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.     

A case of pancreatic carcinoma coexist with pancreatic pseudocyst

Report of Pancreatic carcinoma coexist with Pancreatic pseudocyst is rare. We have experienced a case of pancreatic carcinoma which was diagnosed and resected by coexisted pancreatic pseudocyst. The patient aged 53 years, complained of epigastric distension and visited our hospital. There was no past history of abdominal injuries and he didn't drink much. A cyst 7cm in diameter was found at pancreatic tail lesion by our examination. By blood chemistry tumor marker CA19.9 was 310 and he complicated diabetes mellitus. We suspected pancreatic carcinoma coexist with pancreatic pseudocyst, so distal pancreatectomy and splenectomy was performed. We found a solid tumor sized about 3cm in diameter at just proximal of pancreatic cyst. Since perioperative histological examination revealed adenocarcinoma, we performed lymph node dissection. The tumor was highly differentiated adenocarcinoma which invaded retroperitoneum in some lesion. The patient died 10 months later by recurrence. We concluded that we must take into account not only laboratory finding but their clinical course in such cases.     

Complete remission of pancreatic cancer after multiple resections of locally pancreatic recurrent sites and liver metastasis: Report of a case

Pancreatic cancer has the most dismal prognosis of all gastrointestinal cancers. We herein report a case of complete remission from pancreatic cancer by multire-sections of locally pancreatic recurrent sites and liver metastasis over a 14-year period. A 60-year-old man was admitted to our hospital because of a neoplasm of the tail of the pancreas in April 1992. A distal pancreatectomy was curatively performed on this patient. At 1 year after surgery a solitary liver metastasis appeared, and we thus performed a partial hepatectomy. Thereafter, local recurrences appeared twice and we performed a pancreatectomy each time. Finally, we performed a total pancreatectomy. The histopathological findings of specimens of the pancreas showed papillary adenocarcinoma, although the original pancreatic tumor also demonstrated areas of tubular adenocarcinoma. Metastatic liver tumor showed tubular adenocarcinoma. The patient has survived for 14 years since the first operation. This is a rare case of a long survival of a patient with pancreatic cancer due to its histopathology and biologic characteristics.     

A repeated pancreatectomy in the remnant pancreas 22 months after pylorus-preserving pancreatoduodenectomy for pancreatic adenocarcinoma

We report a case of a repeated curative pancreatic resection in the remnant distal pancreas 22 months after pylorus-preserving pancreatoduodenectomy (PpPD). The patient was a 52-year-old woman with a past history of PpPD for adenocarcinoma of the head of the pancreas 22 months prior to the present operation. The original tumor was histopathologically diagnosed as a papillary adenocarcinoma with clear surgical margin at the surgical cut end of the pancreas (R0, International Union Against Cancer [UICC] classification). Twenty months after the PpPD, a follow-up computed tomography (CT) scan showed multiple low-density lesions in the body and tail of the pancreas without any other distant metastasis. A second operation, curative resection of the remnant pancreas, with splenectomy and distal gastrectomy, was performed. The second tumor was a papillary adenocarcinoma, the same diagnosis as that of the first tumor, and it also showed similar histopathological findings, including immunohistochemical staining of Ki-67 and p53 protein, and the same pattern of K-ras point mutation. The patient is considered to have shown a rare, unique pancreatic cancer with metachronous carcinogenesis in the remnant pancreas.     

Total pancreatectomy combined with partial pancreas autotransplantation for recurrent pancreatic cancer: a case report

We describe a patient presenting with a resectable carcinoma of the remnant pancreas at 3 years after undergoing a pylorus-preserving pancreaticoduodenectomy for invasive ductal carcinoma of the pancreatic head. We also performed a distal pancreas autotransplantation using a part of the resected pancreas to preserve endocrine function. Final histologic findings showed the second tumor to be an invasive ductal carcinoma consisting of a well-differentiated tubular adenocarcinoma with similar histopathologic findings as the first tumor. There were no microscopic lymph node metastases and no evidence of microvascular invasion (pStage IA [pT1, pN0, M0] and R0 according to the International Union Against Cancer TNM classification). The patient was discharged at 20 days after surgery without any trouble and followed by adjuvant chemotherapy with S-1. The carbohydrate antigen 19-9 value was again normalized after the second surgery. Twenty months after the second operation, the patient is alive without cancer recurrence. The pancreas graft is functioning with a blood glucose of 108 mg/dL, HbA1C of 6.2%, and serum C-peptide of 1.4 ng/mL.     

