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Malignant pheochromocytoma

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https://www.readbyqxmd.com/read/29157485/la-chirurgie-d%C3%A2-%C3%A3-pargne-surr%C3%A3-nalienne-du-cortex-%C3%A3-la-m%C3%A3-dulla-cortical-sparing-surgery-from-cortex-to-medulla
#1
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
https://www.readbyqxmd.com/read/29141379/-clinical-value-of-tumor-size-in-the-evaluation-of-adrenal-incidentalomas
#2
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]
https://www.readbyqxmd.com/read/29136674/familial-hyperparathyroidism-disorders-of-growth-and-secretion-in-hormone-secretory-tissue
#3
Stephen J Marx, Delmar Muniz Lourenco
Six syndromes of familial hyperparathyroidism are compared: 1) Familial hypocalciuric hypercalcemia (FHH) expresses primary hyperparathyroidism (PHPT) beginning at birth with lifelong hypercalcemia. There is nonsuppressed PTH secretion from outwardly normal parathyroid glands. It reflects germline heterozygous mutation in CASR, GNA11, or AP2S1. 2) Neonatal severe primary hyperparathyroidism is severest of the six syndromes. It requires urgent total parathyroidectomy in infancy. It usually reflects biallelic inactivation of the CASR...
November 2017: Hormone and Metabolic Research, Hormon- und Stoffwechselforschung, Hormones et Métabolisme
https://www.readbyqxmd.com/read/29130501/tert-promoter-hypermethylation-is-associated-with-metastatic-disease-in-abdominal-paraganglioma
#4
Fredrika Svahn, C Christofer Juhlin, Johan O Paulsson, Omid Fotouhi, Jan Zedenius, Catharina Larsson, Adam Stenman
Telomere maintenance, a hallmark of cancer for cell immortalization, is commonly achieved by telomerase activation through induction of the telomerase reverse transcriptase (TERT) gene. Pheochromocytomas (PCC) and abdominal paragangliomas (PGL) (together PPGL) are endocrine tumours for which TERT promoter mutations and telomerase activation have been previously reported [1]. Only 10-15% of PPGL metastasize, however in the absence of metastases, the identification of malignant disease is a diagnostic dilemma...
November 11, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/29127554/is-there-any-role-for-minimally-invasive-surgery-in-net
#5
REVIEW
M Thomaschewski, H Neeff, T Keck, H P H Neumann, T Strate, E von Dobschuetz
Neuroendocrine tumors (NET) represent the variability of almost benign lesions either secreting hormones occurring as a single lesion up to malignant lesions with metastatic potential. Treatment of NET is usually performed by surgical resection. Due to the rarity of NET, surgical treatment is mainly based on the experience and recommendations of experts and less on the basis of prospective randomized studies. In addition, the development and establishment of new surgical procedures is made more difficult by their rarity...
November 11, 2017: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/29099418/temozolomide-treatment-of-a-malignant-pheochromocytoma-and-an-unresectable-max-related-paraganglioma
#6
Alfonso M Ferrara, Giuseppe Lombardi, Ardi Pambuku, Domenico Meringolo, Roberta Bertorelle, Margherita Nardin, Francesca Schiavi, Maurizio Iacobone, Giuseppe Opocher, Vittorina Zagonel, Stefania Zovato
Pheochromocytomas (PCCs) and paragangliomas (PGLs) are neuroendocrine tumors with a strong genetic background. The mainstay of treatment for PCC/PGLs is surgery. However, for unresectable lesions, no curative treatment is currently available. Temozolomide (TMZ) has been shown to determine radiological and biochemical response in malignant PCC/PGLs. We report two cases of PCC/PGLs treated with TMZ. Case 1 is a 51-year-old man with local and distant recurrence (liver and bone metastases) of right adrenal PCC...
November 2, 2017: Anti-cancer Drugs
https://www.readbyqxmd.com/read/29095319/malignant-pheochromocytoma-with-multiple-vertebral-metastases-causing-acute-incomplete-paralysis-during-pregnancy-literature-review-with-one-case-report
#7
REVIEW
Shuzhong Liu, An Song, Xi Zhou, Xiangyi Kong, William A Li, Yipeng Wang, Yong Liu
RATIONALE: We present a rare case of malignant pheochromocytoma with thoracic metastases during pregnancy that presented with symptoms of myelopathy and was treated with circumferential decompression, stabilization, and radiation. The management of this unique case is not well documented. The clinical manifestations, imaging results, pathological characteristics, treatment and prognosis of the case were analyzed. PATIENT CONCERNS: A 26-year-old pregnant woman with a history of paroxysmal hypertension during the second trimester presented with lower extremity weakness, numbness, urinary incontinence, and back pain...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29094913/secondary-hypertension-discovering-the-underlying-cause
#8
Lesley Charles, Jean Triscott, Bonnie Dobbs
Most patients with hypertension have no clear etiology and are classified as having primary hypertension. However, 5% to 10% of these patients may have secondary hypertension, which indicates an underlying and potentially reversible cause. The prevalence and potential etiologies of secondary hypertension vary by age. The most common causes in children are renal parenchymal disease and coarctation of the aorta. In adults 65 years and older, atherosclerotic renal artery stenosis, renal failure, and hypothyroidism are common causes...
