Neuroendocrine tumors of the pancreas in von Hippel-Lindau disease

Spectrum of appearances at CT and MR imaging with histopathologic comparison

Hani B. Marcos, Steven K. Libutti, H. Richard Alexander, Irina A. Lubensky, David L. Bartlett, McClellan M. Walther, W. Marston Linehan, Gladys M. Glenn, Peter L. Choyke

Research output: Contribution to journalArticle

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Abstract

PURPOSE: To demonstrate the imaging characteristics of neuroendocrine tumors (NETs) of the pancreas in patients with von Hippel-Lindau (VHL) disease to establish diagnostic criteria. MATERIALS AND METHODS: Twenty-five patients with VHL disease and 29 surgically confirmed pancreatic NETs were included. Screening computed tomographic (CT) and/or magnetic resonance (MR) imaging findings were reviewed, and tumor number, diameter, growth rates (doubling time), location, presence of metastatic disease, and attenuation or enhancement properties were determined. RESULTS: Eighteen of 29 (62%) pancreatic NETs were smaller than 3.0 cm in diameter and enhanced homogeneously on contrast material-enhanced CT and MR images. No tumor smaller than 3.0 cm metastasized. Tumors 3.0 cm or larger (11 [38%] of 29) more often enhanced heterogeneously, and two of 11 were associated with hepatic metastases. Smaller (<3.0 cm) tumors displayed longer mean doubling times (mean, 927 vs 351 days) than did larger (≥3.0 cm) tumors; however, there was considerable overlap. Fifteen (52%) tumors were located in the pancreatic head; eight (28%), in the tail; and six (21%), in the body. Ten (40%) patients with pancreatic NETs had associated pheochromocytomas, and 22 (88%) had no or mild pancreatic cystic disease, which is substantially more than the general population of patients with VHL disease. CONCLUSION: Pancreatic NETs in VHL have characteristic features at CT and MR imaging: Most are small, located in the pancreatic head, and enhance homogeneously. Tumors larger than 3.0 cm are prone to metastasize and enhance heterogeneously.

Original languageEnglish (US)
Pages (from-to)751-758
Number of pages8
JournalRadiology
Volume225
Issue number3
StatePublished - Dec 2002
Externally publishedYes

Fingerprint

von Hippel-Lindau Disease
Neuroendocrine Tumors
Pancreas
Magnetic Resonance Imaging
Neoplasms
Pancreatic Diseases
Pheochromocytoma
Contrast Media
Magnetic Resonance Spectroscopy
Neoplasm Metastasis
Liver
Growth

Keywords

  • Pancreas, CT
  • Pancreas, cysts
  • Pancreas, MR
  • Pancreas, neoplasms
  • Pheochromocytoma
  • Von Hippel-Lindau disease

ASJC Scopus subject areas

  • Radiological and Ultrasound Technology

Cite this

Marcos, H. B., Libutti, S. K., Alexander, H. R., Lubensky, I. A., Bartlett, D. L., Walther, M. M., ... Choyke, P. L. (2002). Neuroendocrine tumors of the pancreas in von Hippel-Lindau disease: Spectrum of appearances at CT and MR imaging with histopathologic comparison. Radiology, 225(3), 751-758.

Neuroendocrine tumors of the pancreas in von Hippel-Lindau disease : Spectrum of appearances at CT and MR imaging with histopathologic comparison. / Marcos, Hani B.; Libutti, Steven K.; Alexander, H. Richard; Lubensky, Irina A.; Bartlett, David L.; Walther, McClellan M.; Linehan, W. Marston; Glenn, Gladys M.; Choyke, Peter L.

In: Radiology, Vol. 225, No. 3, 12.2002, p. 751-758.

Research output: Contribution to journalArticle

Marcos, HB, Libutti, SK, Alexander, HR, Lubensky, IA, Bartlett, DL, Walther, MM, Linehan, WM, Glenn, GM & Choyke, PL 2002, 'Neuroendocrine tumors of the pancreas in von Hippel-Lindau disease: Spectrum of appearances at CT and MR imaging with histopathologic comparison', Radiology, vol. 225, no. 3, pp. 751-758.
Marcos HB, Libutti SK, Alexander HR, Lubensky IA, Bartlett DL, Walther MM et al. Neuroendocrine tumors of the pancreas in von Hippel-Lindau disease: Spectrum of appearances at CT and MR imaging with histopathologic comparison. Radiology. 2002 Dec;225(3):751-758.
Marcos, Hani B. ; Libutti, Steven K. ; Alexander, H. Richard ; Lubensky, Irina A. ; Bartlett, David L. ; Walther, McClellan M. ; Linehan, W. Marston ; Glenn, Gladys M. ; Choyke, Peter L. / Neuroendocrine tumors of the pancreas in von Hippel-Lindau disease : Spectrum of appearances at CT and MR imaging with histopathologic comparison. In: Radiology. 2002 ; Vol. 225, No. 3. pp. 751-758.
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title = "Neuroendocrine tumors of the pancreas in von Hippel-Lindau disease: Spectrum of appearances at CT and MR imaging with histopathologic comparison",
abstract = "PURPOSE: To demonstrate the imaging characteristics of neuroendocrine tumors (NETs) of the pancreas in patients with von Hippel-Lindau (VHL) disease to establish diagnostic criteria. MATERIALS AND METHODS: Twenty-five patients with VHL disease and 29 surgically confirmed pancreatic NETs were included. Screening computed tomographic (CT) and/or magnetic resonance (MR) imaging findings were reviewed, and tumor number, diameter, growth rates (doubling time), location, presence of metastatic disease, and attenuation or enhancement properties were determined. RESULTS: Eighteen of 29 (62{\%}) pancreatic NETs were smaller than 3.0 cm in diameter and enhanced homogeneously on contrast material-enhanced CT and MR images. No tumor smaller than 3.0 cm metastasized. Tumors 3.0 cm or larger (11 [38{\%}] of 29) more often enhanced heterogeneously, and two of 11 were associated with hepatic metastases. Smaller (<3.0 cm) tumors displayed longer mean doubling times (mean, 927 vs 351 days) than did larger (≥3.0 cm) tumors; however, there was considerable overlap. Fifteen (52{\%}) tumors were located in the pancreatic head; eight (28{\%}), in the tail; and six (21{\%}), in the body. Ten (40{\%}) patients with pancreatic NETs had associated pheochromocytomas, and 22 (88{\%}) had no or mild pancreatic cystic disease, which is substantially more than the general population of patients with VHL disease. CONCLUSION: Pancreatic NETs in VHL have characteristic features at CT and MR imaging: Most are small, located in the pancreatic head, and enhance homogeneously. Tumors larger than 3.0 cm are prone to metastasize and enhance heterogeneously.",
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T1 - Neuroendocrine tumors of the pancreas in von Hippel-Lindau disease

