors are rare, and mucoepidermoid carcinoma (MEC) in the setting of VHL disease has not been described. METHODS AND RESULTS: We describe a patient with confirmed VHL mutation who presented with a parotid mass and a history of 2 central nervous system (CNS) hemangioblastomas and 1 pheochromocytoma. Fine-needle aspiration (FNA) of the mass suggested a benign Warthin tumor...
December 22, 2016: Head & Neck
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