title: Usefulness of [111In-DTPA0] octreotide scintigraphy in a family with von Hippel-Lindau disease.
abstract: 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 131I-metaiodobenzylguanidine-scintigraphy. The role of 111-indium-diethylenetriaminepentaacetic acid [111In-DTPA0] 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 [111In-DTPA0] 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 90Y-1,4,7,10-Tetraazacyclododecane-N,N',N'',N'''-tetraacetic acid (DOTA0)-Tyr3-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 [111In-DTPA0] octreotide scintigraphy may be helpful in the routine work-up of VHL patients for diagnostic and therapeutic purposes.
authors.affiliation: Department of Clinical and Molecular Endocrinology and Oncology, Federico II University of Naples, 80131 Naples, Italy. mpulcrano@libero.it
authors.forename: M
authors.initials: M
authors.lastname: Pulcrano
authors.forename: L
authors.initials: L
authors.lastname: Camera
authors.forename: L
authors.initials: L
authors.lastname: Pagano
authors.forename: S
authors.initials: S
authors.lastname: Del Vecchio
authors.forename: D
authors.initials: D
authors.lastname: Ferone
authors.forename: L
authors.initials: L
authors.lastname: Bodei
authors.forename: A
authors.initials: A
authors.lastname: Murgia
authors.forename: L
authors.initials: L
authors.lastname: Pace
authors.forename: G
authors.initials: G
authors.lastname: Storto
authors.forename: G
authors.initials: G
authors.lastname: Paganelli
authors.forename: A
authors.initials: A
authors.lastname: Colao
authors.forename: M
authors.initials: M
authors.lastname: Salvatore
authors.forename: G
authors.initials: G
authors.lastname: Lombardi
authors.forename: B
authors.initials: B
authors.lastname: Biondi
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D007205:Indium Radioisotopes; D008297:Male; D015282:Octreotide / Q000031:analogs & derivatives*; D004369:Pentetic Acid / Q000031:analogs & derivatives*; D014055:Tomography, Emission-Computed / Q000379:methods*; D006623:von Hippel-Lindau Disease / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000235:genetics*
chemical_list: C497824:111In-octreotide, DTPA(0)-; D007205:Indium Radioisotopes; D004369:Pentetic Acid; D015282:Octreotide