Strategy for surgical treatment of intraductal papillary-mucinous tumors

The prognosis of malignant intraductal papillary mucinous tumors of the pancreas (IPMTs) should be considered more favorable than that of ordinary pancreatic ductal carcinoma. However, the preoperative diagnosis of malignancy is extremely difficult in IPMT. IPMT with a main pancreatic duct of less than 7 mm, or cystic lesion of less than 30 mm (branched type), or intramural nodule tumor of less than 4 mm, should be observed without performing surgery and followed carefully. The strategy for surgical treatment of IPMT is very important. Which procedure should be selected or performed? A radical operation should be performed with lymph node dissection if a preoperative diagnosis of malignancy is made. If cancer cannot be ruled out in an IPMT, a function-preserving procedure, such as pylorus-preserving pancreaticoduodenectomy, pancreatic head resection with second-portion duodenectomy, segmental resection, partial resection, or spleen-preserving distal pancreatectomy should be selected, and one of these procedures should be carried out with group I lymph node dissection. The greatest challenge in IPMT is making the diagnosis of benign or malignant and selecting the most appropriate treatment.     

IHPBA in Tokyo, 2002: surgical treatment of IPMT vs MCT: a Japanese experience

The differences and similarities between intraductal papillary mucinous tumor (IPMT) and mucinous cystadenoma or carcinoma (mucinous cystic tumor; MCT) of the pancreas have been noted. The similarities include: (1) both tumors originate from pancreatic duct cells, (2) massive mucin production is found in both tumors, and (3) papillary projection is a common histological characteristic. However, there are also many differences. IPMT is most frequently found in men in their sixties, and originates in the head of the pancreas, with 62% (123/199) of tumors reported to be found in the head of the pancreas. This tumor sometimes spreads throughout the entire pancreas. The tumor itself basically is of the dilated pancreatic duct type, and the prognosis is generally good. In contrast, MCT frequently develops in women in their forties. This tumor is usually large, round, and almost totally encapsulated by fibrous tissue, with no communication with the pancreatic duct. The tumor histologically has an ovarian-like stroma. It most often develops in the body or tail of the pancreas. Invasion is often present and the operative prognosis is not good. IPMT resembles the shape of a bunch of grapes and MCT resembles that of an orange. From the differences between these two types of tumors, they are classified into different categories. With regard to therapeutic strategies for MCT, the tumor should be resected with lymph node dissection immediately when it is detected. In contrast, some patients with branch-type IPMT can be followed without surgical procedures. Because IPMT shows good prognosis and little tendency for infiltration, some kinds of organ-preserving procedures would be possible for some patients with this tumor. Such organ-preserving procedures are: duodenum-preserving pancreas head resection, spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein, and so on.     

Pancreatic head resection with segmental duodenectomy for intraductal papillary mucinous tumors of the pancreas

Various modifications of organ-preserving pancreatic resections have been performed for intraductal papillary mucinous tumor (IPMT) of the pancreas. The aim of this study was to evaluate usefulness of pancreatic head resection with duodenal segmentectomy (PHRSD), which is one of the organpreserving pancreatic resections for IPMT. Pancreatic head resection with duodenal segmentectomy was indicated for the branch duct type of IPMT. Eight patients underwent PHRSD. The mean operative time was 390 minutes, and the mean blood loss was 1270 ml. Duodenal ischemia was prevented by preserving the duodenal branches of the gastroduodenal artery and the anterior inferior pancreaticoduodenal artery. Complications occurred in four patients: one with pancreatic leak, one with choledochoduodenal anastomotic stenosis, and two with delayed gastric emptying. However, no deaths occurred. The final pathologic diagnosis was adenoma in seven patients and carcinoma in situ in one patient. Six of eight patients had an adenoma with papillary growth in the main pancreatic duct. Postoperative pancreatic endocrine and exocrine functions were satisfactory. All patients were alive without recurrent disease at a median follow-up of 30 months. Pancreatic head resection with duodenal segmentectomy appears to be a useful procedure as an organ-preserving pancreatic resection for the branch duct type of IPMT, because this procedure allows a safe and complete resection of the pancreatic head without ischemia of the common bile duct and the duodenum.     

