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Special Issue "Pheochromocytoma and Paraganglioma: Research Update"
Deadline for manuscript submissions: closed (30 July 2018).
Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors in the adrenal medulla and extra-adrenal paraganglia. Paragangliomas can be divided into parasympathetic PGL (head and neck PGL) and sympathetic PGL. The distinction between “benign” and “malignant” PPGL has been discussed at length for decades. Current thinking is that all PPGL have some metastatic potential and ICD-O code /3 (cancer) was given by the WHO tumor classification 4th ed. 2017. Therefore, the previous categories of benign and malignant PPLGs have been eliminated for an approach based on risk stratification. Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) is a tool for predicting metastasis and patients’ prognosis. However, GAPP cannot be applied for parasympathetic PGL at present. At least 30% of the tumors are now known to be hereditary, and about 19 genes have been determined and genotype–phenotype correlations have been clarified. Of these gene mutations, succinate dehydrogenase genes SDHA, SDHB, SDHC, and SDHD are strongly correlated with extra-adrenal locations, younger age occurrence, multiplicity, metastasis, and poor prognosis. Tumor, Node, Metastasis (TNM) Classification by American Joint Committee on Cancer (AJCC) Cancer Staging Manual (8th edition) was first applied for PPGLs in 2017. Clinical usefulness of GAPP, genetic analysis, and TNM Classification should be comprehensively discussed. Please join us in presenting this Special Issue on the risk stratification on pheochromocytoma and paraganglioma from the point of view of laboratory and clinical medicine.Dr. Noriko Kimura
Manuscript Submission Information
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- Catecholamine types and levels
- ICD-O code
- TNM classification