Case of 41 year old patient with MEN1 syndrome and three tumours (non-functional NETs) located in the head 17mm, body (65mm) and tail (35mm) of the pancreas; what would you suggest...total pancreatectomy or distal splenopancreatectomy with tumorectomy in the head of the pancreas? CT screenshots are bellow.
There is no indication for surgery. In these cases is better choice to do ablation , vascular obliteration or chemotherapy as main options
Since these tumors seems to be malignant (by the size) I suggest a total pancreatectomy since these tumors in MEN syndrome may recur after partial resection as I have observed in some of my cases .By the other side a PETCT with Gallium 68 Dota is useful to define the presence of lymph nodes metastasis and therefore the extent of resection
Åkerström G. et al.(2012) submitted that Pancreatico-duodenal tumors are the second most common endocrinopathy in multiple endocrine neoplasia syndrome type 1, and have a pronounced effect on life expectancy as the principal cause of disease-related death. Previous discussions about surgical management have focused mainly on syndromes of hormone excess and, in particular, the management of multiple endocrine neoplasia syndrome type 1-related Zollinger-Ellison syndrome. Since hormonal syndromes tend to occur late and indicate the presence of metastases, screening with biochemical markers and endoscopic ultrasound is recommended for early detection of pancreatico-duodenal tumors, and with early surgery before metastases have developed. Surgery is recommended in patients with or without hormonal syndromes in the absence of disseminated liver metastases. The suggested operation includes distal 80% subtotal pancreatic resection together with enucleation of tumors in the head of the pancreas, and in cases with Zollinger-Ellison syndrome, excision of duodenal gastrinomas together with clearance of regional lymph node metastases. This strategy, with early and aggressive surgery before metastases have developed, is believed to reduce the risks for tumor recurrence and malignant progression.
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Article Surgical management of pancreatico-duodenal tumors in multip...
It is difficult to decide the best option. Usually you can study every case carefully and take the final decision in multi-specialists meeting to be the most appropriate for every case
I agree with Mr.Marcel C Machado :
- A total pancreatectomy could be an appropriate approach in this case due to the multifocal NETs appeared not only at the pancreatic head and contained high-risk of recurrence.
- The accuracy of PET-CT with Gallium 68 to detect the presence of lymph nodes metastasis is not very high; therefore, if you choose an aggressive way, do not mainly rely on this diagnostic technique.
- On the other hand, the ablation or vascular obliteration approaches could not be suitable in this case due to the great blood vessels of the pancreas. Chemotherapy could be beneficial in cased of advanced or metastasis. However, we do not have a standard protocol, and it also requires reliable histological identification to achieve maximal efficacy.
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