四种保留器官的胰腺切除术式的指征、技术和疗效

目的 探索四种保留器官的胰腺切除术式在治疗胰腺良性及低度恶性肿瘤中的疗效.方法 回顾性总结1990年1月至2010年5月施行的72例保留器官胰腺切除术的手术经验及疗效,男性24例,女性48例,年龄15～68岁,平均46岁.其中行保留十二指肠的胰头切除术(DPRHP)9例,行保留脾脏的胰体尾切除术(SPDP)29例,行胰腺中段切除术11例,行胰腺头体部巨大肿瘤摘除术23例.结果 行DPRHP的9例患者中,术后并发胰瘘、胆瘘各1例,均经保守治疗愈合.行SPDP的29例患者中,术后并发胰瘘3例,未发生迟发性脾梗死.行胰腺中段切除的11例患者中,术后合并胰肠吻合口出血1例,经手术治疗治愈.行胰腺头体部巨大肿瘤摘除术的15例非功能性胰岛细胞瘤患者中,术后并发胰瘘5例,3例于术后6、12、16个月出现肝转移;行肿瘤摘除术的8例黏液性囊腺瘤患者中,术后并发胰瘘2例.结论 保留器官的胰腺切除术可明显减轻手术创伤,疗效与传统术式相同,应作为胰腺良性或低度恶性肿瘤的首选术式.     

Single-branch resection of the pancreas

The treatment of intraductal papillary mucinous tumors (IPMT) of the pancreas is still controversial. In this report we describe a single-branch resection of the pancreas (SBRP), which is a new method for the removal of branch-type IPMT of the head of the pancreas. A multilobular cystic lesion (50 × 40mm) in the head of the pancreas was incidentally detected in an asymptomatic 40-year-old man who underwent a routine ultrasound examination. The tumor was carefully removed along the border of the cyst and the normal parenchyma, with complete preservation of the main pancreatic duct and the common bile duct. A pancreatic fistula developed during the postoperative period, but was well-controlled by endoscopic naso-pancreatic drainage. SBRP is a technically feasible procedure and this operation represents a minimally invasive alternative to any other segmental resection of the pancreas.     

Intraductal papillary-mucinous tumor of the pancreas head with complete absence of the ventral pancreatic duct of Wirsung

A case of intraductal papillary mucinous tumor of the pancreas with complete absence of the ventral pancreatic duct of Wirsung is presented. A 74-year-old Japanese man was admitted to our hospital because of elevated serum amylase concentration. Abdominal computed tomography (CT) scanning revealed diffuse dilatation of the main pancreatic duct and a diffuse and uncircumscribed area with heterogeneous density in the pancreas head. Endoscopic retrograde cholangiopancreatography revealed that the main pancreatic duct was connected with an accessory papilla and was diffusely dilated, without any irregularity of the duct wall being observed in the entire length of the duct. The common bile duct was detected only by cannulation through Vaters papilla, and no pancreatic duct or its communicating branch was found. Some branches, directed to the dorsal portion of the pancreas head, were found arising from the accessory pancreatic duct. Intraductal ultrasound examination performed through the accessory papilla and the common bile duct revealed a small tumor with a heterogeneous echo level in the pancreas head. From these findings, intraductal papillary-mucinous tumor (IPMT) occurring in the pancreas head was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. The resected specimen revealed IPMT in the pancreas head. A roentgenographic study of the resected specimen revealed a defect caused by the tumor located in the pancreatic duct connected with the accessory papilla and showed that there was complete absence of the pancreatic duct connected with Vaters papilla. Surgical resection enabled us to completely analyze the duct system of pancreas divisum. Although it is not known whether there is a relationship between the pathogenesis of IPMT and embryological anomaly of the pancreatic duct system, this case may provide an insight into the pathogenesis of IPMT.     

