Laparoscopic total pancreatectomy for multiple endocrine neoplasia ...

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Asian J Endosc Surg ISSN 1758-5902

CASE REPORT

Laparoscopic total pancreatectomy for multiple endocrine neoplasia type 1 syndrome-associated multifocal, non-functioning pancreatic neuroendocrine tumor: A case report Palanisamy Senthinathan, Samrat Vijaykumar Jankar, Sandeep C Sabnis, Vivek Kaje, Sivakumar Srivatsan Gurumurthy, Natesan Anand Vijai, Vaiyapurigounder Palanisamy Nalankilli, Palanivelu Praveen Raj, Ramakrishanan Parthasarathi, Subbiah Rajapandian & Chinnusamy Palanivelu Department of Hepatobiliary and Pancreatic Surgery, Gem Hospital and Research Centre, Coimbatore, Tamil nadu, India

Keywords: Laparoscopic total pancreatectomy; MEN1 syndrome; pancreatic neuroendocrine tumor Correspondence Sandeep C Sabnis, Department of Hepatobiliary and Pancreatic Surgery, Gem Hospital and Research Center, Coimbatore, Tamil Nadu, India. Tel: +91 422 232 5100 Fax: +91 422 2324879 Email [email protected]

Abstract Pancreatic neuroendocrine tumors are rare, accounting for less than 3% of all pancreatic tumors. Although laparoscopic pancreas-preserving surgery for managing sporadic pancreatic neuroendocrine tumors has been described in the literature, laparoscopic total pancreatectomy has rarely been reported. We present a 30-year-old man who was incidentally diagnosed with multiple endocrine neoplasia type 1 syndrome with parathyroid hyperplasia and a nonfunctioning pancreatic neuroendocrine tumor. He underwent laparoscopic total pancreatectomy with splenectomy. This report highlights the technical details of laparoscopic total pancreatectomy, which appears to be a feasible and safe option in select cases.

Received 24 January 2017; revised 12 April 2017; accepted 13 April 2017 DOI: 10.1111/ases.12390

Introduction

Case Presentation

In 1943, the first case of total pancreatectomy (TP) for cancer was reported (1). The procedure was initially criticized because of its extensiveness, the patient’s immediate postoperative metabolic disturbances, and the lifelong implications that were expected after the removal of the entire pancreas. Although localized resections with negative margins are standard for most lesions, TP is increasingly being used as a potential option for certain conditions such as diffuse intraductal papillary mucinous neoplasms, multifocal neuroendocrine tumors, and chronic pancreatitis (2). The last two decades have seen enormous improvements in surgical techniques, as well as new minimally invasive technologies and improved postoperative care, all of which has contributed to reduced morbidity and mortality. Herein, we report the case of 30-year-old man who was diagnosed with a multiple endocrine neoplasia type 1 (MEN-1) syndrome-associated multifocal, non-functional pancreatic neuroendocrine tumor (PNET) and underwent laparoscopic TP.

This patient was incidentally diagnosed with MEN-1 syndrome during an examination for recurrent renal calculi associated with unexplained hypercalcemia and multiple cutaneous collagenomas. Serum parathormone and calcium levels were raised. Parathyroid scintigraphy showed parathyroid hyperplasia. Brain MRI was normal. CT abdomen showed multiple pancreatic lesions, with largest in the head (3.2 × 2.6 cm) (Figure 1). Multiple nodal enlargements were noted around the pancreas, hepatoduodenal region, and splenic hilum; the largest was 2.2 × 1.7 cm. Octreotide receptor scintigraphy (Gallium 68 - DOTATOC (Ga68-DOTATOC) PET scan) demonstrated multiple lesions all over the pancreas with nodal involvement. The patient’s serum gastrin, insulin, and chromogranin A levels were normal. Endoscopic ultrasound-guided fine needle aspiration from the pancreatic masses and the lymph nodes were suggestive of a well-differentiated neuroendocrine tumor (World Health Organization grade II). Two weeks before surgery, the patient received vaccination against capsular organisms.

Asian J Endosc Surg •• (2017) ••– •• © 2017 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd

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P Senthinathan et al.

Lap TP for MEN-1–associated PNET

Figure 1 Contrast-enhanced abdominal CT. Coronal section showing multiple lesions in the pancreas. a, head; b, body; c, tail; d, nodal mass in hepatoduodenal area.

Preoperative calcium levels were well controlled by medical treatment. He underwent laparoscopic TP with splenectomy. The initial postoperative period was characterized by episodes of ketosis, which were managed with hydration and insulin infusion. The patient was discharged on postoperative day 9, with long-acting insulin and pancreatic enzyme supplements. His final histopathological report was suggestive of American Joint Committee for Cancer stage T3N1M0, with a World Health Organization grade II neuroendocrine tumor (KI index 7%) (3,4). The procedure followed was in accordance with the ethical standards of the institution’s committee on human experimentation, the Declaration of Helsinki (1964 and later version, and

the Good Clinical Practice standards. Informed consent was obtained from the patient before the procedure and for inclusion in the study. Patient anonymity has been preserved. Operative technique The patient was placed in semi-lithotomy position with his legs apart. The surgeon operated while standing in between the patient’s legs. The camera operator and the retraction assistant stood to the left of the surgeon. The scrub nurse stood to the right of the surgeon. After pneumoperitoneum was created by a closed Veress needle technique, the ports were placed as shown in Figure 2.

