8700/0 Chromaffin tumor


Related terms

Pheochromocytoma, NOS

Definitions

Kidney
ICD-O-3 topography code: C64

A usually benign neuroendocrine neoplasm of the sympathetic nervous system that secretes catecholamines. A very small number of tumours have been described in the kidney. Most tumours are small. The cut surface is grey, often well vascularized. Histopathologically there are the typical cell clusters (Zellballen), surrounded by a network of fine collagenous septa, blood vessels and sustentacular cells 1
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



2
 
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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Adrenal gland
ICD-O-3 topography code: C74

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
3
 
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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