Pancreatic cancer in the remnant pancreas following primary pancreatic resection

Purpose

To clarify the clinical features of cancer in the pancreatic remnant.

Methods

We retrospectively reviewed the clinical and pathological findings of 10 patients who developed remnant pancreatic cancer in our hospital between 2002 and 2012. The KRAS sequences in both the initial pancreatic tumor and remnant pancreatic cancer were examined in two patients.

Results

Eight patients underwent a second pancreatectomy for remnant pancreatic cancer (resected group), while two patients were not operated on and underwent chemotherapy (unresected group). The remnant pancreatic cancer developed at the cut end of the pancreas (pancreaticogastrostomy site) in four patients. In the resected group, four patients died 17 months after the emergence of the remnant pancreatic cancer and four patients survived during the median 40.5-month observation period. The median survival of the unresected group after the emergence of the remnant pancreatic cancer was 10 months. The findings of the KRAS sequencing and immunohistological staining of the remnant pancreatic cancer for MUC1 and MUC2 in the two patients were consistent with those of the initial pancreatic tumor in one patient, and not consistent in the other.

Conclusions

Our results suggest that both local recurrence and a new primary cancer can develop in the pancreatic remnant, and repeated pancreatectomy can prolong survival.     

Clinico-pathological study on the appropriate range of pancreatic resection to obtain operative curability of pancreatic head cancer

We studied the mode of spread on the pancreatic head carcinoma histologically in association with the patient survival period and the mode of cancer recurrence. The histological materials were 41 surgically resected specimens and 12 autopsied specimens. The mode of cancer spread were classified into 5 factors: continuous spreading along the caudal pancreatic duct wall; dysplastic lesion; multicentric cancer lesion; lymphatic infiltration; lymph node metastasis and direct invasion toward the retroperitoneal space. As a result, total pancreatectomy is considered as rationale only when continuous ductal infiltration spreads into the duct in pancreatic tail, but the cut margin of the pancreas should be 2 cm distant, at least, by the macroscopical observation. Additionally, frozen section of the cut line, cytological examinations of the pancreatic juice and duct epithelium in remaining pancreas should be negative. It was remarkable that the patient's prognosis depended upon the lymph-node involvement and retroperitoneal invasion at the surgical resection. Therefore, it is most important to do prophylactic resection of a soft tissue behind the pancreas including lymph nodes in the retroperitoneal space.     

Pancreaticoduodenectomy with resection of the splenic artery and splenectomy for pancreatic double cancers after total gastrectomy. Preservation of the pancreatic function via the blood supply from the posterior epiploic artery: report of a case

The patient was a 56-year-old man who had previously undergone a total gastrectomy without splenectomy, and was diagnosed with pancreatic head and body cancers and primary solitary lung cancer. The pancreas body tumor invaded the origin of the splenic artery, and if the origin of the splenic artery were resected there would be no blood flow to the pancreas tail, resulting in a need for total pancreatectomy. However, we focused on the posterior epiploic artery (PEA), which is a less well known blood supply from the mesocolon to pancreatic body and tail, and planned to preserve the pancreatic tail as long as the resected margin of the pancreas was not malignant, considering his limited life expectancy. We performed a pancreaticoduodenectomy with resection of the origin of the splenic artery and splenectomy, preserving the pancreatic tail and PEA. The patient has been free from insulin therapy for blood sugar control, and has been well for 10 months after the surgery.     

Resection of a cancer developing in the remnant pancreas after a pancreaticoduodenectomy for pancreas head cancer

We report a rare case of a curative resection performed on a carcinoma developing in the remnant pancreas at 3 years 7 months after a pancreaticoduodenectomy for pancreatic cancer. A 63-year-old man underwent a pancreaticoduodenectomy for pancreatic cancer on November 1999. Because the celiac trunk was occluded by atherosclerosis, an aortohepatic bypass with a saphenous vein graft was performed simultaneously. In May 2003, tumor marker levels increased, and a tumor was detected in the remnant pancreas on computed tomography. There were no findings such as invasion into the surrounding tissue or distant metastasis, and therefore we removed the remnant pancreas in July 2003. Histopathologically, the tumor consisted of a well-differentiated tubular adenocarcinoma and was limited to the pancreas. Moreover, the anastomotic site of the pancreaticojejunostomy was negative for cancer, and some foci of papillary hyperplasia and goblet cell metaplasia of the pancreatic ductal epithelium, which was thought to be the precursor of the pancreatic cancer, were seen. These findings suggested that the tumor was a second primary cancer developing in the remnant pancreas. This case provided suggestive evidence for the development of pancreatic cancer, and the surgical procedure for a pancreaticoduodenectomy with occlusion of the celiac trunk is discussed.     