Figure 2 Port positions. a, 10-mm supra-umbilical port for the camera; b, 5mm subxiphoid port for liver and stomach retraction; c, 10–13-mm lower right midclavicular (lower) working port (used for stapler as well); d, 5-mm left midclavicular port for colon retraction; e, 5-mm upper right midclavicular working port; f, 5-mm upper left midclavicular working port; g, percutaneous sling made of suture material to lift the falciform ligament.

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Asian J Endosc Surg •• (2017) •• –•• © 2017 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd



Phase I: Assessment and resection (Figure 3)

Staging laparoscopy was performed to rule out metastatic deposits, and findings were confirmed with intraoperative ultrasonography. The multifocality of the lesion necessitated removal of the entire pancreas. The first step was an extended Kocher maneuver to enable visualization of the superior mesenteric artery. A tunnel was created between the superior mesenteric vein and the posterior surface of the pancreatic neck. The distal part of the stomach was then divided with an ECHELON FLEX stapler (Ethicon Endo Surgery, Somerville, New Jersey, USA). The common bile duct was divided just proximal to insertion of cystic duct. Nodal basins around the hepatoduodenal ligament, along hepatic artery and celiac axis, were retrieved. At this stage, the gastroduodenal artery was clipped and divided at its origin. Division of the ligament of Treitz facilitated the mobilization of the duodeno-jejunal flexure. The jejunum was divided 10 cm distal to the duodeno-jejunal flexure. The splenorenal ligament was divided, and the splenic vein and artery were doubly clipped. Splenic preservation was not attempted because of dense adhesions between the splenic vein and the posterior surface of the pancreas. After division of the short gastric vessels and the inferior mesenteric vein, en-bloc resection involving the pancreas, duodenum, spleen, and peripancreatic lymph nodes was completed (Figure 4). Phase II: Reconstruction

Roux-en-Y anastomosis was performed using the short limb for gastrojejunostomy and long limb for hepaticojejunostomy. A small caliber common duct was managed with an anterior slit and then end-to-side

Figure 4 Resected specimen showing part of the duodenum, total pancreas, and spleen.

anastomosis. The endobag containing the resected specimen was retrieved through a 4–5-cm Pfannenstiel incision. Two drains were placed. The procedure was completed in 380 min, with blood loss of 250 mL.

Discussion TP has been a mostly underused procedure for last century. Surgeons have been wary of the procedure because of its extensiveness, perioperative morbidity, and postoperative lifelong implications such as diabetes and pancreatic exocrine insufficiency. The last two decades have witnessed revived interest in TP, mainly for conditions such as chronic pancreatitis, hereditary pancreatitis, trauma, and in certain cases, intraductal papillary mucinous neoplasm, PNET, and adenocarcinoma. Emerging concepts favor use of underlying disease biology to predict long-term survival for TP patients rather than using metabolic alterations caused by hormonal imbalances (1).

Figure 3 Pancreatic bed after total pancreatectomy. a, portal vein; b, inferior vena cava; c, gastroduodenal artery stump; d, bulldog clamp over the CBD; e, splenic vessel stump.

Asian J Endosc Surg •• (2017) ••– •• © 2017 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd

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Although PNET are rare, the non-functional types are most common. Generally, PNET associated with familial conditions like MEN syndromes are considered non-aggressive, but they are often multifocal, which makes them more likely to be malignant (5). The incidence of PNET in patients with MEN-1 varies between 30% and 70%. The clinical practice guidelines for MEN-1 suggest considering surgical resection for multifocal, non-functional PNET that are larger than 1 cm (6). Margin-negative resection with complete removal of tumor tissues offers advantages in terms of reduced metastasis and decreased cancer-related mortality (7). The recent literature had alleviated concerns about the long-term morbidity in post-TP patients. For example, in a series of 77 patients, the overall diabetes-related quality of life for TP patients was acceptable and comparable to that of patients who had undergone pancreas-preserving surgery (8). Laparoscopic pancreatic surgery represents one of the most demanding fields in surgery. It has grown over the last two decades, with complex operations such as pancreaticoduodenectomy, distal pancreatectomy, and central pancreatectomy being performed successfully by skilled laparoscopic surgeons (9). TP combines the operative steps of distal pancreatectomy and pancreaticoduodenectomy, but it lowers the risk of pancreatic stump-related morbidity. Initial reports of TP via minimally invasive approaches have been encouraging, although they have been performed in select patients (10). In patients with multifocal, locally advanced PNET, radical surgery with a negative margin should provide the best survival benefits. The traditional benefits of the laparoscopic approach, when performed by an expert surgeon, can translate to minimally invasive TP, making it feasible and safe option in select cases.

Acknowledgments The authors have no financial conflicts of interest to disclose.

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Asian J Endosc Surg •• (2017) •• –•• © 2017 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and John Wiley & Sons Australia, Ltd