Nonfunctioning Islet Cell Carcinoma Research Articles

Spontaneously complete regression of pseudolymphoma of the remnant pancreas after pancreaticoduodenectomy

BackgroundPancreatic pseudolymphoma is extremely rare. MethodWe present multiple pseudolymphomas in the head and body of the pancreas. The hypoechoic lesions observed by endoscopic ultrasound were enhanced in late-phase angio-computed tomography and homogeneously hypointensive in T1-weighted magnetic resonance imaging (MRI). 18F-fluorodeoxyglucose positron emission tomography showed strong accumulation in the lesions. The lesions were suspected to be non-functioning islet cell carcinoma. The intraoperative pathological diagnosis for the specimen obtained by a pylorus-preserving pancreaticoduodenectomy was non-neoplastic lymphoid cells. The remnant lesion in the pancreatic body was preserved. ResultsMacroscopically, the mass was well-circumscribed gray-white colored lesion. The pathological diagnosis was pancreatic pseudolymphoma. The lesion in the remnant pancreas spontaneously disappeared within one year after the operation. ConclusionThe differential diagnosis of pancreatic pseudolymphoma from malignant tumor is very difficult, however, the image findings demonstrated here may be informative. The spontaneous disappearance of pancreatic pseudolymphoma was firstly observed in the present case.

Management of nonfunctioning islet cell tumors.

To more clearly define the clinical and pathological characteristics and appropriate diagnosis and treatment of nonfunctioning (NFICTs) islet cell tumors, and to review our institutional experience over the last 30 years. The records of 43 patients confirmed to have nonfunctioning islet cell tumors of pancreas were retrospectively reviewed. Survival was estimated by the Kaplan-Meier methods and potential risk factors for survival were compared with the log-rank tests. The mean age was 31.63 years (range, 8 to 67 years). There were 7 men and 36 women. Twenty-eight patients had a confirmed diagnosis of nonfunctioning islet cell carcinoma (NFICC) and benign islet cell tumors were found in 15 patients. The most common symptoms in patients with NFICTs were abdominal pain (55.8%), nausea and/or vomiting (32.6%), fatigue (25.6%) and abdominal mass (23.3%). Preoperative ultrasonic and computed tomography localized the tumors in all patients. Forty-three NFICTs were distributed throughout the pancreas, with 21 located to the right of the superior mesenteric vessels, 10 in the body of the pancreas, 6 in the tail of the pancreas, and multiple tumors were found in one patient. Thirty-nine of 43 patients (91%) underwent surgical resection. Surgical treatment was curative in 30 patients (70%) and palliative in 9(21%). The resectability and curative resection rate in patients with NFICC of pancreas were 89% and 61%, respectively. The overall cumulative 5- and 10-year survival rates for patients with NFICC were 58.05% and 29.03%, respectively. Radical operation and diameter of cancer small than 10 cm were positive prognostic factors in females younger than 30 years old. Multivariate Cox regression analysis indicated that radical operation was the only independent prognostic factor, P=0.007. Nonfunctioning islet cell tumors of pancreas are found mainly in young women. The long-term results for patients undergone surgery, especially curative resection are good.

Nonfunctioning islet cell carcinoma of the pancreas: Survival results in a contemporary series of 163 patients

Background. The natural history of nonfunctioning islet cell carcinoma of the pancreas is poorly defined. We therefore reviewed our institutional experience during a period of 12 years to define more clearly the natural history of this disease as a basis for individual therapeutic recommendations. Methods. The records of all patients who had histologically or cytologically confirmed nonfunctioning islet cell carcinoma of the pancreas were retrospectively reviewed. Patients were grouped by extent of disease at diagnosis and by initial treatment. Survival distributions were estimated by Kaplan-Meier analysis. Results. One hundred sixty-three patients with nonfunctioning islet cell carcinoma of the pancreas were identified. The overall median survival duration was 3.2 years. The median survival was 7.1 years in patients with localized disease who underwent a potentially curative resection and 5.2 years in those with locally advanced, unresectable, nonmetastatic disease (P =.04). Patients with metastatic disease that could not be resected had a median survival of 2.1 years. Conclusions. Patients with completely resected localized disease had a long median survival. Patients with nonmetastatic but unresectable locally advanced disease also had a surprisingly long median survival; major treatment-related morbidity may be hard to justify in this subgroup. The short median survival in patients with metastatic disease suggests that the frequent practice of observation in this patient subgroup needs to be reexamined and that continued investigation of regional and systemic therapies with novel agents is warranted. (Surgery 2001;130:1078-85.)

Nonfunctioning islet cell carcinoma of the pancreas: an evaluation of seven patients who underwent resection followed by long-term survival.

