logo logo
Search: Organ site Search: Tumor / Syndrome
or
WHO Classification of Tumours
Pheochromocytoma, malignant
Adrenal gland


Definition

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
Click for details
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



.

Malignant phaeochromocytomas comprise up to 10% of all phaeochromocytomas

Click to access Pubmed
Medeiros LJ, Wolf BC, Balogh K, Federman M (1985)
Adrenal pheochromocytoma: a clinicopathologic review of 60 cases.
Hum Pathol 16: 580-9



.
The usual prognosis of malignant phaeochromocytoma is poor, with a 45- 55% 5-year survival

Click to access Pubmed
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




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




Click to access Pubmed
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
Click to access Pubmed
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



.