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WHO Classification of Tumours
Pheochromocytoma, NOS
Adrenal gland


Definition

Benign phaeochromocytoma and malignant phaeochromocytomas arise in the adrenal medulla and are derived from chromaffin cells of neural crest origin.

Phaeochromocytomas can occur at any age, but are most frequent in the fourth and fifth decades. Clinical symptoms may result from abnormal secretion of catecholamines and include headaches, hypertension, fever, and tremor. Microscopically, a characteristic nesting growth pattern (zellballen) is seen. Phaeochromocytomas may occur in the setting of several hereditary cancer syndromes, including multiple endocrine neoplasia type 2A , multiple endocrine neoplasia type 2B , von Hippel Lindau disease , and neurofibromatosis type 1 .
The prognosis for patients with benign phaeochromocytoma is primarily dependent upon a successful surgical resection and extent of preoperative complications related to hypertension

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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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