Islet cell carcinoma of the pancreas is a rare, indolent malignancy associated with a higher resectability rate and better survival than ductal carcinoma. This retrospective study presents the results of surgical treatment for nonfunctioning islet cell carcinoma of the pancreas in seven patients diagnosed and treated at Kurume University Hospital. There were two men and five women, with an average age of 54 years. Of the five tumors, four were located in the head of the pancreas, and the other three tumors were located in the body or tail of the pancreas. Epigastric pain was frequently the primary clinical symptom. By the time of diagnosis, four of the patients had regional disease, one of whom had lymph node and liver metastases, and one, liver metastases. Another patients was found to have lymph node metastases intraoperatively. Only one of the patients died of recurrent cancer, 21 years after the original operation. All of the patients who had liver metastases at the time of initial surgical treatment are now living at home. Thus, we conclude that nonfunctioning islet cell carcinomas are slow-growing tumors with a good prognosis if the main tumors and metastatic lesions are removed.

Nonfunctioning Islet Cell Carcinoma of the Pancreas: An Evaluation of Seven Patients Who Underwent Resection Followed by Long-Term Survival

Nonfunctioning Islet Cell Carcinoma of the Pancreas: An Evaluation of Seven Patients Who Underwent Resection Followed by Long-Term Survival

A CASE OF NONFUNCTIONING ISLET CELL CARCINOMA OF THE PANCREAS FOUND 3 YEARS AFTER A HEPATECTOMY FOR LIVER METASTASES

We experienced a rare case of nonfunctioning islet cell carcinoma of the pancreas found as the primary lesion 3 years after a hepatectomy for liver metastases. A 65-year-old man was admitted to the hospital for treatment of a liver tumor which was found during clinical observation for gastric ulcer. Abdominal computed tomography revealed a tumor 6cm in diameter in the right hepatic lobe. Selective subsegmentectomy with reconstruction of middle hepatic vein was performed for the tumor. With histopathological and immunohistochemical examinations, it was diagnosed as either primary or metastatic carcinoid tumor of the liver. Extrahepatic lesions were not detected prior to hepatectomy. Subsequently, this patient had a pancreas tumor and recurrence of liver tumor 3 years and 1 month after the hepatectomy, and a distal pancreatectomy with splenectomy and another hepatectomy was peformed. Immunohistochemically, those pancreas and liver tumors were positive for Grimelius and chromogranin A stains and were negative for insulin, glucagon, somatostatin, serotonin, amylase and α1-antitrypsin stains. From these results, this patient was finally diagnosed as having a nonfunctioning islet cell carcinoma of the pancreas with liver metastases. He died from systemic lymph node metastases 4 years and 4 months after the initial hepatectomy. Islet cell tumor of the pancreas histologically resembles closely carcinoid tumor. When liver tumor was diagnosed as carcinoid tumor without extrahepatic disease, attention should be paid to a possible presence of nonfunctioning islet tumor of the pancreas as the primary lesion in postoperative periodical scrutinies.

A nonfunctioning islet cell carcinoma with tumor thrombus in both the portal and splenic veins — a case report of successful resection

A rare case of nonfunctioning islet cell carcinoma associated with tumor thrombi in both the portal and splenic veins is reported. The patient, a 49-year-old male, had a 2-year history of occasional abdominal pain. Computed tomography (CT) disclosed a huge mass in the body of the pancreas, and celiac arteriogram showed a tumor stain in the body and tail of the pancreas. Percutaneous transhepatic portography (PTP) demonstrated an irregular filling defect, indicating intraportal tumor growth. Curative surgery, which included total pancreatectomy with combined resection (50 mm in length) and reconstruction of the portal vein, distal gastrectomy, and partial resection of the transverse colon, was performed. Histological examination of the surgical specimen led to a diagnosis of nonfunctioning islet cell carcinoma with a negative immunohistochemical stain for insulin, glucagon, somatostatin, and adrenocorticotropic hormone. The patient has been well for 38 months to date without any sign of tumor recurrence. Our experience with this case has introduced a radical resection for islet cell tumor of the pancreas, even if the tumor has extended into the portal vein.

Nonfunctioning islet cell carcinoma of the pancreas with an initial appearance of massive hemorrhage

Diagnosis of nonfunctioning tumors is difficult, since they often lack apparent clinical signs and symptoms. Here we report nonfunctioning islet cell carcinoma of the pancreas with massive hemorrhage. A 57-year-old woman who had sudden severe abdominal pain and high fever was admitted to our hospital. A series of examinations after admission revealed cystic tumor located at the tail of the pancreas and a hematoma adjacent to the tumor. The plasma levels of hormones, except for prolactin, were normal. After antibiotic treatment, blood transfusion, and other conservative therapy had produced a stable condition, operation was performed. The operation revealed a tumor measuring 6.2×7.8×7.2 cm located at the pancreatic tail and a hematoma 3.2×2.7×2.5 cm adjacent to the tumor. Distal pancreatectomy, including the tumor, combined with splenectomy and removal of the hematoma, was carried out. The surgical specimen showed a nonfunctioning islet cell carcinoma arising from the pancreatic tail; this tumor had ruptured and was bleeding. Seventeen months after the operation, the patient is alive without recurrence or symptoms. The major characteristic in this patient was the sudden severe abdominal pain and shock due to bleeding from the tumor, which features may not have been reported in the literature.

