Potential value of EUS in pancreatic surveillance of VHL patients

Abstract

Background: Patients with von Hippel-Lindau (VHL) disease are prone to develop pancreatic neuroendocrine tumors (pNETs). However, the best imaging technique for early detection of pNETs in VHL is currently unknown. In a headto-head comparison, we evaluated endoscopic ultrasound (EUS) and 11 C-5-hydroxytryptophan positron emission

Potential role of 68Ga-DOTATOC PET/CT in screening for pancreatic neuroendocrine tumour in patients with von Hippel-Lindau disease

“…Indeed, with PET/MRI machines now clinically available, future vHLD screening should be performed with 68 Ga-DOTATOC PET/MRI rather than 68 Ga-DOTATOC PET/CT to reduce the radiation exposure in genetically susceptible patients. Endosonography (EUS) is another imaging tool that has been shown to be superior to octreoscan and 11 C-5-HTP PET/CT in the detection of pNETs in vHLD [10]. The major advantage of EUS is that it is radiation-free and allows simultaneous fine-needle aspiration cytology for confirmation of pNET.…”

“…Previous studies have shown that there is a very limited or no role for 18 F-FDG, 18 F-DOPA and 11 C-5-HTP PET in the detection of pNET in vHLD [10,12]. In vHLD the National Cancer Network (NCCN) guidelines recommend three-phase CT or MRI for the diagnosis of pNET [10,13]. For further characterization of pancreatic lesions somatostatin receptor (SR) scintigraphy as a functional imaging tool and tumour markers chromogranin A (CgA) and/or pancreatic polypeptide may be considered.…”

“…The recently observed improved overall survival of patients with vHLD has been attributed to improved screening methods for haemangioblastoma, renal clear cell carcinoma and phaeochromocytoma. These highly sensitive screening methods may also increase the detection and thus the prevalence of pNET in vHLD [10].…”

“…The repeated use of highly sensitive and accurate imaging methods for prognosis, in combination with other predictors of malignancies such as germline mutation in the vHLD gene located on exon 3, and prognostic parameters such as tumour size and doubling time are critically needed [11]. The best imaging method for screening for pNETs is still under discussion [10]. Multiple cystic pancreatic lesions pose a challenge in the detection of small solid pNETs on CT or MRI [1,8,9,12].…”

“…Multiple cystic pancreatic lesions pose a challenge in the detection of small solid pNETs on CT or MRI [1,8,9,12]. Previous studies have shown that there is a very limited or no role for 18 F-FDG, 18 F-DOPA and 11 C-5-HTP PET in the detection of pNET in vHLD [10,12]. In vHLD the National Cancer Network (NCCN) guidelines recommend three-phase CT or MRI for the diagnosis of pNET [10,13].…”