Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism

Olga T. Hardy, Miguel Hernandez-Pampaloni, Janet R. Saffer, Joshua S. Scheuermann, Linda M. Ernst, Richard Freifelder, Hongming Zhuang, Courtney MacMullen, Susan Becker, N. Scott Adzick, Chaitanya Divgi, Abass Alavi, Charles A. Stanley

Research output: Contribution to journalArticle

93 Citations (Scopus)

Abstract

Objectives: Focal lesions in infants with congenital hyperinsulinism (HI) represent areas of adenomatosis that express a paternally derived ATP-sensitive potassium channel mutation due to embryonic loss of heterozygosity for the maternal 11p region. This study evaluated the accuracy of 18F-fluoro- L-dihydroxyphenylalanine ([18F]DOPA) positron emission tomography (PET) scans in diagnosing focal vs. diffuse disease and identifying the location of focal lesions. Design: A total of 50 infants with HI unresponsive to medical therapy were studied. Patients were injected iv with [18F]DOPA, and PET scans were obtained for 50-60 min. Images were coregistered with abdominal computed tomography scans. PET scan interpretations were compared with histological diagnoses. Results: The diagnosis of focal or diffuse HI was correct in 44 of the 50 cases (88%). [18F]DOPA PET identified focal areas of high uptake of radiopharmaceutical in 18 of 24 patients with focal disease. The locations of these lesions matched the areas of increased [ 18F]DOPA uptake on the PET scans in all of the cases. PET scan correctly located five lesions that could not be visualized at surgery. The positive predictive value of [18F]DOPA in diagnosing focal adenomatosis was 100%, and the negative predictive value was 81%. Conclusions: [18F]DOPA PET scans correctly diagnosed 75% of focal cases and were 100% accurate in identifying the location of the lesion. These results suggest that [18F]DOPA PET imaging provides a useful guide to surgical resection of focal adenomatosis and should be considered as a guide to surgery in all infants with congenital HI who have medically uncontrollable disease.

Original languageEnglish (US)
Pages (from-to)4706-4711
Number of pages6
JournalJournal of Clinical Endocrinology and Metabolism
Volume92
Issue number12
DOIs
StatePublished - Dec 2007

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Congenital Hyperinsulinism
Positron emission tomography
Positron-Emission Tomography
Hyperinsulinism
Surgery
KATP Channels
Dihydroxyphenylalanine
Radiopharmaceuticals
fluorodopa F 18
Loss of Heterozygosity
Tomography
Mothers
Imaging techniques
Mutation

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism

Cite this

Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism. / Hardy, Olga T.; Hernandez-Pampaloni, Miguel; Saffer, Janet R.; Scheuermann, Joshua S.; Ernst, Linda M.; Freifelder, Richard; Zhuang, Hongming; MacMullen, Courtney; Becker, Susan; Adzick, N. Scott; Divgi, Chaitanya; Alavi, Abass; Stanley, Charles A.

In: Journal of Clinical Endocrinology and Metabolism, Vol. 92, No. 12, 12.2007, p. 4706-4711.

Research output: Contribution to journalArticle

Hardy, OT, Hernandez-Pampaloni, M, Saffer, JR, Scheuermann, JS, Ernst, LM, Freifelder, R, Zhuang, H, MacMullen, C, Becker, S, Adzick, NS, Divgi, C, Alavi, A & Stanley, CA 2007, 'Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism', Journal of Clinical Endocrinology and Metabolism, vol. 92, no. 12, pp. 4706-4711. https://doi.org/10.1210/jc.2007-1637
Hardy, Olga T. ; Hernandez-Pampaloni, Miguel ; Saffer, Janet R. ; Scheuermann, Joshua S. ; Ernst, Linda M. ; Freifelder, Richard ; Zhuang, Hongming ; MacMullen, Courtney ; Becker, Susan ; Adzick, N. Scott ; Divgi, Chaitanya ; Alavi, Abass ; Stanley, Charles A. / Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism. In: Journal of Clinical Endocrinology and Metabolism. 2007 ; Vol. 92, No. 12. pp. 4706-4711.
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title = "Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism",
abstract = "Objectives: Focal lesions in infants with congenital hyperinsulinism (HI) represent areas of adenomatosis that express a paternally derived ATP-sensitive potassium channel mutation due to embryonic loss of heterozygosity for the maternal 11p region. This study evaluated the accuracy of 18F-fluoro- L-dihydroxyphenylalanine ([18F]DOPA) positron emission tomography (PET) scans in diagnosing focal vs. diffuse disease and identifying the location of focal lesions. Design: A total of 50 infants with HI unresponsive to medical therapy were studied. Patients were injected iv with [18F]DOPA, and PET scans were obtained for 50-60 min. Images were coregistered with abdominal computed tomography scans. PET scan interpretations were compared with histological diagnoses. Results: The diagnosis of focal or diffuse HI was correct in 44 of the 50 cases (88{\%}). [18F]DOPA PET identified focal areas of high uptake of radiopharmaceutical in 18 of 24 patients with focal disease. The locations of these lesions matched the areas of increased [ 18F]DOPA uptake on the PET scans in all of the cases. PET scan correctly located five lesions that could not be visualized at surgery. The positive predictive value of [18F]DOPA in diagnosing focal adenomatosis was 100{\%}, and the negative predictive value was 81{\%}. Conclusions: [18F]DOPA PET scans correctly diagnosed 75{\%} of focal cases and were 100{\%} accurate in identifying the location of the lesion. These results suggest that [18F]DOPA PET imaging provides a useful guide to surgical resection of focal adenomatosis and should be considered as a guide to surgery in all infants with congenital HI who have medically uncontrollable disease.",
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T1 - Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism

