Tumors of the pancreas are rare among children, and there are few signs that enable the diagnosis to be made before operating. Laparoscopy for pancreas surgery was initially described experimentally in a pig model by Soper in 1994 [13] and later applied in human adults. We describe the application of this technique to a child.

We report the case of a 9-year-old boy with a Frantz’s tumor who was treated with a laparoscopic spleen-preserving distal pancreatectomy.

Case report

An ultrasound examination of a 9-year-old boy who presented with paraumbilical abdominal pain revealed a pancreatic mass, 2 cm in diameter, at the body–tail junction of the pancreas. He had complained of this pain two or three times per month over the past 2 months. His general state was not altered, and his weight was stable. Bowel transit was normal, without diarrhea. There was no signs of hypoglycemia. Results of the clinical exam were normal. Results of complementary exams, including conventional blood tests, thyroid hormone levels, serum lipase and amylase levels, and tumor markers (CEA, CA 19-9), were all normal. The CT scan showed a solid 2-cm, mass localized in the pancreas tail. It was not accessible to a percutaneous needle biopsy. We decided to perform a laparoscopic biopsy and to proceed to a resection, if needed. Prior to surgery, patient consent was obtained for both a laparoscopic and an open approach.

The intervention was made under general anesthesia. The patient was placed in supine and reverse Tredelenburg position, with the surgeon standing between the patient’s legs. The screen was on the left side of the patient’s head. A four-port laparoscopic technique was used. A 10-mm camera port was placed at the umbilicus. Three additional working 5-mm ports, along with a 2-mm port for the linear stapling device, were placed in the xyphoid area, left flank, and right flank, respectively. Electrocautery and Harmonic Scalpel were used for dissection. A wide window was made in the gastrocolic omentum; the lesser sac was entered through this window. A forceps was used to elevate the stomach from the epigastric trocar, enabling good exposure of the body and tail of the pancreas. We added a transcutaneous suture-assisted gastric retraction. The inferior border of the pancreas was dissected and the tail detached from the retroperitoneum. This mobilization enabled us to visualize the posterior wall of the gland. A tumor was found on the posterior face of the gland, just above the splenic vein. The mass was separated from the splenic vein and a wedge biopsy performed. On frozen section, it was identified as an epithelial tumor of the pancreas. Consequently, we decided to perform a complete resection of the tumor.

The friable consistency of the tumor led us to perform a distal pancreatectomy (rather than enucleation). The splenic vein was freed up to the distal extremity of the tail of the pancreas. This was the most delicate surgical step. The remaining spleen had a healthy appearance and was therefore preserved. The pancreatectomy was performed by transection, using an endoscopic linear stapler (Tyco, Elancourt, France). The dissected part was extracted in an endoscopic bag retrieval system through a minimally enlarged umbilical trocar incision. A drain had been placed and drawn out through one of the 5-mm port sites. Operative time was 3 h 30 min, including loss of time to localize the tumor.

The follow-up was uneventful, with recovery of alimentation and normal serum amylase level at postoperative day 2. The boy was discharged on postoperative day 6. Six months later, the control CT scan was normal. The final pathologic diagnosis was solid pseudopapillary tumor (Fig. 1), with complete resection. The lymph nodes were free of disease.

Figure 1
figure 1

Solid cystic papillary tumor consisting of solid sheets of uniform cells with eosinophilic or vascuolated cytoplasm and ovoid indented nuclei (inset). Hematoxylin and eosin staining.

Full size image

Discussion

To our knowledge, this is the first report of a laparoscopic pancreatectomy for a solid tumor in a child. There are only a few published adults series, all with a very limited number of cases.