October 1, 2017: American Family Physician
https://www.readbyqxmd.com/read/29082061/forty-three-year-old-female-with-dopamine-secreting-pheochromocytoma-of-the-adrenal-gland
#9
Tyler Haden, Marcin Zuberek, Naveen Pokala
We report on a 43-year-old, asymptomatic female who presented with incidental finding of left adrenal mass. MRI gave concerns for possible pheochromocytoma but markers for pheochromocytoma were not elevated as expected. 24-hour urine dopamine levels (6988 μg/day) were significantly elevated. The patient successfully underwent robotic assisted radical left adrenalectomy and was diagnosed with a dopamine secreting pheochromocytoma. Pathology revealed increased malignant potential associated with the tumor...
2017: Case Reports in Urology
https://www.readbyqxmd.com/read/29081018/treatment-for-malignant-pheochromocytomas-and-paragangliomas-5%C3%A2-years-of-progress
#10
REVIEW
Paola Jimenez, Claudio Tatsui, Aaron Jessop, Sonali Thosani, Camilo Jimenez
PURPOSE OF REVIEW: The purpose of this manuscript is to review the progress in the field of therapeutics for malignant pheochromocytomas and sympathetic paraganglioma (MPPG) over the past 5 years. RECENT FINDINGS: The manuscript will describe the clinical predictors of survivorship and their influence on the first TNM staging classification for pheochromocytomas and sympathetic paragangliomas, the treatment of hormonal complications, and the rationale that supports the resection of the primary tumor and metastases in patients with otherwise incurable disease...
October 28, 2017: Current Oncology Reports
https://www.readbyqxmd.com/read/29067922/primary-adrenal-teratoma-an-unusual-tumor-challenges-in-diagnosis-and-surgical-management
#11
P Ramakant, C Rana, K R Singh, A Mishra
We report a rare case of a 25-year-old lady who presented with right hypochondriac pain associated with weakness for 3 months. Her hormonal evaluation was normal. Computed tomography scan revealed an adrenal mass for which a right open transperitoneal adrenalectomy was performed, and the lesion was diagnosed as mature teratoma on histopathological examination. Teratoma is an unusual neoplasm which arises from one or all the three germ layers. Extragonadal teratomas are rare in adults as compared to children and are mostly retroperitoneal in location, constituting only 4% of all primary teratomas...
October 23, 2017: Journal of Postgraduate Medicine
https://www.readbyqxmd.com/read/29067245/dna-methylation-analysis-in-malignant-pheochromocytoma-and-paraganglioma
#12
Toshihiro Oishi, Kazumi Iino, Yuta Okawa, Keisuke Kakizawa, Shoko Matsunari, Miho Yamashita, Terumi Taniguchi, Masato Maekawa, Takafumi Suda, Yutaka Oki
AIMS: In recent years, aberrant DNA methylation of specific CpG sites has been detected in many types of malignant tumors, and the epigenetic regulation of promoter CpG sites is considered an important mechanism underlying carcinogenesis. This study aimed to establish the epigenetics of the malignant transformation of malignant pheochromocytoma (PCC) and paraganglioma (PGL) by performing a methylation analysis. MATERIALS AND METHODS: Based on the results of the Infinium HumanMethylation450 BeadChip array using DNA samples of PCC/PGL patients, candidate CpG sites that were hyper/hypo-methylated in metastatic tumors relative to those in the primary tumors of 2 patients with malignant PCC/PGL were selected...
March 2017: Journal of Clinical & Translational Endocrinology
https://www.readbyqxmd.com/read/29048679/enhanced-wild-type-p53-expression-by-small-activating-rna-dsp53-285-induces-cell-cycle-arrest-and-apoptosis-in-pheochromocytoma-cell-line-pc12
#13
Dengqiang Lin, Li Meng, Feifei Xu, Jianpo Lian, Yunze Xu, Xin Xie, Xiaojing Wang, Hongchao He, Chenghe Wang, Yu Zhu
Malignant pheochromocytoma (PHEO) is diagnosed only when metastasis has occurred, making it less likely for patients to obtain the benefits of traditional chemotherapy. Anti-oncogene TP53 mutation has been detected in PHEO and is possibly related to disease progression. However, whether the upregulation of wild-type TP53 has antitumoral effects on PHEO remains completely unknown. In the present study, we used RNA activation (RNAa) technique to upregulate the expression of wild-type TP53 by transfecting synthetic dsP53‑285 into PHEO cell line PC12...
September 25, 2017: Oncology Reports
https://www.readbyqxmd.com/read/29019745/imaging-of-nonmalignant-adrenal-lesions-in-children
#14
Kiran M Sargar, Geetika Khanna, Rebecca Hulett Bowling
The adrenal glands in children can be affected by a variety of benign lesions. The diagnosis of adrenal lesions can be challenging, but assessment of morphologic changes in correlation with the clinical presentation can lead to an accurate diagnosis. These lesions can be classified by their cause: congenital (eg, discoid adrenal gland, horseshoe adrenal gland, and epithelial cysts), vascular and/or traumatic (eg, adrenal hemorrhage), infectious (eg, granulomatous diseases), enzyme deficiency disorders (eg, congenital adrenal hyperplasia [CAH] and Wolman disease), benign neoplasms (eg, pheochromocytomas, ganglioneuromas, adrenal adenomas, and myelolipomas), and adrenal mass mimics (eg, extralobar sequestration and extramedullary hematopoiesis)...