AU - Hardy, Olga T.

AU - Hernandez-Pampaloni, Miguel

AU - Saffer, Janet R.

AU - Scheuermann, Joshua S.

AU - Ernst, Linda M.

AU - Freifelder, Richard

AU - Zhuang, Hongming

AU - MacMullen, Courtney

AU - Becker, Susan

AU - Adzick, N. Scott

AU - Divgi, Chaitanya

AU - Alavi, Abass

AU - Stanley, Charles A.

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N2 - Objectives: Focal lesions in infants with congenital hyperinsulinism (HI) represent areas of adenomatosis that express a paternally derived ATP-sensitive potassium channel mutation due to embryonic loss of heterozygosity for the maternal 11p region. This study evaluated the accuracy of 18F-fluoro- L-dihydroxyphenylalanine ([18F]DOPA) positron emission tomography (PET) scans in diagnosing focal vs. diffuse disease and identifying the location of focal lesions. Design: A total of 50 infants with HI unresponsive to medical therapy were studied. Patients were injected iv with [18F]DOPA, and PET scans were obtained for 50-60 min. Images were coregistered with abdominal computed tomography scans. PET scan interpretations were compared with histological diagnoses. Results: The diagnosis of focal or diffuse HI was correct in 44 of the 50 cases (88%). [18F]DOPA PET identified focal areas of high uptake of radiopharmaceutical in 18 of 24 patients with focal disease. The locations of these lesions matched the areas of increased [ 18F]DOPA uptake on the PET scans in all of the cases. PET scan correctly located five lesions that could not be visualized at surgery. The positive predictive value of [18F]DOPA in diagnosing focal adenomatosis was 100%, and the negative predictive value was 81%. Conclusions: [18F]DOPA PET scans correctly diagnosed 75% of focal cases and were 100% accurate in identifying the location of the lesion. These results suggest that [18F]DOPA PET imaging provides a useful guide to surgical resection of focal adenomatosis and should be considered as a guide to surgery in all infants with congenital HI who have medically uncontrollable disease.

AB - Objectives: Focal lesions in infants with congenital hyperinsulinism (HI) represent areas of adenomatosis that express a paternally derived ATP-sensitive potassium channel mutation due to embryonic loss of heterozygosity for the maternal 11p region. This study evaluated the accuracy of 18F-fluoro- L-dihydroxyphenylalanine ([18F]DOPA) positron emission tomography (PET) scans in diagnosing focal vs. diffuse disease and identifying the location of focal lesions. Design: A total of 50 infants with HI unresponsive to medical therapy were studied. Patients were injected iv with [18F]DOPA, and PET scans were obtained for 50-60 min. Images were coregistered with abdominal computed tomography scans. PET scan interpretations were compared with histological diagnoses. Results: The diagnosis of focal or diffuse HI was correct in 44 of the 50 cases (88%). [18F]DOPA PET identified focal areas of high uptake of radiopharmaceutical in 18 of 24 patients with focal disease. The locations of these lesions matched the areas of increased [ 18F]DOPA uptake on the PET scans in all of the cases. PET scan correctly located five lesions that could not be visualized at surgery. The positive predictive value of [18F]DOPA in diagnosing focal adenomatosis was 100%, and the negative predictive value was 81%. Conclusions: [18F]DOPA PET scans correctly diagnosed 75% of focal cases and were 100% accurate in identifying the location of the lesion. These results suggest that [18F]DOPA PET imaging provides a useful guide to surgical resection of focal adenomatosis and should be considered as a guide to surgery in all infants with congenital HI who have medically uncontrollable disease.

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