[Von Hippel syndrome with recurrent pheochromocytoma].
A patient with Von Hippel disease is described. The Von Hippel syndrome is a genetic disease characterized by a retinal angiomatosis and multiple tumors (renal and pancreatic sites are common). The pheochromocytoma is-on the contrary-very uncommon and the association with 2 pheochromocytomas has probably never been reported. In our case, a double pheochromocytoma (the first in 1973, the second in 1986) was present, in conjunction with a thyroid tumor and with an anomalous renal vein (circumaortic ring). The clinical picture was characterized by a severe hypertension and a fourth stage retinopathy. A correct diagnosis was established only 10 years after the clinical exordium. The thyroidectomy and the ablation of both the pheochromocytomas were necessary.
Casiglia
E
E
Istituto di Clinica Medica I, Università degli Studi, Padova.
Perrone
S
S
Zamboni
S
S
Spandri
P
P
Rossi
G
G
Pessina
A C
AC
Dal Palù
C
C
D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D005260:Female; D006801:Humans; D009364:Neoplasm Recurrence, Local; D010673:Pheochromocytoma / Q000150:complications* / Q000601:surgery; D006623:von Hippel-Lindau Disease / Q000150:complications*
Sindrome di Von Hippel con feocromocitoma recidivante.