Nonfunctioning islet cell carcinoma of the pancreas.

Nonfunctioning islet cell carcinoma of the pancreas has a variable and often indolent natural history, which has resulted in a wide range of treatment recommendations. To more clearly define the natural history and appropriate treatment of this disease, we reviewed our institutional experience over the last 39 years. The records of all patients confirmed to have a nonfunctioning islet cell carcinoma of the pancreas were retrospectively reviewed. Kaplan-Meier life tables were constructed and log-rank comparisons were performed. The 73 patients studied had an overall 5-year actuarial survival rate of 50%. Patients with localized disease at presentation (n = 39) had a significantly higher survival rate (p = 0.03) compared with patients with metastatic disease (n = 34). The 19 patients who underwent a potentially curative resection of the primary tumor had a significantly higher survival rate (p = 0.03) compared with the 20 patients with locally advanced, unresectable, nonmetastatic disease. Nine of these 20 patients died of complications of the primary tumor. In contrast, only 2 of 22 cancer-related deaths in the 34 patients with metastatic disease at diagnosis were due to the primary tumor. (1) Surgical resection should be performed in patients with resectable nonmetastatic disease. (2) Resection of the primary tumor in the presence of metastatic disease is rarely indicated. (3) Innovative treatment strategies are needed for patients with locally advanced, unresectable, nonmetastatic tumors of the pancreatic head.

A CASE OF MALIGNANT NONFUNCTIONING ISLET CELL TUMOR OF THE PANCREAS

This is a report of a case of nonfunctioning islet cell carcinoma which occupied almost the entire upper abdomen in a 72-year-old female complaining of an upper abdominal tumor and epigastric pain. She had the tumor for 10 years and it's size increased slightly over the last three years. Enhanced CT pictures showed irregular tumor staining and a cystic pattern. An angiography revealed the hypervascular tumor. Serum levels of various endocrine hormones were within normal range. Therefore, the tumor was thought to be a nonfunctioning islet cell tumor of the pancreas. During surgery it was found that the tumor was located in the tail and body of the pancreas, and was 15×13×8.0 cm in size with macroscopic small metastatic lesions in the liver. Postoperative histopathological examination revealed malignancy with venous and lymphatic invasions.Nonfunctioning islet cell tumors are rare, have a high frequency of malignancy, but grow slowly, and the prognosis after surgical resection is generally good. In this case recurred liver metastasis could be detected by remarkable increase in serum neuron specific enolase which had been monitored after surgery, its availability as a tumor marker being suggested.Aiming at life-prolongation, intensive treatments including intra-arterial infusion chemotherapy and transhepatic arterial embolization are now in consideration.

Frantz's tumor: A papillary and cystic tumor of the pancreas in girls

Four girls with Frantz's tumor, a papillary and cystic tumor of the pancreas, are studied and discussed in comparison with 112 cases in the literature, including 58 Japanese cases. The neoplasms occur predominantly in girls and young women. Up to recently, the tumors have possibly been misclassified as nonfunctioning islet cell tumor or carcinoma, acinar cell carcinoma, papillary cystadenocarcinoma, or pancreatoblastoma according to microscopic findings and frequently have been managed with aggressive surgery such as pancreatoduodenectomy. The tumors are well encapsulated and the cut surfaces show characteristically solid and hemorrhagic-necrotic patterns. Ultrasonography and CT scan are the most useful tools for the diagnosis. The neoplasms usually behave like a very low-grade malignancy, so that complete removal is the treatment of choice for the tumor arising anywhere in the pancreas. Immunochemical and electron microscopic studies can differentiate Frantz's tumor from other neoplasms and also suggest that the tumors originate from primordial cells or multipotential stem cells capable of differentiating into both exocrine and endocrine lines.

Hemorrhagic pancreatitis: A complication of transcatheter embolization treated successfully by total pancreatectomy

Since pancreatitis can be produced experimentally in dogs by embolization of microspheres into the pancreatic arterial circulation, there has been speculation that intentional or inadvertent embolization of the pancreas in human subjects could also produce pancreatitis. Although such therapeutic embolization has increased, no pathologically documented case of this complication has been recorded. We have reported the first such case occurring in a patient with a large, highly vascular, nonfunctioning islet cell carcinoma of the tail of the pancreas preoperatively embolized with Gianturco coils and Gelfoam particles suspended in sodium tetradecylsulfate solution to facilitate distal pancreatectomy. The resultant hemorrhagic pancreatitis and duodenal necrosis required a total pancreatectomy. We conclude that, by itself, occlusion of the origin of the splenic and gastroduodenal arteries with coils would have been effective and without complication; however, the addition of Gelfoam particles in a sclerosing solution reduced the microscopic pancreatic circulation to a critical point and resulted in hemorrhagic pancreatitis.

