Malignant Pheochromocytoma and Paraganglioma: 272 Patients Over 55 Years.
Oksana Hamidi,William F. Young,Nicole M. Iñiguez-Ariza,Nana Esi Kittah,Lucinda Gruber,Cristian Bancos,Shrikant Tamhane,Irina Bancos +7 more
TLDR
The clinical course of patients with malignant PPGL is remarkably variable and rapid disease progression is associated with male sex, older age at diagnosis, synchronous metastases, larger tumor size, elevated dopamine, and not undergoing resection of primary tumor.Abstract:
Context Malignant pheochromocytoma (PHEO) and paraganglioma (PGL) are rare and knowledge of the natural history is limited. Objective We aimed to describe baseline characteristics and outcomes of patients with malignant PHEO and PGL (PPGL) and to identify predictors of shorter survival. Design Retrospective review of patients with malignant PPGL evaluated from 1960 to 2016. Setting Referral center. Patients The group comprised 272 patients. Main outcome measures Baseline description, survival outcomes, and predictors of shorter survival were evaluated in patients with rapidly progressive (n = 29) and indolent disease (n = 188). Results Malignant PPGL was diagnosed at a median age of 39 years (range, 7 to 83 years), with synchronous metastases in 96 (35%) patients. In 176 (65%) patients, metastases developed at a median of 5.5 years (range, 0.3 to 53.4 years) from the initial diagnosis. Median follow-up was 8.2 years (range, 0.01 to 54.1 years). Median overall and disease-specific survivals were 24.6 and 33.7 years, respectively. Shorter survival correlated with male sex (P = 0.014), older age at the time of primary tumor (P = 0.0011), synchronous metastases (P Conclusions The clinical course of patients with malignant PPGL is remarkably variable. Rapid disease progression is associated with male sex, older age at diagnosis, synchronous metastases, larger tumor size, elevated dopamine, and not undergoing resection of primary tumor. An individualized approach to patients with metastatic PPGL is warranted.read more
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Journal ArticleDOI
Pheochromocytoma and Paraganglioma.
TL;DR: Pheochromocytoma and Paraganglioma are related tumors that differ mainly in location; pheochROMocytomas are adrenal, and paragang Liomas can be anywhere else.
Journal ArticleDOI
New Perspectives on Pheochromocytoma and Paraganglioma: Toward a Molecular Classification.
TL;DR: The current and future PPGL classifications are reviewed, with a focus on hereditary syndromes, and the current strengths and shortcomings of precision medicine are discussed.
Journal ArticleDOI
Neuroendocrine and adrenal tumors, version 2.2021
Manisha H. Shah,Whitney S. Goldner,Al B. Benson,Emily K. Bergsland,Lawrence S. Blaszkowsky,Pamela Brock,Jennifer A. Chan,Satya Das,Paxton V. Dickson,Paul T. Fanta,Thomas J. Giordano,Thorvardur R. Halfdanarson,Daniel M. Halperin,Jin He,Anthony P. Heaney,Martin J. Heslin,Fouad Kandeel,Arash Kardan,Sajid A. Khan,Boris W. Kuvshinoff,Christopher H. Lieu,Kimberly A. Miller,Venu G. Pillarisetty,Diane Lauren Reidy,Sarimar N Agosto Salgado,Shagufta Shaheen,Heloisa P. Soares,Michael C. Soulen,Jonathan R. Strosberg,Craig R. Sussman,Nikolaos A. Trikalinos,Nataliya Uboha,Namrata Vijayvergia,Terence Z. Wong,Beth Lynn,Cindy Hochstetler +35 more
TL;DR: The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Neuroendocrine and Adrenal Gland Tumors focus on the diagnosis, treatment, and management of patients with neuroendocrine tumors (NETs), adrenal tumors, pheochromocytomas, paragangliomas, and multiple endocrine neoplasia as mentioned in this paper.
Journal ArticleDOI
Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the Working Group on Endocrine Hypertension of the European Society of Hypertension.
