Concomitant occurrence of an adrenal ganglioneuroma and a contralateral pheochromocytoma in a patient with von Recklinghausen's neurofibromatosis. An immunocytochemical study.
This report presents concomitant occurrence of an adrenal ganglioneuroma and a contralateral pheochromocytoma in a patient with von Recklinghausen's disease. The patient's daughter also has cutaneous neurofibromatosis and an adrenal medullary tumor indicating that the observed "three component disease" may represent an inherited neurocristopathy. Immunocytochemically the ganglioneuroma showed a positive reaction with a tyrosinhydroxylase antiserum, but a negative reaction with a dopamine-beta-hydroxylase antiserum, suggesting the capacity of dopamine synthesis. Frequent ganglion cells were immunopositive against neuropeptide Y, but occasional ganglion cells were also positive against enkephalin and substance P. Adrenergic nerve fibers were abundant in the Schwann cell portion of the tumor, but peptide containing nerve cell processes were also demonstrated.
Jansson
S
S
Department of Surgery, University of Göteborg, Sweden.
Dahlström
A
A
Hansson
G
G
Tisell
L E
LE
Ahlman
H
H
D000310:Adrenal Gland Neoplasms / Q000473:pathology*; D005260:Female; D005729:Ganglioneuroma / Q000473:pathology*; D006801:Humans; D007106:Immune Sera; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000473:pathology*; D010673:Pheochromocytoma / Q000473:pathology*; D012583:Schwann Cells / Q000473:pathology
D007106:Immune Sera