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. 2017 Feb 5;26(1):38-42.
doi: 10.4274/mirt.70894.

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

Pelin Arıcan  1 , Berna Okudan Tekin, Seniha Naldöken, Rıza Şefizade, Dilek Berker
Affiliations

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

Pelin Arıcan et al. Mol Imaging Radionucl Ther. .

Abstract
in English, Turkish

Von Hippel-Lindau syndrome (VHLS) is an autosomal dominant hereditary familial disorder characterized by development of malignant and benign neoplasms. Differential diagnosis of the adrenal and pancreatic masses are difficult in patients with VHLS. Iodine-123 metaiodobenzylguanidine (I-123 MIBG) and indium-111 somatostatin receptor scintigraphies (In-111 SRS) have important roles in the differential diagnosis of adrenal and pancreatic masses in those patients. In this case report, we present the findings of I-123 MIBG single-photon emission computerized tomography (SPECT/CT) and In-111 SRS SPECT/CT in three members of a family with VHLS. In case 1, a residual neuroendocrine tumor (NET) was detected in the head of pancreas on In-111 SRS SPECT/CT images. In case 2 and 3, I-123 MIBG SPECT/CT confirmed the adrenal masses as pheochromocytoma, and the extra-adrenal mass as NET, before surgery. We thought that In-111 SRS and I-123 MIBG scan might be helpful in the routine work up of VHLS patients for diagnostic and therapeutic purposes. Hybrid SPECT/CT system may improve diagnostic accuracy of planar images since it assesses morphologic and functional information together.

Von Hippel-Lindau sendromu (VHLS) malign ve benign tümörler ile karakterize otozomal dominant herediter ailesel bir hastalıktır. VHLS’li hastalarda adrenal ve pankreatik kitlelerin ayırıcı tanısı zordur. Bu hastalarda iyot-123 metaiyodobenzilguanidin (I-123 MIBG) ve indiyum-111 somatostatin reseptör sintigrafileri (In-111 SRS) adrenal ve pankreatik kitlelerin ayırıcı tanısında önemli rol oynar. Bu olgu sunumunda VHLS’li ailenin üç üyesinin I-123 MIBG bilgisayarlı tek foton emisyonlu tomografisi (SPECT/BT) ve In-111 SRS SPECT/BT bulgularını sunuyoruz. Olgu 1’de, In-111 SRS SPECT/BT görüntülerinde pankreas başında rezidü nöroendokrin tümör (NET) saptandı. Olgu 2 ve 3’de, I-123 MIBG SPECT/BT cerrahi öncesi adrenal bezdeki kitleleri feokromositoma ve adrenal dışı kitleyi NET olarak onayladı. In-111 SRS ve I-123 MIBG sintigrafilerinin VHLS’li hastaların tanı ve tedavisi için rutin kullanımda yararlı olabileceğini düşündük. Hibrid SPECT/BT morfolojik ve fonksiyonel bilgileri birlikte değerlendirdiği için, planar görüntülerin tanısal doğruluğunu artırır.

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Conflict of interest statement

Conflict of Interest: No conflict of interest was declared by the authors.

Figures

Figure 1
Figure 1. In-111 somatostatin receptor scintigraphies findings of case 1. Anterior-posterior whole body images show focal radiotracer accumulation medial to the right kidney (A) at 4, and (B) 24 hours (arrows). Axial (C) and coronal (D) computed tomography (CT), single photon emission computerized tomography (SPECT), and SPECT/CT images show that this accumulation is localized to the head of the pancreas (arrows). The diagnosis of residual neuroendocrine tumor was confirmed by histopathologic examination
Figure 2
Figure 2. Iodine-123 metaiyodobenzilguanidin scintigraphy findings of case 2. Anterior-posterior whole body images show focal tracer uptake superior to the kidneys bilaterally (A) at 4, and (B) 24 hours, before surgery (arrows), (C) axial (D) coronal computed tomography (CT), single photon emission computerized tomography (SPECT), and SPECT/CT images reveal that those radiotracer accumulations are localized to the adrenal glands bilaterally (arrows). Histopathologic examination of the masses were reported as pheochromocytoma after surgery
Figure 3
Figure 3. Iodine-123 metaiyodobenzilguanidin scintigraphy findings of case 3. Anterior-posterior whole body images show a large focal tracer uptake medial to the liver (arrows with black contours) and small focal activity superior to the left kidney (white arrows) (A) at 4, and (B) 24 hours, before surgery. Axial computed tomography (C), and single photon emission computerized tomography slices show the intense abnormal radiotracer accumulation in the right adrenal gland (arrows), and (D) a smaller activity in the extra adrenal and prevertebral region (arrows). After surgery, the histopathologic examination confirmed the mass in the right adrenal as pheochromocytoma, and the mass on the left side as paraganglioma
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References

    1. Richard S, Graff J, Lindau J, Resche F. Von Hippel-Lindau disease. Lancet. 2004;363:1231–1234. - PubMed
    1. Crossey PA, Richards FM, Foster K, Green JS, Prowse A, Latif F, Lerman MI, Zbar B, Affara NA, Ferguson-Smith MA, et al. Identification of intragenic mutations in the von Hippel-Lindau disease tumour suppressor gene and correlation with disease phenotype. Hum Mol Genet. 1994;3:1303–1308. - PubMed
    1. Sharma P, Dhull VS, Bal C, Malhotra A, Kumar R. Von Hippel-Lindau syndrome: demonstration of entire disease spectrum with (68)Ga-DOTANOC PET-CT. Korean J Radiol. 2014;15:169–172. - PMC - PubMed
    1. Rufini V, Calcagni ML, Baum RP. Imaging of neuroendocrine tumors. Semin Nucl Med. 2006;36:228–247. - PubMed
    1. Pulcrano M, Camera L, Pagano L, Del Vecchio S, Ferone D, Bodei L, Murgia A, Pace L, Storto G, Paganelli G, Colao A, Salvatore M, Lombardi G, Biondi B. Usefulness of [111In-DTPA0] octreotide scintigraphy in a family with von Hippel-Lindau disease. J Endocrinol Invest. 2008;31:352–359. - PubMed

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