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.

Pheochromocytomas and paragangliomas in von Hippel–Lindau disease: not a needle in a haystack

“…The frequency of PPGL among participants with vHL type 2 was 60.0% (95% CI = 53.4–66.3%), with moderate heterogeneity being observed ( I 2 = 54.6%) ( 16 , 17 , 21 , 25 , 26 , 28 , 29 , 35 , 36 , 40 , 44 , 45 , 47 , 48 , 53 , 55 , 56 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 77 , 78 , 79 , 80 , 81 , 82 , 83 , 84 , 85 , 86 , 87 , 88 , 89 , 90 , 91 , 92 , 93 ) ( Table 1 ). We observed lower heterogeneity in subgroup analyses restricted to convenience sampling ( I 2 = 25.6%) and to patients whose diagnosis was performed only clinically ( I 2 = 23.1%) (Supplementary Table 7).…”

“…A total of 52 studies, assessing 840 participants with vHL type 2, provided information on the frequency of PPGL. Most of these studies were conducted in Europe ( n =18) ( 16 , 17 , 21 , 25 , 26 , 28 , 29 , 62 , 66 , 68 , 70 , 71 , 72 , 73 , 76 , 83 , 91 , 92 ), followed by Eastern Asia ( n =17) ( 35 , 36 , 40 , 44 , 45 , 47 , 48 , 74 , 75 , 77 , 78 , 79 , 80 , 81 , 82 , 86 , 87 ) and North America ( n =9) ( 53 , 59 , 60 , 61 , 63 , 64 , 65 , 69 , 84 ). All studies assessed patients of all age groups, with the mean onset age of PPGL being 28.8 years (as reported in 21 studies ( 21 , 26 , 35 , 40 , 44 , 47 , 55 , 59 , 60 , 61 , 65 , 67 , 71 , 72 , 74 , 77 , 79 , 85 , 86 , …”

“…All studies assessed patients of all age groups, with the mean onset age of PPGL being 28.8 years (as reported in 21 studies ( 21 , 26 , 35 , 40 , 44 , 47 , 55 , 59 , 60 , 61 , 65 , 67 , 71 , 72 , 74 , 77 , 79 , 85 , 86 , 87 , 88 )). Six studies reported a consecutive sample ( 16 , 17 , 29 , 66 , 77 , 78 ), whereas in 39 studies a convenience sample was assessed ( 21 , 25 , 35 , 45 , 47 , 53 , 56 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 67 , 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 79 , 80 , 81 , 82 , 83 , 84 , 85 , 86 , 87 , 88 , 89 , 90 , 91 , 92 , 93 ).…”

“…In addition, 58.3%(20,25,26,32,35,37,41,(43)(44)(45)47,52,53,56,58).A total of 52 studies, assessing 840 participants with vHL type 2, provided information on the frequency of PPGL. Most of these studies were conducted in Europe (n=18)(16,17,21,25,26,28,29,62,66,68,(70)(71)(72)(73)76,83,91,92), followed by Eastern Asia (n=17)(35,36,40,44,45,47,48,74,75,(77)(78)(79)(80)(81)(82)86,87) and North Americ...…”

A Family with Von Hippel-Lindau Syndrome: The Findings of Indium-111 Somatostatin Receptor Scintigraphy, Iodine-123 Metaiodobenzylguanidine Scintigraphy and Single Photon Emission Computerized Tomography

“…He was re-operated. Pulcrano et al (5) reported the findings of In-111-DTPA0-octreotide scintigraphy in three members of a family with VHLS. The authors concluded that scintigraphy might be useful in the management plan of those patients.…”