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.     

Mucinous carcinoma of Vater's ampulla with a unique extension along the main pancreatic duct

We report a case of mucinous carcinoma of Vater's ampulla with a unique extension along only the main pancreatic duct (MPD) and microinvasion to the pancreas. A 52-year-old man was referred to our hospital for the evaluation and treatment of acute pancreatitis. Abdominal computed tomography (CT) demonstrated swelling in the head of the pancreas with a mass in the duodenum. Hypotonic duodenography and endoscopic examination revealed a well-defined mass, measuring about 25 mm in size, in Vater's ampulla. A biopsy specimen of the tumor showed moderately differentiated adenocarcinoma. A pylorus-preserving pancreaticoduodenectomy with a regional lymphadenectomy was performed, under a preoperative diagnosis of adenocarcinoma of Vater's ampulla with direct invasion into the head of the pancreas. The resected specimen of the duodenum confirmed the presence of the mass, which measured 22 × 15 mm in size, in Vater's ampulla. Microscopically, the tumor consisted of two components: moderately differentiated adenocarcinoma in the peripheral region of the tumor Vater's papilla and mucinous carcinoma in the central region of the tumor. The mucinous carcinoma component uniquely extended along only the MPD with microinvasion to the pancreas. Immunohistochemically, both the moderately differentiated adenocarcinoma and the mucinous carcinoma were positive for cytokeratin 20 (CK20) and negative for cytokeratin 7 (CK7) which is the pattern of intestinal-type carcinoma of Vater's ampulla. We concluded that the original site of this tumor may have been the duodenal epithelium of Vater's ampulla originally moderately differentiated adenocarcinoma—which subsequently changed to mucinous carcinoma that extended along only the MPD with microinvasion to the pancreas.     

Cystic pancreatic neuroendocrine neoplasms with uncertain malignant potential: Report of two cases

Neuroendocrine tumors of the pancreas (NETP) represent only 1%–2% of all pancreatic neoplasms. They can be classified as functioning or non-functioning, respectively, according to the presence or absence of paraneoplastic syndrome. Case 1 concerned a 70-year-old woman with a cystic lesion of the pancreatic head and body. All tumor markers were negative. The patient underwent a distal pancreatectomy. The histology revealed a well-differentiated endocrine tumor with uncertain malignant potential. Case 2 was a 61-year-old man with chronic polyserositis. The serum tumor markers were negative, while he was strongly positive for intracystic tumor markers carcinoembryonic antigen, carbohydrate antigen (CA) 19–9, and CA 125. The patient underwent a cephalo-pancreatic duodenectomy. The preoperative differential diagnosis of cystic NETP is still a challenge due to the high rate of the nonfunctional variant. Although cystic NETPs are well differentiated, they are still tumors with a malignant potential, and therefore an early diagnosis and radical surgical resection could be associated with a better long-term survival.     

Pancreatic mucin-producing adenocarcinoma associated with a pancreatic stone: Report of a case

We present an autopsy case of an 83-year-old Japanese man with a mucin-producing adenocarcinoma accompanied by pancreatolithiasis in the head of the pancreas. He suffered from obstructive jaundice and died of disseminated intravascular coagulation. He did not normally drink alcohol and had no history of chronic pancreatitis. The autopsy findings revealed a mucinous cystic tumor, composed of multiple dilatated branches, in the head of the pancreas. Histological examinations showed papillary adenocarcinoma, which scirrhously infiltrated the distal common bile duct with perineural invasion and lymph node involvement. He was thus diagnosed to have mucin-producing branch-type cancer in the head of the pancreas. The main pancreatic duct was dilated, and the residual pancreatic tissue showed moderate fibrosis and parenchymal atrophy. A stone was observed in a dilated branch of the primary lesion. To the best of our knowledge, there have only been five previously reported cases of mucin-producing tumor associated with pancreatolithiasis. Intraductal calcification is a major characteristic of chronic pancreatitis, but it is clinically important not to misdiagnose cancers associated with pancreatolithiasis such as chronic pancreatitis.     