T2 - Spectrum of appearances at CT and MR imaging with histopathologic comparison

AU - Marcos, Hani B.

AU - Libutti, Steven K.

AU - Alexander, H. Richard

AU - Lubensky, Irina A.

AU - Bartlett, David L.

AU - Walther, McClellan M.

AU - Linehan, W. Marston

AU - Glenn, Gladys M.

AU - Choyke, Peter L.

PY - 2002/12

Y1 - 2002/12

N2 - PURPOSE: To demonstrate the imaging characteristics of neuroendocrine tumors (NETs) of the pancreas in patients with von Hippel-Lindau (VHL) disease to establish diagnostic criteria. MATERIALS AND METHODS: Twenty-five patients with VHL disease and 29 surgically confirmed pancreatic NETs were included. Screening computed tomographic (CT) and/or magnetic resonance (MR) imaging findings were reviewed, and tumor number, diameter, growth rates (doubling time), location, presence of metastatic disease, and attenuation or enhancement properties were determined. RESULTS: Eighteen of 29 (62%) pancreatic NETs were smaller than 3.0 cm in diameter and enhanced homogeneously on contrast material-enhanced CT and MR images. No tumor smaller than 3.0 cm metastasized. Tumors 3.0 cm or larger (11 [38%] of 29) more often enhanced heterogeneously, and two of 11 were associated with hepatic metastases. Smaller (<3.0 cm) tumors displayed longer mean doubling times (mean, 927 vs 351 days) than did larger (≥3.0 cm) tumors; however, there was considerable overlap. Fifteen (52%) tumors were located in the pancreatic head; eight (28%), in the tail; and six (21%), in the body. Ten (40%) patients with pancreatic NETs had associated pheochromocytomas, and 22 (88%) had no or mild pancreatic cystic disease, which is substantially more than the general population of patients with VHL disease. CONCLUSION: Pancreatic NETs in VHL have characteristic features at CT and MR imaging: Most are small, located in the pancreatic head, and enhance homogeneously. Tumors larger than 3.0 cm are prone to metastasize and enhance heterogeneously.

AB - PURPOSE: To demonstrate the imaging characteristics of neuroendocrine tumors (NETs) of the pancreas in patients with von Hippel-Lindau (VHL) disease to establish diagnostic criteria. MATERIALS AND METHODS: Twenty-five patients with VHL disease and 29 surgically confirmed pancreatic NETs were included. Screening computed tomographic (CT) and/or magnetic resonance (MR) imaging findings were reviewed, and tumor number, diameter, growth rates (doubling time), location, presence of metastatic disease, and attenuation or enhancement properties were determined. RESULTS: Eighteen of 29 (62%) pancreatic NETs were smaller than 3.0 cm in diameter and enhanced homogeneously on contrast material-enhanced CT and MR images. No tumor smaller than 3.0 cm metastasized. Tumors 3.0 cm or larger (11 [38%] of 29) more often enhanced heterogeneously, and two of 11 were associated with hepatic metastases. Smaller (<3.0 cm) tumors displayed longer mean doubling times (mean, 927 vs 351 days) than did larger (≥3.0 cm) tumors; however, there was considerable overlap. Fifteen (52%) tumors were located in the pancreatic head; eight (28%), in the tail; and six (21%), in the body. Ten (40%) patients with pancreatic NETs had associated pheochromocytomas, and 22 (88%) had no or mild pancreatic cystic disease, which is substantially more than the general population of patients with VHL disease. CONCLUSION: Pancreatic NETs in VHL have characteristic features at CT and MR imaging: Most are small, located in the pancreatic head, and enhance homogeneously. Tumors larger than 3.0 cm are prone to metastasize and enhance heterogeneously.

KW - Pancreas, CT

KW - Pancreas, cysts

KW - Pancreas, MR

KW - Pancreas, neoplasms

KW - Pheochromocytoma

KW - Von Hippel-Lindau disease

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