Jacques W.M. Lenders,Jacques W.M. Lenders,Michiel N. Kerstens,Laurence Amar,Aleksander Prejbisz,Mercedes Robledo,David Taïeb,Karel Pacak,Joakim Crona,Tomáš Zelinka,Massimo Mannelli,Timo Deutschbein,Henri J L M Timmers,Frederic Castinetti,Henning Dralle,Jřri Widimský,Anne-Paule Gimenez-Roqueplo,Graeme Eisenhofer +17 more
TL;DR: The relevant genetic causes of PPGLs are addressed and how genetic testing can be incorporated within clinical care is outlined, and risk factors for developing metastatic disease and how they may facilitate personalised follow-up are discussed.
Journal ArticleDOI
Efficacy and Safety of High-Specific-Activity 131I-MIBG Therapy in Patients with Advanced Pheochromocytoma or Paraganglioma
Daniel A. Pryma,Bennett B. Chin,Richard B. Noto,Joseph S. Dillon,Stephanie M. Perkins,Lilja Solnes,Lale Kostakoglu,Aldo N. Serafini,Miguel Hernandez Pampaloni,Jessica Jensen,Thomas Armor,Tess Lin,Theresa White,Nancy Stambler,Stuart Apfel,Vincent A. DiPippo,Syed Mahmood,Vivien Wong,Camilo Jimenez +18 more
TL;DR: HSA 131I-MIBG offers multiple benefits, including sustained blood pressure control and tumor response in PPGL patients, according to a multicenter, phase 2 trial evaluated the efficacy and safety of high-specific-activity131I-meta-iodobenzylguanidine in patients with advanced PPGl.
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TL;DR: In this article, the differences in clinical features in carriers of SDHB mutations and SDHD mutations were determined in a population-based genetic screening for Paraganglioma syndromes type 4 and type 1 (PGL-1), respectively.
Journal ArticleDOI
Genetic Testing in Pheochromocytoma or Functional Paraganglioma
Laurence Amar,Jérôme Bertherat,Eric Baudin,Christiane Ajzenberg,Brigitte Bressac-de Paillerets,Olivier Chabre,Bernard Chamontin,Brigitte Delemer,Sophie Giraud,Arnaud Murat,Patricia Niccoli-Sire,Stéphane Richard,Vincent Rohmer,Jean-Louis Sadoul,Laurence Strompf,Martin Schlumberger,Xavier Bertagna,Pierre-François Plouin,Xavier Jeunemaitre,Anne-Paule Gimenez-Roqueplo +19 more
TL;DR: Genetic testing oriented by family/sporadic presentation should be proposed to all patients with pheo or functional pgl to allow the confirmation of suspected inherited disease as well as the diagnosis of unexpected inherited disease.
Journal ArticleDOI
Exome sequencing identifies MAX mutations as a cause of hereditary pheochromocytoma
Iñaki Comino-Méndez,Francisco Javier Gracia-Aznarez,Francesca Schiavi,Iñigo Landa,Luis J Leandro-García,Rocío Letón,Emiliano Honrado,Rocío Ramos-Medina,Daniela Caronia,Guillermo Pita,Álvaro Gómez-Graña,Aguirre A. de Cubas,Lucía Inglada-Pérez,Agnieszka Maliszewska,Elisa Taschin,Sara Bobisse,Giuseppe Pica,Paola Loli,Rafael Hernández-Lavado,José Ángel Díaz,Mercedes Gómez-Morales,Anna González-Neira,Giovanna Roncador,Cristina Rodríguez-Antona,Javier Benitez,Massimo Mannelli,Giuseppe Opocher,Mercedes Robledo,Alberto Cascón +28 more
TL;DR: The involvement of the MYC-MAX-MXD1 network in the development and progression of neural crest cell tumors is supported and the lack of functional MAX in rat PCC (PC12) cells is supported, which suggests that loss of MAX function is correlated with metastatic potential.
Journal ArticleDOI
Prevalence of Clinically Unsuspected Pheochromocytoma. Review of a 50-Year Autopsy Series
TL;DR: A review of 54 autopsy-proven cases of pheochromocytoma seen at the Mayo Clinic over the 50-year period 1928-1977 is presented in this paper.
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