Recurrence of mucosal carcinoma of the bile duct, with superficial flat spread, 12 years after operation

We report herein a case of recurrent mucosal cancer of the extrahepatic bile duct, with superficial flat spread, 12 years after operation. A 67-year-old woman had undergone common bile duct (CBD) resection and Roux-en-Y reconstruction. Histologically, the tumor was papillary adenocarcinoma, with superficial flat spread, with no invasive component. The epithelium at the distal margin had been exfoliated, so the absence or presence of any remnant cancerous lesion was unclear. But the superficial flat spread had expanded to within at least 3 mm from the distal margin. About 12 years postoperatively, she was hospitalized with upper abdominal pain, and duodenoscopy demonstrated a tumor in the second portion of the duodenum. Biopsy identified adenocarcinoma. Computed tomography showed a low-density mass between the duodenum and pancreatic head. Pancreatoduodenectomy was performed. Histologically, papillary adenocarcinoma was found within the whole of the intrapancreatic bile duct, and its histological appearance resembled that of the original tumor. Moderately differentiated tubular adenocarcinoma had invaded around the tissue of the intrapancreatic CBD. These findings suggest that remnant intramucosal flat carcinoma within the intrapancreatic bile duct had developed into invasive carcinoma over the course of 12 years. This case suggests that remnant intraepithelial flat carcinoma within the CBD may develop a late local recurrence.     

Multicentric pancreatic intraepithelial neoplasias (PanINs) presenting with the clinical features of chronic pancreatitis

A 46-year-old woman was readmitted to our hospital in August 2005 because of severe abdominal pain and nausea. Computed tomography demonstrated a huge cystic lesion in the retroperitoneal space behind the hepatoduodenal ligament and lesser peritoneal cavity. Endoscopic retrograde pancreatography revealed communication between the dilated main pancreatic duct and a pseudocyst. The condition was preoperatively diagnosed as chronic pancreatitis associated with a pseudocyst or an intraductal papillary mucinous neoplasm without mucin hypersecretion. The patient underwent a distal pancreatectomy with splenectomy. The pathologic diagnosis was multicentric pancreatic intraepithelial neoplasia (PanIN), and histological examination revealed a positive surgical margin around the remnant pancreas. Four months after the surgery, the patient underwent a total pancreatectomy. Macroscopic observation revealed diffuse fibrosis of the pancreatic parenchyma compatible with chronic pancreatitis. Histological examination revealed a constellation of noninvasive intraductal neoplasias with high-grade atypia, diffusely distributed in the small pancreatic ducts of the resected pancreas. Localized fibrosis and cystic dilation of the small ducts were detected in a lobule of exocrine glands draining into a ductule involved by PanIN lesions in the head of the pancreas. In summary, multicentric PanIN lesions are associated with lobular atrophy of the pancreatic parenchyma and chronic pancreatitis-like changes that follow. Total pancreatectomy may be recommended for patients with multicentric precursor lesions throughout the entire pancreas.     

Multifocal intraductal papillary adenocarcinoma of the pancreas: Report of a case

This paper descibes a case of a mucin-producing tumor of the pancreas, histologically diagnosed as multifocal intraductal papillary adenocarcinoma. The patient had a markedly dilated main pancreatic duct, detected by ultrasound (US) and computed tomography (CT), and endoscopy showed a papillary tumor projecting from the patulous orifice of the enlarged papilla of Vater. Several biopsies taken from the tumor indicated a diagnosis of papillary adenocarcinoma. Total pancreatectomy was thus performed, revealing a multifocal papillary tumor growing along the main duct in the head, body, and tail of the pancreas. Microscopically, these multiple tumors were found to be mucin-producing papillary adenocarcinoma of the main and subsidiary pancreatic ducts with nodular and microfocal periductal invasion. The surgical treatment of multifocal tumors, may therefore necessitate total pancreatectomy to achieve curative resection, in some cases.     

38例胰腺囊性肿瘤的临床诊断及外科治疗

目的探讨胰腺囊腺瘤、囊腺癌的诊断和治疗方法.方法对1980年1月～2005年4月中国医科大学附属第一医院收治的38例胰腺囊腺瘤和囊腺癌患者的临床表现、检查、手术方式及预后进行了分析.结果胰腺囊腺瘤、囊腺癌主要发生在胰体尾部（79%）,女性多见（84%）,肿瘤无特异性临床表现.超声和CT是主要的影像学检查方法,本组病例超声的诊断率为66%,CT为78%,血清肿瘤标志物检查对诊断意义不大.38例胰腺囊腺瘤、囊腺癌中行胰十二指肠切除2例,保留十二指肠的胰头切除1例,胰体部分切除1例,胰体尾切除21例,肿瘤摘除8例.手术切除的28例胰腺囊腺瘤中25例获得随访,2例因其他疾病死亡,23例健在.8例胰腺囊腺癌手术切除5例,4例获得随访,3例存活.手术后胰瘘的发生率为28%.结论胰腺囊腺瘤手术切除后可以达到治愈,囊腺癌切除后也有较高的生存率.术中病理检查是术式选择的关键,对于诊断明确的良性肿瘤可行胰腺局部切除术,或行肿瘤摘除术.     