October 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28981236/-adrenal-disease-diagnosis-management-of-adrenal-incidentaloma
#15
A S Azzi, N Driessens
Adrenal glands are specialized in biosynthesis of several hormones correlated to different clinical phenotypes in case of excess or lack of production. In addition to secretion disorders, tumors, secreting or not, can take place in adrenal glands. Incidentalomas are the most common adrenal diseases in clinical practice. The challenge of the management is to determine whether the lesion is benign or malignant and secreting or not in order to direct therapeutic management towards surgical option, pharmacotherapy or clinical follow-up...
2017: Revue Médicale de Bruxelles
https://www.readbyqxmd.com/read/28979675/apogossypolone-apog2-induces-ros-dependent-apoptosis-and-reduces-invasiveness-of-pc12-cells-in-vitro-and-in-vivo
#16
Dengqiang Lin, Xiaoxia Li, Lieyu Xu, Jianpo Lian, Yunze Xu, Li Meng, Xin Xie, Xiaojing Wang, Hongchao He, Danfeng Xu, Chenghe Wang, Yu Zhu
Malignant pheochromocytoma is accurately diagnosed only at occurrence of metastatic foci. However, at that time, patients are less likely to get many benefits from traditional chemotherapy. Over-expression of BCL-2 family proteins is tightly correlated with progression of pheochromocytoma. ApoG2, as the most potent gossypol derivative, has exhibited anti-tumor activities in various tumors. In the present study, we found that the staining degree of Bcl-2 being stronger than Bax was more frequently observed in pheochromocytoma than adrenocorticohyperplasia, which was possibly related to shorter overall survival...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28977615/proteasome-inhibitors-a-potential-medical-therapy-for-malignant-pheochromocytoma
#17
Run Yu
No abstract text is available yet for this article.
October 1, 2017: Endocrinology
https://www.readbyqxmd.com/read/28974878/recurrent-malignant-pheochromocytoma-with-lymph-nodal-metastasis-in-a-child-a-rare-case
#18
Jayesh Mittal, Ramanitharan Manikandan, Lalgudi Narayanan Dorairajan, Pampa Ch Toi
Malignant pheochromocytoma in children manifesting as local recurrence with multiple lymph nodal metastasis is a rare entity. We report a case of a 14-year-old child with recurrent sporadic malignant pheochromocytoma presenting 8 years after primary surgery with retroperitoneal lymphadenopathy. The child underwent excision of the tumor along with retroperitoneal lymphadenectomy. Histopathology confirmed pheochromocytoma with extensive lymph node metastasis. The patient has no signs of disease recurrence till date...
October 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28974544/tert-structural-rearrangements-in-metastatic-pheochromocytomas
#19
Trisha Dwight, Aidan Flynn, Kaushalya Amarasinghe, Diana E Benn, Richard Lupat, Jason Li, Daniel Cameron, Annette Hogg, Shiva Balachander, Ida Lm Candiloro, Stephen Wong, Bruce G Robinson, Anthony T Papenfuss, Anthony J Gill, Alexander Dobrovic, Rodney J Hicks, Roderick Clifton-Bligh, Richard William Tothill
Pheochromocytomas (PC) and paragangliomas (PGL) are endocrine tumors for which the genetic and clinico-pathological features of metastatic progression remain incompletely understood. As a result, the risk of metastasis from a primary tumor cannot be predicted. Early diagnosis of individuals at high risk of developing metastases is clinically important and the identification of new biomarkers that are predictive of metastatic potential is of high value. Activation of TERT has been associated with a number of malignant tumors, including PC/PGL...
October 3, 2017: Endocrine-related Cancer
https://www.readbyqxmd.com/read/28954983/a-phase-i-clinical-trial-for-131-i-meta-iodobenzylguanidine-therapy-in-patients-with-refractory-pheochromocytoma-and-paraganglioma-a-study-protocol
#20
Anri Inaki, Kenichi Yoshimura, Toshinori Murayama, Yasuhito Imai, Yoshikazu Kuribayashi, Tetsuya Higuchi, Megumi Jinguji, Tohru Shiga, Seigo Kinuya
Objective Pheochromocytoma and paraganglioma (PPGLs) are rare neuroendocrine tumors derived from the adrenal medulla or extra-adrenal paraganglioma from extra-adrenal chromaffin tissue. Although malignant PPGLs has miserable prognosis, the treatment strategy remains to be established. An internal radiation therapy using [(131)I]meta-iodobenzylguanidine ((131)I-mIBG) called MIBG therapy has been attempted as one of the systemic treatment of malignant PPGLs. The aim of this study is therefore to evaluate the safety and the efficacy of MIBG therapy for refractory PPGLs...
2017: Journal of Medical Investigation: JMI
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