Malignant tumors of the pancreas are rare in infants and children, and they have a more favorable outcome than those in adults. Tumors can be secreting or not. In secreting tumors, the clinical signs indicating hormonal secretion are adequate to establish the diagnosis. In our case, the boy had a nonsecreting solid tumor. This solid pseudopapillary tumor—also called “solid cystic tumor”, “papillary cystic and solid tumor,” or “Frantz’s tumor”—is most frequently discovered in young women in their 2nd or 3rd decade (mean age, 21 years). It constitutes a distinctive and unusual clinicopathologic entity and was first described by Frantz in 1959 [5]. It is usually benign, sometimes of low-grade malignancy, and has a slow evolution. The tumor is often large (10 cm) and encapsulated, and it occurs more frequently in the body and tail of the gland [2, 6, 8, 15]. The recommended treatment is still debated: either complete resection or, for tumors with a distal localization, pancreatectomy with spleen preservation. After the resection, the prognosis is favorable [9]. No chemotherapy or radiotherapy is necessary. However, the tumor may recur after 1–10 years [6], in a metastatic form. Therefore, all such patients should be submitted to prolonged follow-up.

Laparoscopic pancreatic resection was initially described in both experimental and human studies in the early 1990s. Thereafter, thanks to improvements in instrumentation, such as the development of endoscopic staplers, and expanded training in laparoscopy, great strides have been made in the treatment of adults. Yet laparoscopic pancreatic surgery has been slow to gain acceptance, and its role in pancreatic disease is still unclear.

Pediatric cases are rare. A partial pancreatectomy has been described in a 4-week-old baby with persistent hyperinsulinemic hypoglycemia. He required a complementary open surgery within a few days [1]. Nevertheless, the main import of that cases was that it demonstrated the feasibility of the technique, even in a low-weight infant (4.9 kg). Distal pancreatectomy for blunt injury of the pancreas in a 10-years-old boy has also been reported [12]. Procedures have been done without prolonged operative time or unnecessary risk.

The published series in adults include the following areas of investigation and treatment: staging for malignant pancreatic tumors, laparoscopic management of pseudocysts, enucleation for islet cell tumors, and partial pancreatectomies—more specifically, caudal surgery for suspected neuroendocrine tumors and benign cystic tumors [4, 6, 7, 10, 14]. During open procedures, access to the pancreas requires wide exposure. The laparoscopic approach offers the advantage of large magnification and facilitates many procedures in this organ with difficult access. Laparoscopic resection of the left pancreas is a recent procedure. It offers benefits to the patient in terms of an expedient postoperative recovery, coupled with acceptable rates of perioperative morbidity and complication. This operation is well suited to the laparoscopic approach because of the absence of anastomosis. The magnified view of the operative field facilitates separation of splenic vein and artery from the pancreatic parenchyma. However, the published series are based on a relatively few cases and operating times are relatively long (mean, 4.5 h) [3].

The main complication is pancreatic fistula (8–30%). No perioperative deaths have been reported, a equivalent to the open procedure. Splenic conservation was feasible in 76% of the cases. The fistula rate and overall morbidity was comparable to recent large open series of distal pancreatectomy. The first laparoscopic pancreatoduodenectomy was done in 1994, and caudal pancreatectomy was first proposed in 1996. The conversion rate is presumably dependent on the surgical team’s experience with the procedure.

This technique should be considered an advanced laparoscopic procedure; surgical technique for its application in pediatric cases is still in the development phase. An exploration of variations on the technique and classification of the operative indications will follow. Only surgical teams with advanced laparoscopic skills should attempt to perform it [4].

Conclusion

We report a laparoscopic distal pancreatectomy in a 9-year-old boy. The technique used for this advanced surgical case was simple and reproducible, was completed safely within a reasonable operative time, and yielded a good result. Obviously, we cannot draw firm conclusions from our preliminary results, and further evaluation is mandatory, but as experience with this technique continues to grow, laparoscopic distal pancreatectomy may well become the approach of choice in selected patients.

Since we managed the reported case, one of our patients has presented with a recurrence. A girl treated with immunosuppression therapy after kidney transplantation developed a Frantz’s tumor at the age of 12 years. To eliminate a lymphphoma, a laparoscopic biopsy of the tumor was performed. Then the resection was conducted using a laparotomy. Two years later, at the age of 14 years, the child has developed a recurrence in the form of a peritoneal carcinomatosis. At this stage, we cannot rule out the possibility that the laparoscopic biopsy has contributed to this evolution. Consequently, we have stopped using the laparoscopic approach for solid tumors of the pancreas until we have cleared up the role of laparoscopy in the recurrence.