Prospects for standardization of surgical procedures for carcinoma of the pancreas

Since physicians need to guarantee the efficacy of medical therapy for patients, therapies for patients with cancer should be standardized to some extent. Carcinoma of the pancreas has the highest death rate of all cancers, with a resection rate as low as about 25% to 30% and a 5-year survival rate of around 9%. It is very difficult in such a situation to standardize the surgical strategy for carcinoma of the pancreas. Because pancreatic cancer is a general disease, the treatment strategy should include not only complete surgical resection but also local control methods with intraoperative radiation, prevention of liver metastasis, development of effective anti-cancer drugs, etc. Major progress in therapy for pancreatic carcinoma may be expected in the near future by with the cumulative use of effective therapies. Standard resection and extended resection: For carcinoma of the head of the pancreas, pancreaticoduodenectomy with regional lymph node dissection is performed in Japan, as is extended resection with thorough lymph node dissection of the retroperitoneal and paraaortic region. However, so far the prognosis of patients who undergo extended resection is not better than those who undergo standard resection. A randomized controlled trial of the two types of resection is now being conducted and its results are awaited. For carcinoma of the body and tail of the pancreas, distal pancreatectomy and splenectomy with lymph node dissection is performed if hematogenous or massive lymph node metastasis or direct invasion of the large vessels has not occurred. The Appleby procedure is performed in some cases. Reconstruction and complications of surgical procedures of carcinoma of the pancreas: It appears that a decrease in complications and a lower death rate have been achieved due to pancreaticoduodenectomy rather than due to the extent of lymph node dissection. In particular, progress in anastomosis techniques of the pancreas and intestine and in perioperative control has been marked. For prevention of complications, it is important that absorbable synthetic sutures be used in the pancreaticojejunal anastomosis, that the cut end of the pancreas be sutured and covered by the jejunum without dead space, and that the stent tube be inserted into the main pancreatic duct. The pancreaticojejunal anastomosis should be bordered by the greater omentum. This technique will prevent both the spread of the pancreatic juice into the intraabdominal cavity and rupture of the blood vessels, which can cause fatal postoperative bleeding. Sufficient intraabdominal drains should be in place, especially around the pancreaticojejunal anastomosis. Radiochemotherapy: There are no effective anticancer drugs for the treatment of carcinoma of the pancreas. It was reported that low-dose 5-fluorouracil and cisplatin (5-FU and CDDP) and gemcitabine plus either 5-FU, epirubicin, or CDDP has some effect. The efficacy of intraoperative radiotherapy has not been confirmed. It is not apparent whether radiochemotherapy is superior to surgery. Curable pancreatic carcinoma: Intraductal papillary-mucinous tumors of the pancreas (IPMT) take their name from the histological feature of mucin production and correspond to so-called mucin-producing tumors of the pancreas. This tumor is classified into two types, the main pancreatic duct type and the branch type. About 90% of the main pancreatic duct type and 20% of the branch type are malignant. The branch type of IPMT resembles a bunch of grasps in imaging procedures. Approximately 60% of cases with the branch type of IPMT can be followed up without surgery. Since the prognosis of IPMT is fairly good and the 5-year survival after surgery is about 70% to 80%, limited resection of the pancreas with organ preservation is under investigation. Mucinous cystic tumors of the pancreasin are characterized by development in the body and tail of the pancreas in middle-aged women, with histological ovarian-type stroma in the wall of the tumor, and round cystic lesions with a fibrous capsule containing multiple cystic components of various sizes, which resembles a Chinese citron upon imaging procedures. Surgery should be performed if such a diagnosis is made.     

Clinicopathological features and treatment of intraductal papillary mucinous tumour of the pancreas

BACKGROUND: The surgical strategy in patients with a pancreatic intraductal papillary mucinous tumour (IPMT) is still controversial. In this study the pathological findings in a series of patients were used to rationalize surgical choice. METHODS: Fifty-one patients with IPMT were observed between 1988 and 1998 and treated by pancreatic resection. Factors evaluated included symptoms, tumour site, type of operation, histological findings and resection margins, tumour stage, follow-up and survival. RESULTS: Pancreaticoduodenectomy was the most frequent surgical treatment (33 patients; 65 per cent), followed by left pancreatectomy (ten), total pancreatectomy (five) and middle pancreatectomy (three). Histological assessment revealed the tumour to be an adenoma in 13 patients (25 per cent), a borderline tumour in ten (20 per cent) and a carcinoma in 28 (55 per cent), 19 of which were invasive. Mild to moderate dysplasia was present at the resection margin in 20 specimens (41 per cent), and carcinoma in one. Local recurrence was observed in four patients (8 per cent), all of whom underwent a second resection. The 3-year actuarial survival rate for benign and malignant disease was 94 and 69 per cent respectively (P = 0.03). CONCLUSION: These results suggest that resection should be the treatment for IPMT. Management of the resection margin could be crucial in avoiding tumour recurrence.     

