Limits...
Neuroendocrine tumours of the ampulla of Vater: clinico-pathological features, surgical approach and assessment of prognosis.

Dumitrascu T, Dima S, Herlea V, Tomulescu V, Ionescu M, Popescu I - Langenbecks Arch Surg (2012)

Bottom Line: Although frequent, lymph node metastases did not influenced both overall (p = 0.760) and disease-free survival rates (p = 0.745).No significant differences of survival were observed in patients with ENETS stage I/II disease, as compared to ENETS stage III disease (p = 0.169 and p = 0.137, respectively).The WHO 2010 classification appear to accurately predict patient prognosis, while the ENETS or UICC staging systems have a limited value (especially in regard to lymph node metastases).

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Center of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Fundeni Street no 258, Bucharest, Romania. traian.dumitrascu@srchirurgie.ro

ABSTRACT

Background/aims: Neuroendocrine tumours occur very rarely in the ampulla of Vater and their clinical behaviour is unknown. The aim of this study is to assess the clinico-pathological features, surgical approach and prognosis of these patients.

Methods: Six patients with neuroendocrine tumours of the ampulla of Vater treated with curative intent surgery at a single centre were retrospectively analysed. A univariate analysis of potential prognostic factors was also performed (data provided from the present study and literature review).

Results: Pancreaticoduodenectomy was curative in all the patients. Overall and disease-free survival rates were significantly better for G1/G2 tumours (p = 0.006 and p = 0.004, respectively). Although frequent, lymph node metastases did not influenced both overall (p = 0.760) and disease-free survival rates (p = 0.745). No significant differences of survival were observed in patients with ENETS stage I/II disease, as compared to ENETS stage III disease (p = 0.169 and p = 0.137, respectively). No differences were observed according to UICC staging system (p = 0.073 and p = 0.177, respectively). Tumours that are less than 2 cm or limited to the ampulla appear to have a better prognosis.

Conclusion: The WHO 2010 classification appear to accurately predict patient prognosis, while the ENETS or UICC staging systems have a limited value (especially in regard to lymph node metastases). Radical surgery (i.e. pancreaticoduodenectomy with lymphadenectomy) should be the standard approach in most patients with NET of the ampulla of Vater because this procedure removes all the potential tumour-bearing tissue.

Show MeSH

Related in: MedlinePlus

a Magnetic resonance cholangiopancreatography showing an ampullary soft tissue mass (T), with secondary dilatation of the common bile duct (c) and main pancreatic duct (arrow head); b endoscopic retrograde cholangiopancreatography showing an irregular, fusiform stenosis of the common bile duct toward the ampulla of Vater, with secondary dilatation; c endoscopic ultrasound revealing the ampulla with a 8 × 7 mm hypoechoic lesion (T), not invading the muscularis propria and with secondary dilatation of the common bile duct (c) and Wirsung's duct (W); d histological features (hematoxylin-eosin, original magnification ×40) revealing metastases into a locoregional lymph node of a G1 neuroendocrine neoplasm—nests of uniforms, polygonal tumour cells with round nuclei and “salt and pepper” chromatin
© Copyright Policy
Related In: Results  -  Collection


getmorefigures.php?uid=PMC3401309&req=5

Fig1: a Magnetic resonance cholangiopancreatography showing an ampullary soft tissue mass (T), with secondary dilatation of the common bile duct (c) and main pancreatic duct (arrow head); b endoscopic retrograde cholangiopancreatography showing an irregular, fusiform stenosis of the common bile duct toward the ampulla of Vater, with secondary dilatation; c endoscopic ultrasound revealing the ampulla with a 8 × 7 mm hypoechoic lesion (T), not invading the muscularis propria and with secondary dilatation of the common bile duct (c) and Wirsung's duct (W); d histological features (hematoxylin-eosin, original magnification ×40) revealing metastases into a locoregional lymph node of a G1 neuroendocrine neoplasm—nests of uniforms, polygonal tumour cells with round nuclei and “salt and pepper” chromatin

Mentions: Preoperative imaging included CT (two patients), MRI (three patients) or both (one patient). The CT and/or MRI showed the ampullary tumour in all cases; in patients with cholestasis syndrome, on noticed dilatation of the intra- and extrahepatic bile ducts, along with dilatation of Wirsung's duct (Fig. 1a). No distant metastases to the liver, lung, peritoneum or other organs were observed. Enlarged locoregional lymph nodes were observed in one patient on preoperative imaging.Fig. 1


Neuroendocrine tumours of the ampulla of Vater: clinico-pathological features, surgical approach and assessment of prognosis.

