Update on paraganglioma and pheochromocytoma based on WHO tumour classification 2017

  • Kimura Noriko
    Department of Diagnostic Pathology, National Hospital Organization Hakodate Hospital
  • Shiga Kiyoto
    Department of Head & Neck Surgery, Iwate Medical University School of Medicine
  • Kaneko Ken-ich
    Department of Otolaryngology-Head and Neck Surgery, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki
  • Takekoshi Kazuhiro
    Division of Sports Medicine, Faculty of Medicine, University of Tsukuba

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Other Title
  • 頭頸部および腹部パラガングリオーマの類似点と相違点
  • —臨床病理学的解析—

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Pheochromocytoma (PCC) and paraganglioma (PGL) are derived from the neural crest: PCC is from the adrenal medulla and PGLs are from the extra-adrenal paraganglions. They have similar histologic features such as Zellballen pattern. PGLs are subdivided into head and neck parasympathetic PGLs (HNPGL) including carotid body tumor (60%), middle ear PGL (30%), vagal PGL (10%), and sympathetic PGLs (S-PGL) in the abdominal, pelvic, and thoracic regions. PCCs and S-PGLs are catecholamine-producing tumors, however, HNPGLs are usually non-functioning tumors. Chromogranin A and dopamine-beta-hydroxylase are the most useful markers for PCC and S-PGLs, however, expression of these proteins is far less in HNPGLs than in PCCs and S-PGLs. In HNPGLs, 30% are familial, 10-25% are bilateral, 17-37% are multiple, and 4-8% are metastatic. In S-PGLs, the metastatic rate is about 30-40%, and the WHO Classification of Tumours of Endocrine Organs 4th edition (2017) defined that all PCCs and PGLs(PPGLs)have some metastatic potential, assigned them ICD-O3 (malignant tumor), and deleted the term “benign PPGLs.„ To date, 20 susceptible genes for PPGLs have been detected and genotype-phenotype correlations have been clarified. Of these genes, mutations of succinate dehydrogenase complex, subunit SDHD, SDHAF2, SDHC and SDHB show high penetrance in HPGLs, and are especially important for hereditary, multiplicity and metastasis. Immunohistochemistry of SDHB shows negativity in PPGLs of SDH mutations, which is a useful tool for screening of such mutations. Pathologic diagnosis for predicting metastasis has been the most important issue for PPGLs. Grading of adrenal pheochromocytoma and paraganglioma (GAPP) scoring was made by the nationwide survey of Japanese study on PPGLs. It is recommended to make a diagnosis based on GAPP scoring, however, its application to HNPGLs may be difficult due to histologic damage by preoperative embolization. In conclusion, multicenter research is necessary to accumulate clinicopathologic data including metastasis, diagnostic tools, biologic and genomic studies, treatments, and patients' prognosis.


  • Toukeibu Gan

    Toukeibu Gan 45 (4), 350-353, 2019

    Japan Society for Head and Neck Cancer


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