Endocrine Pathology

, Volume 16, Issue 1, pp 23–32 | Cite as

Histological grading of adrenal and extra-adrenal pheochromocytomas and relationship to prognosis: A clinicopathological analysis of 116 adrenal pheochromocytomas and 30 extra-adrenal sympathetic paragangliomas including 38 malignant tumors

  • Noriko Kimura
  • Toshiya Watanabe
  • Takao Noshiro
  • Soichiro Shizawa
  • Yukio Miura
Japan EPS Proceedings


Pheochromocytomas and extra-adrenal sympathetic paragangliomas show varied histological patterns, and it is difficult to diagnose malignancy or predict the clinical course using current histological criteria. In the present study, we reviewed 146 sympathetic paragangliomas including 116 adrenal (102 unilateral, 14 bilateral) and 30 extra-adrenal tumors including 38 metastatic tumors. We developed a scoring scale according to the following six factors: histological pattern, cellularity, coagulation necrosis, vascular/capsular invasion, Ki-67 immunoreactivity, and types of catecholamine produced. The tumors were classified as well (WD), moderately (MD), and poorly differentiated (PD) types according to their scores. The frequency of these tumor types were 113 WD (77%), 27 MD (19%), and 6 PD (4%). Metastasis was observed in 15 of 113 WD (13%), 17 of 27 MD (63%), and all 6 PD (100%). Five-year survivals of patients with metastases were 92% with WD, 69% with MD, 0% with PD. Respective 10-yr survivals were 83%, 38%, and 0%. Differences between groups were statistically significant. The data show that using this grading scoring system for sympathetic paragangliomas correlates with both metastatic potential and patient survival.

Key Words

Pheochromocytoma paraganglioma malignant grading metastasis prognosis 


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Copyright information

© Humana Press Inc 2005

Authors and Affiliations

  • Noriko Kimura
    • 3
  • Toshiya Watanabe
    • 1
  • Takao Noshiro
    • 1
  • Soichiro Shizawa
    • 2
  • Yukio Miura
    • 1
  1. 1.Second Department of Internal MedicineTohoku University School of MedicineSendaiJapan
  2. 2.Department of MedicineNational Nishitaga HospitalSendaiJapan
  3. 3.Department of Pathology and Laboratory MedicineTohoku Rosai Hospital, Japan Labor Health and Welfare OrganizationSendaiJapan

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