8700/3 Pheochromoblastoma



Definitions

Adrenal gland
ICD-O-3 topography code: C74
ICD10: C74
           

A pheochromocytoma that metastasizes to other anatomic sites. Common sites of metastasis include lymph nodes, bones, liver, and lung. Morphologic features associated with malignant pheochromocytomas include: atypical mitotic figures, capsular and vascular invasion, tumor cell necrosis, and high mitotic activity 1
 
Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004



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Malignant phaeochromocytomas comprise up to 10% of all phaeochromocytomas
2
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Medeiros LJ, Wolf BC, Balogh K, Federman M (1985)
Adrenal pheochromocytoma: a clinicopathologic review of 60 cases.
Hum Pathol 16: 580-9



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The usual prognosis of malignant phaeochromocytoma is poor, with a 45- 55% 5-year survival
3
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Modlin IM, Farndon JR, Shepherd A, Johnston ID, Kennedy TL, Montgomery DA, Welbourn RB (1979)
Phaeochromocytomas in 72 patients: clinical and diagnostic features, treatment and long term results.
Br J Surg 66: 456-65



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Pommier RF, Vetto JT, Billingsly K, Woltering EA, Brennan MF (1993)
Comparison of adrenal and extraadrenal pheochromocytomas.
Surgery 114: 1160-5; discussion 1165-6



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Scott HW, Halter SA (1984)
Oncologic aspects of pheochromocytoma: the importance of follow-up.
Surgery 96: 1061-6



. However, some patients may have indolent disease, with life expectancy of more than 20 years 6
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Young AL, Baysal BE, Deb A, Young WF (2002)
Familial malignant catecholamine-secreting paraganglioma with prolonged survival associated with mutation in the succinate dehydrogenase B gene.
J Clin Endocrinol Metab 87: 4101-5



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