Total pancreatectomy for ductal carcinoma of the head of the pancreas: current status.

Because of dismal results after pancreaticoduodenectomy for adenocarcinoma of the head of the pancreas, a review was made of patients treated by total pancreatectomy for this condition. Although the early experience with total pancreatic resection was disappointing, the results during the past decade have been encouraging. Among the forty-two patients reported on with sufficient detail for analysis the operative mortality has been 17 per cent, and the morbidity has been 21 per cent. Sixty-five per cent of these patients (20 of 30) have survived at least one year and 53 per cent (16 of 30) have lived at least two years after surgery. The management of the diabetes and pancreatic insufficiency has not been difficult. Total pancreatectomy appears to be preferable to pancreaticoduodenectomy in the treatment of ductal carcinoma of the head of the pancreas.     

Magnetic resonance cholangiopancreatography for postoperative follow-up of intraductal papillary-mucinous tumors of the pancreas

BACKGROUND: After resection of an intraductal papillary-mucinous tumor (IPMT), benign tumors or portions of the resected tumor are sometimes left in place to avoid total pancreatectomy. We evaluated the role of magnetic resonance cholangiopancreatography (MRCP) in postoperative follow-up. METHODS: Twenty-two patients underwent MRCP 0.5 to 6.5 years after pancreatic resection for IPMT. RESULTS: Two patients with surgical margin involvement of the main pancreatic duct showed mildly enhanced ductal dilatation due to anastomotic stenosis. In 4 patients with residual IPMT of the branch ducts, postoperative MRCP demonstrated no changes. MRCP revealed new IPMT 1 year after surgery in 1 patient. No patients showed intraductal or intracystic mural nodules postoperatively. In 3 patients with postoperative pancreatitis or recurrent abdominal discomfort, MRCP demonstrated ductal dilatation and poor secretin-stimulated pancreatic secretion into the gastrointestinal tract, which suggested pancreatoenterostomic stenosis. CONCLUSIONS: MRCP is useful for postoperative follow-up of IPMT, in terms of investigating residual or recurrent IPMT and evaluating postpancreatectomy long-term complications.     

A novel technique for multiple pancreatectomies: removal of unicinate process of the pancreas combined with medial pancreatectomy

Preservation of normal pancreatic tissue in the surgical treatment of benign tumors of the pancreas offers advantages over more extended pancreatic resections. Removal of the uncinate process of the pancreas with the preservation of Wirsung's duct allows resection of a localized tumor within the uncinate process of the pancreas, maintains the flow of pancreatic juice into the duodenum, and preserves the dorsal part of the head of the gland. A pancreatic duct stent is particularly useful to identify the pancreatic duct (Wirsung's duct) intraoperatively to avoid injury which causes postoperative pancreatic leak. We have developed and employed a novel technique whereby tumors are completely excised, in combination with medial pancreatectomy, for the management of multiple mucin-producing tumors of the pancreas localized in the uncinate process and in the body of the pancreas. The cut end of the head of the pancreas is closed by interrupted sutures. Reconstruction for the distal pancreas is effected with a Roux-en-Y pancreatico-jejunostomy to the tail of the pancreas. Recovery was uncomplicated in our patient, with no endocrine or exocrine pancreatic insufficiency after 2-year follow-up. Received for publication on July 1, 1999; accepted on Aug. 17, 1999     

Laparoscopic-assisted spleen-preserving and pylorus-preserving total pancreatectomy for main duct type intraductal papillary mucinous tumors of the pancreas: a case report

Minimally invasive and function-preserving pancreatectomy would be the ideal approach for benign and borderline malignant tumors of the pancreas. Total pancreatectomy can be indicated for the main duct type of intraductal papillary mucin-producing tumor (IPMT) to achieve radical resection. Recently, several studies advocating total pancreatectomy in IPMT have been published, but they are all believed to be done by conventional laparotomy. Herein, we report a case of a 72-year-old female patient who successfully underwent laparoscopic-assisted total pancreatectomy with the spleen and pylorus preserved in borderline malignant main duct type IPMT. A marginal ulcer around the duodenojejunostomy was developed, but managed by a proton-pump inhibitor. She was discharged 20 days after surgery. She was followed for more than 2 years without evidence of tumor recurrence. Her blood sugar level was well controlled by insulin pump therapy and image study showed well-preserved spleen function.     

