Usefulness of [111In-DTPA0] octreotide scintigraphy in a family with von Hippel-Lindau disease.

The identification of patients with von Hippel-Lindau (VHL) disease dictates accurate genetic counseling of family members, whereas screening for early detection of visceral and neurological involvement is usually performed by a combination of radiological and nuclear medicine techniques such as ultrasonography or contrast-enhanced computed tomography of the upper abdomen, magnetic resonance imaging of the central nervous system and (131)I-metaiodobenzylguanidine-scintigraphy. The role of (111)-indium-diethylenetriaminepentaacetic acid [(111)In-DTPA(0)] octreotide scintigraphy in this clinical context has never been investigated. Here, we report imaging findings in a VHL patient and in 3 consecutive family members undergoing clinical and radiological screening that included [(111)In-DTPA(0)] octreotide scintigraphy in addition to the above-mentioned procedures. Somatostatin receptor expression was investigated in vitro by immunohistochemistry in pancreatic tumor sections. On the basis of in vivo and in vitro findings, octreotide long-acting release treatment followed by (90)Y-1,4,7,10-Tetraazacyclododecane-N, N',N '',N '''tetralacetic acid (DOTA(0))-Tyr(3)-octreotide led to a lack of progression in this patient although this result is a possibility which needs to be proved by further investigation and longer follow-up. The results of this study suggest that [(111)In-DTPA(0)] octreotide scintigraphy may be helpful in the routine work-up of VHL patients for diagnostic and therapeutic purposes.