Dumitrascu T, Dima S, Herlea V, Tomulescu V, Ionescu M, Popescu I - Langenbecks Arch Surg (2012)

a Magnetic resonance cholangiopancreatography showing an ampullary soft tissue mass (T), with secondary dilatation of the common bile duct (c) and main pancreatic duct (arrow head); b endoscopic retrograde cholangiopancreatography showing an irregular, fusiform stenosis of the common bile duct toward the ampulla of Vater, with secondary dilatation; c endoscopic ultrasound revealing the ampulla with a 8 × 7 mm hypoechoic lesion (T), not invading the muscularis propria and with secondary dilatation of the common bile duct (c) and Wirsung's duct (W); d histological features (hematoxylin-eosin, original magnification ×40) revealing metastases into a locoregional lymph node of a G1 neuroendocrine neoplasm—nests of uniforms, polygonal tumour cells with round nuclei and “salt and pepper” chromatin
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3401309&req=5

Fig1: a Magnetic resonance cholangiopancreatography showing an ampullary soft tissue mass (T), with secondary dilatation of the common bile duct (c) and main pancreatic duct (arrow head); b endoscopic retrograde cholangiopancreatography showing an irregular, fusiform stenosis of the common bile duct toward the ampulla of Vater, with secondary dilatation; c endoscopic ultrasound revealing the ampulla with a 8 × 7 mm hypoechoic lesion (T), not invading the muscularis propria and with secondary dilatation of the common bile duct (c) and Wirsung's duct (W); d histological features (hematoxylin-eosin, original magnification ×40) revealing metastases into a locoregional lymph node of a G1 neuroendocrine neoplasm—nests of uniforms, polygonal tumour cells with round nuclei and “salt and pepper” chromatin
Mentions: Preoperative imaging included CT (two patients), MRI (three patients) or both (one patient). The CT and/or MRI showed the ampullary tumour in all cases; in patients with cholestasis syndrome, on noticed dilatation of the intra- and extrahepatic bile ducts, along with dilatation of Wirsung's duct (Fig. 1a). No distant metastases to the liver, lung, peritoneum or other organs were observed. Enlarged locoregional lymph nodes were observed in one patient on preoperative imaging.Fig. 1

Bottom Line: Although frequent, lymph node metastases did not influenced both overall (p = 0.760) and disease-free survival rates (p = 0.745).No significant differences of survival were observed in patients with ENETS stage I/II disease, as compared to ENETS stage III disease (p = 0.169 and p = 0.137, respectively).The WHO 2010 classification appear to accurately predict patient prognosis, while the ENETS or UICC staging systems have a limited value (especially in regard to lymph node metastases).

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Center of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Fundeni Street no 258, Bucharest, Romania. traian.dumitrascu@srchirurgie.ro

ABSTRACT

Background/aims: Neuroendocrine tumours occur very rarely in the ampulla of Vater and their clinical behaviour is unknown. The aim of this study is to assess the clinico-pathological features, surgical approach and prognosis of these patients.

Methods: Six patients with neuroendocrine tumours of the ampulla of Vater treated with curative intent surgery at a single centre were retrospectively analysed. A univariate analysis of potential prognostic factors was also performed (data provided from the present study and literature review).

Results: Pancreaticoduodenectomy was curative in all the patients. Overall and disease-free survival rates were significantly better for G1/G2 tumours (p = 0.006 and p = 0.004, respectively). Although frequent, lymph node metastases did not influenced both overall (p = 0.760) and disease-free survival rates (p = 0.745). No significant differences of survival were observed in patients with ENETS stage I/II disease, as compared to ENETS stage III disease (p = 0.169 and p = 0.137, respectively). No differences were observed according to UICC staging system (p = 0.073 and p = 0.177, respectively). Tumours that are less than 2 cm or limited to the ampulla appear to have a better prognosis.

Conclusion: The WHO 2010 classification appear to accurately predict patient prognosis, while the ENETS or UICC staging systems have a limited value (especially in regard to lymph node metastases). Radical surgery (i.e. pancreaticoduodenectomy with lymphadenectomy) should be the standard approach in most patients with NET of the ampulla of Vater because this procedure removes all the potential tumour-bearing tissue.

Show MeSH
Related in: MedlinePlus