A successful multivascular resection with reconstruction of the major parapancreatic arteries so extensively performed to assist total duodenopancreatectomy has not been described before. That is not only attributable to non-functioning PNTs being so exceptionally seldom encountered[2
], the absence of distant metastases at the time of such a sizable G3 tumor diagnosis[3
] and reluctance to resort to challenging surgical aggression for locally advanced tumors, but is also due to the rarity of the above-described celiaco-mesenteric arterial anatomy. The CHA arising from the SMA is a remarkably unusual occurrence per se
(hepato mesenteric trunk, Michels type 9), reported by Michels in 4.5 % (18) and by others in 2.1%-8% of cases[20
]. The aberrant arterial anatomy observed in the case under discussion is many times so much so extraordinary owing to the exquisiteness of the CHA having a transpancreatic course[38
]. It was precisely these considerations that accounted for and required resection of such a substantial stretch of the arterial vasculature.
Review of the literature suggests the advantages of surgical management of locally advanced pancreatic neuroendocrine cancer[40
]. However, the dissimilarities between the analyzed groups (different stages of the disease, mixed functioning and non-functioning PNTs) and diversity of multi-modality adjuvant therapies preclude this inference from being completely valid, that is to say, the prolonged survival including adequate life quality and acceptable mortality and morbidity rates for these patients has yet to be demonstrated[8
There can still be only scant foreknowledge of the evolution of PNTs in spite of the development, adoption and perpetual updating of tumor staging and histopathologic evaluation systems and classification schemes[8
]. Although in most instances conventional markers do correlate with prognosis[44
], tumor heterogeneity of pancreatic NECs invites further investigation into their biology.
Poorly differentiated and disseminated NECs carry a dismal prognosis[8
]. Therefore preoperative diagnostic work-up and assessment of the extent of tumor spread are of critical importance. Radionuclide procedures such as somatostatin receptor scintigraphy (with 111
Ga-DOTATOC positron emission tomography (PET), 111
In-DTPAOC single photon emission computed tomography and F-DOPA PET are essential facets in extension determination of neuroendocrine neoplasm and NEC (including extrahepatic spread) and detection of somatostatin receptors. We had no way of using these reagents from the fact that none of them had been registered in Russia by the time of the patient’s presentation. Just then the somatostatin analogue manufactured in Russia (111
In-octreotide) was undergoing clinical trials and was likewise unavailable for use. Taking into account the above state of affairs we reasoned that the CT and hepatocyte-specific contrast-enhanced MRI evidence obtained would suffice to evaluate the extent of tumor involvement. We believe it is wise that surgery in patients with locally advanced poorly differentiated NEC should unfailingly be preceded by neoadjuvant therapy. However, in this instance we were unsuccessful in our attempts to collect enough material by fine-needle aspiration to reliably judge the degree of tumor differentiation.
It is still not clear why, despite the enormousness of the poorly differentiated NEC and so high a proliferative index in a patient with MEN-1 syndrome[14
], there were no metastases detected both prior and a year after the surgery. The latest recommendations made by the European Neuroendocrine Tumor Society espouse an aggressive surgical approach to locally advanced PNTs with mandatory high patient selection, no circumferential encasement of the SMA and the superior mesenteric vein and PV being out of circumferential tumor contact in the absence of portal cavernoma[8
The clinical description of the case under review was only partially consistent with these recommendations, and yet skipping neoadjuvant therapy in favor of upfront surgery was motivated by the totality of a number of reasons: constant rather refractory pain syndrome, inability to eat normally because of frequent vomiting , progressive rapid weight loss, young age, hugeness of the tumor, invaded arteries and absence of distant metastases were the rationale behind the patient’s having no alternative treatment option but to undergo surgical therapy.
The above case shows combined duodenopancreatectomy with multiarterial resection to be safe enough when carried out in such a step-by-step order as to prevent prolonged bowel and liver ischemia, after thorough preoperative planning, with the surgeon’s experience and skill in extended pancreatectomy.
Choosing the strategy of treatment for locally advanced PNTs is most difficult every time and the realistic assessment of operability and resectability still presents a challenge even to an expert. As regards deciding on the policy of treatment, the preoperative determination of the degree of tumor differentiation is crucial in choosing between heroic surgery and a neoadjuvant approach, although even with this clue in hand, opting for either of them still poses a dilemma. Besides the tumor biology and technical feasibility of the procedure, the patient’s ability to come through and the surgeon’s capability to deal with foreseeable postoperative sequelae, postsurgical life quality as well as potential benefits from available alternative therapies and previous experience with extended pancreatectomy and vascular resection as its part must be taken into consideration[8