Intraductal papillary-mucinous tumor of the pancreas: difficulties and mistakes of diagnostics and treatment

15 patients with intraductal papillary-mucinous tumors (IPMT) of the pancreas were observed. Clinical manifestation corresponded with chronic pancreatitis. Ultrasound study, including endosonography, contrast-enhanced computer and magnetic resonance tomography were used in complex observation of the patients. Dilation of main or lateral pancreatic ducts, connection of tumor with pancreatic duct system and absence of septal calcinosis are typical signs in radiodiagnostics of IPMT. Visualization of parietal papillary proliferations and their contrast enhancement are undeniable signs f or neoplastic character of pancreatic duct dilation. Sensitivity of CT, MRТ and endoUS amounted accordingly 66, 83 and 88%. All patients were operated. Extent of operation was determined by morphological character, localization and size of the tumor (pylorus-preserving pancreaticoduodenal resection was carried out to 6 patients, distal resection including robot-assisted - to 7 patients, midline resection - to 1 patient, duodenum-preserving resection of head and body of pancreas - to 1 patient). Intraoperative urgent histologic study of pancreatic section was carried out by all means. Tumors with borderline degree of malignancy were detected in 4 cases.     

A surgical and pathological based classification of resective treatment of pancreatic cancer. Summary of an international workshop on surgical procedures in pancreatic cancer.

BACKGROUND: The extent of pancreatic resection and lymphadenectomy, both for Kausch-Whipple pancreatoduodenectomy and for left pancreatectomy, is variable between surgeons, according to their training. METHODS: On May 30, 1998, a consensus conference on the surgical treatment of pancreatic cancer took place in Castelfranco Veneto, Italy. A group of 29 European surgeons and pathologists, recognized as international experts, analyzed the surgical and pathological procedures used in European countries to resect pancreatic cancer and examine the specimen. RESULTS: A general agreement was reached on the definitions of standard , 'radical and 'extended radical Kausch-Whipple pancreatoduodenectomy for carcinoma of the head of the pancreas, and standard and 'radical left pancreatectomy for carcinoma of the body and tail of the pancreas. Segmental venous resection, as well as adjacent organ resection, can be performed at the time of standard, radical or extended radical pancreatoduodenectomy or left pancreatectomy if required. The pylorus-preserving procedure is contraindicated only for carcinomas of the anteriorsuperior part of the head of the pancreas. Guidelines for a standardized pathological examination of the resected specimen were produced. CONCLUSION: Adoption of the recommended terminology will improve outcome comparisons between institutions performing the different procedures. Moreover, standardization of operations, terminology and pathological reporting is essential for prospective randomized trials comparing different operations either alone or within the context of adjuvant therapy studies.     

Surgical management of pancreatic neuroendocrine tumors

This study outlines the surgical management and clinicopathological findings of pancreatic neuroendocrine tumors (P-NETs). There are various surgical options, such as enucleation of the tumor, spleen-preserving distal pancreatectomy, distal pancreatectomy with splenectomy, pancreatoduodenectomy, and duodenum-preserving pancreas head resection. Lymph node dissection is performed for malignant cases. New guidelines and classifications have been proposed and are now being used in clinical practice. However, there are still no clear indications for organ-preserving pancreatic resection or lymph node dissection. Hepatectomy is the first choice for liver metastases of well-differentiated neuroendocrine carcinoma without extrahepatic metastases. On the other hand, cisplatin-based combination therapy is performed as first-line chemotherapy for metastatic poorly differentiated neuroendocrine carcinoma. Other treatment options are radiofrequency ablation, transarterial chemoembolization/embolization, and liver transplantation. Systematic chemotherapy and biotherapy, such as that with somatostatin analogue and interferon-α, are used for recurrence after surgery. The precise surgical techniques for enucleation of the tumor and spleen-preserving distal pancreatectomy are described.     