CT features of nonfunctioning islet cell carcinoma.

To determine the computed tomographic (CT) characteristics of nonfunctioning islet cell carcinoma of the pancreas, the CT scans of 27 patients with that disease were reviewed. The pancreatic tumor was identified as a mass in 26 patients (96%). Demonstrated masses were 3-24 cm in diameter. Eight of the tumors (31%) were larger than 10 cm. Six tumors (22%) contained calcification. Of the 25 tumors evaluated with contrast enhancement, 20 became partially or diffusely hyperdense relative to nearby normal pancreatic tissue. Hepatic metastases were identified in 15 patients (56%), regional lymphadenopathy in 10 (37%), atrophy of the gland proximal to the tumor in six (22%), dilatation of the biliary ducts in five (19%), and dilatation of the pancreatic duct in four (15%). The CT appearances of the nonfunctioning islet cell tumors were compared with those of 100 ordinary (ductal) pancreatic adenocarcinomas. Although the two types of tumors were sometimes indistinguishable, features found to be more characteristic of islet cell carcinoma included a pancreatic mass of unusually large size, calcification within the tumor, and contrast enhancement of either the primary tumor or hepatic metastases. Involvement of the celiac axis or proximal superior mesenteric artery was limited to ductal carcinoma.

Serum neuron-specific enolase: A serum marker for nonfunctioning pancreatic islet cell carcinoma

The isoenzyme, neuron-specific enolase (NSE) was evaluated as a serum marker for nonfunctioning pancreatic islet cell carcinoma. Serum NSE was measured by radioimmunoassay in 6 patients with islet cell cancer and in 22 healthy adults. Mean serum NSE in control subjects was 5 ng/ml (range 3.2 to 8.4 ng/ml). Three of six patients had clearly elevated serum NSE with values that ranged from 22 to 44 ng/ml. One patient had an equivocally elevated serum NSE of 9.2 ng/ml while receiving chemotherapy. When relapse occurred, the NSE level increased to 44 ng/ml. One of two patients with normal serum NSE had undergone radical pancreatectomy and had no evidence of disease when studied. Elevated serum NSE returned to normal limits after removal of all gross tumor in the only patient from whom a preoperative measurement was obtained. Results of this preliminary study suggest that serum NSE levels can aid in the diagnosis and monitoring of the course of nonfunctioning islet cell carcinoma.

New role of angiography in management of malignant tumors in the body of the pancreas.

1. Preoperative arteriography was performed on 45 patients with malignant tumors in the body of the pancreas, which extended to the surroundings far beyond the border of the gland. 2. According to the pattern of tumor infiltration to the splenic artery, these were divided into the normal appearing type, the smooth tapering type, the rough tapering type and the obstructing type, the incidence of which was six, nine, twenty-one and nine, respectively. The former two types belonged to the expansive group, which consisted of cystadenacarcinoma, nonfunctioning islet cell carcinoma, acinar cell carcinoma, sarcoma and carcinoid as well as ductal cell carcinoma. The latter two belonged to the infiltrative group, all of which were ductal cell carcinomas. 3. Almost all of the tumors with increased vascularity belonged to the expansive group, on which effect of chemotherapy might be expectable. 4. On laparotomy, the tumors with the expansive group tended to be associated with liver metastasis, while those with the infiltrative group often showed peritoneal dissemination rather than hematogenous metastasis. 5. Removal of the main tumor could be performed fairly easily for lesions with the normal appearing type, but none of those with the obstructing type was resected. 6. The longest survival was in those with the normal appearing type. In contrast, the poorest prognosis was in those with the obstructing type. In the smooth tapering and the rough tapering types, the prognosis was also poor, but removal of the main tumor in the smooth tapering type resulted in prolongation of survival time. 7. It was found that splenic arteriography can be used as a part of the evaluation of malignant tumors in the body of the pancreas, for which only a few available tools to define its clinical aspects as well as its management have yet been established.

Prolonged survival with islet cell carcinoma producing obstructive jaundice

An unusual case of nonfunctioning islet cell carcinoma is presented. The patient presented with obstructive jaundice and survived thirteen years after palliative cholecystojejunostomy. Five other cases of obstructive jaundice produced by islet cell carcinomas of the pancreas have been reported. The prognosis of these tumors is considerably better than with the usual periampullary carcinoma and possibly better than with most islet cell carcinomas. Definite microscopic diagnosis of nonresectable tumors of the periampullary area is encouraged.