Intramural pseudocyst of the duodenum caused by pancreatic head cancer: report of a case

An intramural pseudocyst in the alimentary tract develops as a rare complication of acute pancreatitis or trauma. A 60-year-old woman with pancreatic head cancer underwent preoperative radiological examinations, which revealed a 45-mm cystic mass around the second portion of the duodenum. Endoscopic ultrasonography confirmed a cystic lesion in the submucosal layer of the duodenum and fine needle aspiration cytology of the cystic contents suggested adenocarcinoma. The cystic fluid was amylase-rich, at 17040 U/l. We performed pancreaticoduodenectomy for the pancreatic head cancer. Pancreatography of the resected specimen showed a communication between the main pancreatic duct and the cystic lesion. The cut surface of the resected specimen revealed a cystic lesion, which surrounded the duodenum. Pathologically, the cystic lesion was diagnosed as a pseudocyst, located between the dissociated smooth muscle layers of the duodenum.     

保留脾脏的胰体尾肿瘤切除术的应用探讨

目的探讨保留脾脏的胰体尾肿瘤切除术在胰体尾部肿瘤治疗中的可行性及作用。方法收集大连市中心医院于1999年1月至2010年12月期间收治的胰体尾肿瘤患者49例,其中行保留脾脏的胰体尾肿瘤切除术18例,包括胰腺腺癌7例,胰尾囊腺瘤9例,胰岛素瘤2例,其中3例患者接受了腹腔镜辅助保留脾脏胰体尾肿瘤切除术。回顾性分析患者的临床症状、术前检查、术后并发症等指标,并进行术后随访观察。结果开腹和腹腔镜辅助保留脾脏胰体尾肿瘤切除术均顺利实施．所选术式患者耐受性好,术后并发症少,多数患者获得长期生存。结论术前影像学检查和肿瘤标志物检查是早期诊断胰体尾肿瘤的可靠办法,CTA、DSA等检查是术前判定的重要手段,保留脾脏的胰体尾肿瘤切除术应作为胰体尾肿瘤的首选术式。腹腔镜辅助保留脾脏胰体尾肿瘤切除术是安全可行的,具有创伤轻、恢复快、并发症少等优点。     

Invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma without mucin hypersecretion but with changes in the pancreatic duct on pancreatography

We present a case of invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma without mucin hypersecretion in a 65-year-old man with a 45-year history of alcohol abuse and a 2-year follow-up of chronic pancreatitis. Two years previously, in May 1998, he was admitted for investigation of abdominal pain. Computed tomography (CT) showed diffuse dilation of the main pancreatic duct with atrophy of the pancreatic parenchyma. Endoscopic retrograde pancreatography (ERP) showed a diffusely dilated main pancreatic duct with irregular side branches in the head of the pancreas. Chronic alcoholic pancreatitis was diagnosed on the basis of the pancreatography findings. The patient was readmitted for investigation of progressive weight loss in August 2000. Serum CA19-9 levels were markedly elevated (750 U/ml) and CT showed enlargement of the head and body of the pancreas. ERP showed irregularity of the main pancreatic duct in the head of the pancreas, and the distal main pancreatic duct (which was dilated on initial ERP examination) was interrupted in the body of the pancreas. Suspected pancreatic carcinoma was diagnosed, and pylorus-preserving pancreatoduodenectomy was performed. Frozen section examination of the cut end of the pancreas revealed ductal carcinoma, and total pancreatoduodenectomy with portal vein resection was performed. Histologically, the resected tumor was diagnosed as an invasive carcinoma derived from intraductal papillary adenocarcinoma without mucin hypersecretion. We recommend observing changes in the pancreatic duct on pancreatography to diagnose invasive carcinoma of the pancreas derived from intraductal papillary adenocarcinoma in a resectable state. Received: February 6, 2002 / Accepted: June 10, 2002 Offprint requests to: S. Ariizumi