Pancreatic neuroendocrine tumours (PNETs) in the context of multiple endocrine neoplasia syndrome (MEN) type 1 are rare, given the low prevalence of this syndrome. Their treatment is still controversial, both in terms of surgical indication and technique1. Although laparoscopy may offer advantages2,3, the multicentric nature of tumours in MEN 1 syndrome may limit its indications, and few studies have been published in the literature4–6. The aim of this study is to analyse our experience in a tertiary hospital of MEN 1 patients operated on for PNET by laparoscopic approach (1984–2020).
Out of 101 MEN 1 patients, 54% (n = 55) have presented with pancreatic pathology. Sixty-seven per cent (n = 37) underwent surgery, with corporocaudal pancreatectomy (CCP) performed in 53% (n = 36) of cases.
Laparoscopic surgery was performed in 14% (n = 5) of the patients. All had a single tumour in the tail and/or body of the pancreas according to the preoperative morphological study, and underwent splenic-preserving PCC. All patients underwent computed tomography (CT), echoendoscopy and octreoscan, with associated PET-Gallium scintigraphy since 2019. Table 1 shows the characteristics of these patients. The mean follow-up was 86.4 months (24–144). With regard to postoperative complications, patient 1 presented a range of symptoms that was resolved with antibiotic treatment and radiological drainage, and patient 3 presented a pancreatic fistula that was resolved with medical treatment with absolute diet, parenteral nutrition and somatostatin.
Patients operated on for pancreatic neuroendocrine tumour by laparoscopic approach.
| N | Year | Sex | Age | Site | Type of tumour | Tumour size (cm) | Histology | Stay (days) | F (months) | Rx |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 2018 | M | 41 | Body-tail | NF | 3.3 | G2 | 10 | 24 | No |
| 2 | 2008 | M | 58 | Tail and doubtful uptake in head-body approach | NF | 1.8 | G1 | 5 | 144 | Yes |
| 3 | 2015 | M | 28 | Tail | Insulinoma | 2.2 | G1 | 20 | 60 | No |
| 4 | 2008 | W | 65 | Body-tail | NF | 3 | G1 | 6 | 144 | No |
| 5 | 2015 | M | 64 | Tail | NF | 1.8 | G1 | 5 | 60 | No |
| Multicentre |
F: follow-up; G2: grade 1 degree of differentiation: well differentiated; 2: moderately differentiated; Hospital stay; M: man; N: Number of patient; year of surgical intervention; NF: not functioning; Rx: tumour recurrence; W: woman.
Patient 2 also had a 6 cm tumour in the left adrenal gland. The octreoscan image showed a high uptake in the pancreatic tail, with a doubtful uptake in the head-body. No tumour lesion was evident on CT imaging. PCC was performed together with adrenalectomy. At the age of 9 years, a tumour recurrence was seen on the CT scan, in the location where the octreoscan image was captured; the patient was reintervened and an open enucleation was performed.
The benefits of laparoscopic surgery are well known in tumours of the pancreatic body and tail, specifically described in PNETs2,3,7. In MEN 1 patients, it should be evaluated, as these tumours are usually located in the pancreatic tail, although a significant percentage may be multicentric8. Few studies have been published on this approach in MEN14–6. The study by López et al. compares open versus minimally invasive approaches5, showing results similar to those described in the meta-analysis by Drymousis et al.2 in terms of shorter hospital stay, less blood loss, and no differences in the percentage of pancreatic fistulas5.
More than half of the patients had a length of stay of less than one week, less than previously described5. In relation to pancreatic fistulas, the percentage described varies from 29% to 62%2,5,6, although no differences are found according to the type of approach, only a higher number of fistulas in cases of enucleation9. In our case, the percentage was 20%, although there were no cases with enucleation, and in relation to overall complications, the percentage was similar to that of other studies (40%)2.
Another aspect to take into account in our series is that in 100% of the patients it was possible to perform splenic preservation without complications, which seems to be more feasible in minimally invasive surgery5.
However, the aspect we consider most important is the possibility of multicentricity and recurrence in these patients8. In this regard, the preoperative study is important, as currently, with the introduction of echoendoscopy and Ga PET, sensitivity is close to 100% in tumour detection, and allows us to better select for a laparoscopic approach. In addition, intraoperative ultrasound also allows for better assessment, and is currently a test that we use routinely10.
Another point to take into account is the functionality or non-functionality of the tumour and the type of secretion. Both non-functioning tumours and insulinomas tend to appear in the pancreatic tail-body, and a laparoscopic approach could be considered, as reflected in our results. However, the indication for more aggressive tumours with a preferential location in the pancreatic head or even duodenum, as in the case of gastrinoma, would be more controversial1.
In the only study performed in MEN 1 patients comparing recurrence according to the approach, the rate was higher in cases of open surgery, but the follow-up was lower in cases of minimally invasive surgery5. On the other hand, it should be noted that the laparoscopic approach in these patients offers the advantage of a lower percentage of postoperative adhesions, which may facilitate surgery in case of reoperation2,3,7.
In conclusion, the laparoscopic approach to pancreatic surgery for non-functioning tumours and insulinomas in patients with MEN 1 is feasible, and can be considered in selected patients with a complete preoperative work-up showing single tumours in the pancreatic corpus coli or multicentric tumours not located in the pancreatic head that allow a laparoscopic PCC to be performed with confidence.
FundingThis study did not receive any type of funding.