Histopathology in the evaluation of total pancreatectomy for ductal carcinoma.

Whipple resections for pancreatic head carcinoma are often inadequate because tumor is left behind in the body and tail. Thirty-six patients have undergone total pancreatectomy for various conditions, of which 25 have undergone total pancreatectomy, for ductal carcinoma. Thirty-seven per cent of these 25 patients have shown histologic evidence that a Whipple resection would not have adequately removed tumor-bearing pancreatic tissue. Three patients had carcinoma spreading up and along the common bile duct from a primary ductal carcinoma in the head of the pancreas. Four patients had tumor infiltrating in continuity into the pancreatic body and tail at a distance from the palpable tumore in the head well to the left of a Whipple transection site. Five patients had widespread multifocal autonomous tumor involving other areas in the gland but with tumor palpable only in the head of the pancreas. Three patients (12%) died postoperatively. The two year survival rate is 32%, and the five year survival, 19%. Histological factors affecting the survival prognosis include 1) positive nodes, 2) tumor extension up the common duct, and 3) intrapancreatic extension and multicentricity of tumor mandating total pancreatectomy for hope of cure in at least 38% of cases.     

Lesions of the spleen in chronic pancreatitis by thrombophlebetic splenomegaly and cyst penetration

Chronic pancreatitis leads to changes of nearby organs with possible acute and chronic complications including lesions of the spleen. Among 341 patients operated upon between January 1981 and June 2002 in the surgical department Gera, we found 7 spontaneous spleen ruptures or such after minimal trauma and 4 pseudocysts, which expanded to the splenic hilus. In all cases splenectomy was carried out with resection of the tail of the pancreas with or without drainage of the pancreas. 2 patients with a history of splenectomy after minimal trauma underwent duodenum-preserving resection of the pancreatic head to Frey, and distal pancreatectomy, resp. CONCLUSIONS: Lesions of the spleen belong to the rare complications of chronic pancreatitis. With known case history and mostly delayed course, the operative concept must be concentrated not only on the splenic lesion but also on the therapy of the chronic pancreatitis.     

Familial pancreatic cancer: report of one Japanese family

Most familial pancreatic carcinomas have been reported from European countries and the United States, and there has been only one report from Japan. A 50-year-old Japanese woman presented with a pancreatic head mass and underwent pylorus-preserving pancreatoduodenectomy with portal vein resection. The histological diagnosis was well-differentiated adenocarcinoma of the head of the pancreas. Her mother died of pancreatic head carcinoma, which had been shown on computed tomography at the age of 70 years. One of her uncles on her fathers side had had pancreatic tail carcinoma, and at the age of 59, had undergone distal pancreatectomy, splenectomy, wedge resection of the liver, and partial resection of the colon. The histological diagnosis was moderately differentiated tubular adenocarcinoma of the pancreas. He had had a subtotal gastrectomy for early gastric cancer (tubular adenocarcinoma limited to the mucosa) at the age of 53. He died of recurrence of the pancreatic tail carcinoma 3 months after the distal pancreatectomy had been performed. This communication reports a second Japanese family with familial pancreatic cancer, as shown by pancreatic carcinomas in two first-degree relatives and in one third-degree relative.     

Middle-preserving pancreatectomy with reversed pancreaticogastrostomy: report of a case

Parenchyma-sparing pancreatic resections have been reported increasingly in recent years; however, for multifocal diseases involving the head and the tail of the pancreas, total pancreatectomy is still the preferred procedure. The possible consequence of this procedure is loss of normal pancreatic parenchyma, resulting in insufficiency of pancreatic exocrine and endocrine functions. Various types of limited resection have been introduced for isolated or multiple pancreatic lesions, depending on the location of the tumor. Even for multifocal diseases, if the pancreatic body is spared, a middle-preserving pancreatectomy (MPP) can be performed to assure maximal pancreatic function and uncompromised quality of life. Yet, few papers have introduced the feasibility of MPP for a better outcome. This report describes a new surgical technique for MPP using an alternative approach for the remnant pancreas anastomosis. We used this technique successfully to remove a bifocal neoplasm: adenocarcinoma of the distal bile duct and mucinous cyst adenoma in the tail of the pancreas.