Risk Stratification on Pheochromocytoma and Paraganglioma from Laboratory and Clinical Medicine

Journal of Clinical Medicine
Noriko KimuraMitsuhide Naruse

Abstract

Pheochromocytoma (PCC) and sympathetic paraganglioma (PGL) are rare neuroendocrine tumors characterized by catecholamine production in the adrenal medulla and extra-adrenal paraganglia. PCC and PGL (PPGL) with metastasis was termed malignant PPGL. However, the distinction between "benign" and "malignant" PPGLs has been debated. Currently, all PPGLs are believed to have some metastatic potential and are assigned malignant tumors (ICD-O/3) by the WHO Classification of Endocrine Organs (2017, 4th edition). Therefore, the previous categories benign and malignant PPGL have been eliminated in favor of risk stratification approach. The Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) is a tool for risk stratification for predicting metastasis and the prognosis of patients. At least 30% of PPGLs are hereditary, with 20 genes identified and genotype-phenotype correlations clarified. Of these genes, VHL, RET and NF1 have been well investigated and are the primary cause of bilateral PCC. In addition, mutation of succinate dehydrogenase gene subunits SDHB and SDHD are strongly correlated with extra-adrenal location, younger age, multiple tumors, metastasis and poor prognosis. Disease stratification by catecholamine phenotype an...Continue Reading

References

Jan 1, 1992·Virchows Archiv. A, Pathological Anatomy and Histopathology·N KimuraH Nagura
May 10, 2002·The New England Journal of Medicine·Hartmut P H NeumannUNKNOWN Freiburg-Warsaw-Columbus Pheochromocytoma Study Group
Jun 29, 2002·European Journal of Endocrinology·E van der HarstF Boomsma
Aug 17, 2005·PLoS Genetics·Patricia L M DahiaCharles D Stiles
Sep 7, 2006·The Journal of Clinical Endocrinology and Metabolism·Patrick J PollardEamonn R Maher
May 3, 2008·Canadian Journal of Anaesthesia = Journal Canadien D'anesthésie·Geoff A BellinghamPatrick P Luke
Oct 9, 2008·The Journal of Clinical Endocrinology and Metabolism·Henri J L M TimmersGraeme Eisenhofer
Jan 16, 2009·The American Journal of Surgical Pathology·David WuVânia Nosé
Sep 28, 2010·American Journal of Surgery·Avital Harari, William B Inabnet
Jun 21, 2011·Nature Genetics·Iñaki Comino-MéndezAlberto Cascón
Jul 15, 2011·The Journal of Clinical Endocrinology and Metabolism·Esther KorpershoekRonald R de Krijger
Nov 1, 2011·Endocrine·Tim I M Korevaar, Ashley B Grossman
Dec 21, 2011·Endocrine Pathology·Judith FavierAnne-Paule Gimenez-Roqueplo
Feb 14, 2012·Hormone and Metabolic Research = Hormon- Und Stoffwechselforschung = Hormones Et Métabolisme·A-P Gimenez-RoqueploM Robledo
Mar 7, 2012·Endocrine Pathology·Svenja Nölting, Ashley B Grossman
Aug 13, 2013·Molecular and Cellular Endocrinology·Kathryn S King, Karel Pacak
Jan 21, 2014·Nature Reviews. Cancer·Patricia L M Dahia
Feb 14, 2014·Endocrine-related Cancer·Noriko KimuraMitsuhide Naruse
Mar 25, 2014·Endocrine-related Cancer·Noriko KimuraMitsuhide Naruse
Aug 15, 2015·Endocrine-related Cancer·Diana E BennRoderick J Clifton-Bligh
Oct 16, 2016·Endocrine Pathology·Yusuke MatsudaYoshihiro Ogawa
Feb 25, 2017·Current Opinion in Endocrinology, Diabetes, and Obesity·Alejandro Roman-Gonzalez, Camilo Jimenez
Jun 13, 2017·The Journal of Clinical Endocrinology and Metabolism·Oksana HamidiIrina Bancos

❮ Previous
Next ❯

Citations

Dec 19, 2019·The Journal of Clinical Endocrinology and Metabolism·Ewa GniadoSona Sharma
Jan 3, 2020·Nuclear Medicine Communications·Evangelia SkouraJamshed Bomanji
Nov 30, 2019·Der Chirurg; Zeitschrift für alle Gebiete der operativen Medizen·F WeberH Dralle
Oct 16, 2019·The Journal of Clinical Endocrinology and Metabolism·Matthew P ThorpeSalvador Borges-Neto
Mar 1, 2019·Cancers·Lavinia Vittoria LottiRenato Mariani-Costantini
Apr 26, 2019·Case Reports in Endocrinology·Nelson Carvalho CunhaIsabel Paiva
Mar 12, 2020·Frontiers in Endocrinology·Laura D ScribaCharlotte Steenblock
Sep 12, 2020·IJU Case Reports·Hirofumi KuroseTsukasa Igawa
Feb 17, 2021·ANZ Journal of Surgery·Nikhil KunduAndrew Kiu
Nov 14, 2020·Current Opinion in Oncology·Charles DarianeJudith Favier
Jun 9, 2021·Langenbeck's Archives of Surgery·Miquel Torres-CostaJosé Manuel Rodríguez
May 25, 2021·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Yunying CuiYuxiu Li
Jun 26, 2021·Journal of the Endocrine Society·César Ernesto Lam-ChungDaniel Cuevas-Ramos
Jun 20, 2021·Endocrine Reviews·Svenja NöltingKarel Pacak
Jul 20, 2021·The American Journal of Surgical Pathology·Zhonghua LiuMiao Zhang

❮ Previous
Next ❯

Software Mentioned

GAPP
Cluster

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.