Pancreatic cystic manifestations in von Hippel-Lindau disease

Roberto Girelli, Claudio Bassi, Massimo Falconi, Lucia De Santis, Antonio Bonora, Erminia Caldiron, Naro Sartori, Roberto Salvia, Gianfranco Briani, Paolo Pederzoli

Research output: Contribution to journalArticle

31 Citations (Scopus)

Abstract

Conclusion: In view of the frequent absence of symptoms related to pancreatic lesions, screening tests for VHL should always include assessment of the pancreas and, considering the frequency of polycystic manifestations, VHL should always be borne in mind in the differential diagnosis of multiple pancreatic cysts, especially when occurring in young patients and in the absence of a positive history of pancreatic disease. Background: Von Hippel-Lindau disease (VHL) is a hereditary disease transmitted with an autosomal dominant character and characterized by hemangioblastomas of the central nervous system and retina, renal tumors and cysts, and pheochromocytoma. Pancreatic manifestations of VHL are reported in the literature with incidences ranging from 16 to 29% of cases and consist mainly in cystadenomas of the serous type and in multiple cystic lesions, often with complete replacement of the gland. Methods and Results: We report five cases of VHL with a polycystic pancreas as the main or only manifestation, all devoid of symptoms related to involvement of the pancreas, who were referred to our Pancreatic Surgery center with diagnoses of multiple pancreatic pseudocysts of undefined origin.

Original languageEnglish
Pages (from-to)101-109
Number of pages9
JournalInternational Journal of Pancreatology
Volume22
Issue number2
DOIs
Publication statusPublished - Oct 1997

Fingerprint

von Hippel-Lindau Disease
Pancreas
Serous Cystadenoma
Pancreatic Cyst
Hemangioblastoma
Pancreatic Pseudocyst
Pancreatic Diseases
Inborn Genetic Diseases
Pheochromocytoma
Retina
Cysts
Differential Diagnosis
Central Nervous System
Kidney
Incidence
Neoplasms

Keywords

  • pancreatic cystadenoma
  • pancreatic cysts
  • Von Hippel-Lindau disease

ASJC Scopus subject areas

  • Oncology
  • Gastroenterology
  • Endocrinology

Cite this

Girelli, R., Bassi, C., Falconi, M., De Santis, L., Bonora, A., Caldiron, E., ... Pederzoli, P. (1997). Pancreatic cystic manifestations in von Hippel-Lindau disease. International Journal of Pancreatology, 22(2), 101-109. https://doi.org/10.1007/BF02787467

Pancreatic cystic manifestations in von Hippel-Lindau disease. / Girelli, Roberto; Bassi, Claudio; Falconi, Massimo; De Santis, Lucia; Bonora, Antonio; Caldiron, Erminia; Sartori, Naro; Salvia, Roberto; Briani, Gianfranco; Pederzoli, Paolo.

In: International Journal of Pancreatology, Vol. 22, No. 2, 10.1997, p. 101-109.

Research output: Contribution to journalArticle

Girelli, R, Bassi, C, Falconi, M, De Santis, L, Bonora, A, Caldiron, E, Sartori, N, Salvia, R, Briani, G & Pederzoli, P 1997, 'Pancreatic cystic manifestations in von Hippel-Lindau disease', International Journal of Pancreatology, vol. 22, no. 2, pp. 101-109. https://doi.org/10.1007/BF02787467
Girelli, Roberto ; Bassi, Claudio ; Falconi, Massimo ; De Santis, Lucia ; Bonora, Antonio ; Caldiron, Erminia ; Sartori, Naro ; Salvia, Roberto ; Briani, Gianfranco ; Pederzoli, Paolo. / Pancreatic cystic manifestations in von Hippel-Lindau disease. In: International Journal of Pancreatology. 1997 ; Vol. 22, No. 2. pp. 101-109.
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