Pheochromocytoma in von hippel-lindau disease: distinct histopathologic phenotype compared to pheochromocytoma in multiple endocrine neoplasia type 2.
Pheochromocytomas are rare neuroendocrine tumors that arise from chromaffin tissue. In a small subset of patients, pheochromocytomas occur as a manifestation of von Hippel- Lindau (VHL) disease. The histology of VHL-associated pheochromocytomas has not been reported in detail. In this article, we describe histopathologic features of 14 pheochromocytomas in eight patients with VHL disease and demonstrate that VHL-associated pheochromocytomas have a distinct histologic phenotype as compared with pheochromocytomas in patients with multiple endocrine neoplasia type 2 (MEN 2). VHL tumors are characterized by a thick vascular tumor capsule; myxoid and hyalinized stroma; round, small to medium tumor cells intermixed with small vessels; predominantly amphophilic and clear cytoplasm; absence of cytoplasmic hyaline globules; and lack of nuclear atypia or mitoses. In contrast to MEN 2, there is no extratumoral adrenomedullary hyperplasia in the VHL adrenal gland. Our findings of a distinct histologic phenotype of VHL pheochromocytoma may further help in subdividing patients who clinically present with multiple, bilateral pheochromocytomas.
Koch
Christian A
CA
Pediatric and Reporoductive ENdocinology Branch, National Institute of Child Health and Human Development, Bethesda, Maryland 20892, USA.
Mauro
David
D
Walther
McClellan M
MM
Linehan
W Marston
WM
Vortmeyer
Alexander O
AO
Jaffe
Ronald
R
Pacak
Karel
K
Chrousos
George P
GP
Zhuang
Zhengping
Z
Lubensky
Irina A
IA
D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000473:pathology*; D000328:Adult; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018813:Multiple Endocrine Neoplasia Type 2a / Q000150:complications / Q000473:pathology*; D010673:Pheochromocytoma / Q000150:complications / Q000473:pathology*; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000473:pathology*