Diagnosis and treatment of functional pancreatic dndocrine tumor / 中华内分泌外科杂志

Objective To explore the diagnosis and management of functional pancreatic endocrine tumor.Methods Clinical data of 19 cases of functional pancreatic endocrine tumor were retrospectively analyzed.Results 15 cases of insulinoma,2 cases of gastrinoma and 2 cases of glucagonoma were qualitatively diagnosed.The positive rate of preoperative diagosis for type B ultrasonic inspection,CT,MRI,EUS,selective portovenous sampling and intraoperative type B ultrasonic inspection was 15.8％ (3/19),67.5％ (10/16),71.4％ (5/7),87.5％ (7/8),100％(2/2) and 85.7％(6/7) respectively.Of the total 19 cases,7 cases underwent open surgery,11 cases unde rwent laparoscopic surgery,and one case didn't undergo any surgery as liver metastasis had occurred when glucagonoma was diagnosed.The operation methods included tumor enucleation (n=13),distal pancreatic resection (n=3),distal pancreatic resection plus splenectomy (n=1),and pancreatic head resection with duodenum preserved (n=1).Conclusions The measurement of serum insulin,gastrin and glucagon is the main basis for qualitative diagonosis of pancreatic endocrine tumor.Two stage spinal CT thin scanning is the main method for tumor location.Intraoperative type B ultrasonic inspection is the supplement to preoperative location.Tumor enucleation is the main choice of treatment.

Nonfunctional islet cell tumor: a report on 26 cases and review of the literature / 中华肝胆外科杂志

Objective To review our experience in the diagnosis and treatment of nonfunctional islet cell tumors(NICT).Method The clinicopathological data of 26 cases of NICT were retrospectively analysed and the medical literature was reviewed.Results In addition to the 26 cases seen in our center,there were 569 cases published in 37 articles in the medical literature.In China,NICT was more common in women.The male to female ratio was about 3 ∶ 7.The mean age of onset of the disease was 35 years old.Most NICT were solitary and malignant,surgery was effective in prolonging long-term survival.Conclusions NICT is rare and it had no specific clinical presentation.BUS and CT are useful for diagnosis and surgery is effective.

A Case of a Calcitonin-Secreting Pancreatic Endocrine Tumor in a Pateint with Multiple Endocrine Neoplasia Type 1 / 대한내과학회지

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant hereditary disorder characterized by the combined occurrence of tumors of the parathyroid gland, exocrine pancreas, and anterior pituitary gland. Calcitonin-secreting pancreatic endocrine tumors are rare, and calcitonin-secreting pancreatic endocrine tumors with MEN1 have not been reported in Korea. A 46-year-old woman was admitted for a right breast cancer operation. Abnormal blood chemistry findings were hypercalcemia and elevated calcitonin. The patient was diagnosed with a calcitonin-secreting pancreatic endocrine tumor, left thyroid papillary carcinoma, right breast invasive ductal carcinoma, a thymic carcinoid tumor, left adrenal adenoma, uterine myoma, and adenomyosis by computed tomography scan, and with pituitary macroadenoma by brain magnetic resonance imaging. We present this case with a review of the literature, because it is the first reported calcitonin-secreting pancreatic endocrine tumor with MEN 1 in Korea.

Recent Update of Radiologic Findings and Interventional Methods of Pancreas Endocrine Tumor / 대한내과학회지

Pancreatic endocrine tumors (PET) are rare neoplasms of the pancreas accounting for less than 5% of all primary pancreatic malignancies. Insulinomas, gastrinomas, glucagonomas and somatostatinomas is included in PET. PETs are usually classified into functioning and non-functioning tumors and presents with a range of benignity or malignancy. It is very important to accurate diagnose the PET location and to predict the benignity or malignancy of PET in terms of the treatment strategy, because PET have higher respectability, better response to chemotherapy and better prognosis compared to that of pancreatic adenocarcinoma. The utility and reliability of different imaging modalities depends on the characteristics of PETs, specifically their size. Functioning PET tend to be small (less than 2 cm), well circumscribed, homogeneous, and usually shown as strong enhancement on contrast enhanced CT or MR imaging. Non-functioning PET tend to be larger (4~10 cm), heterogeneous, and may contain the cystic areas of degeneration and necrosis. In this article, we present the various imaging findings of PET according to recent WHO classification.

Proservação de paciente tratado por tumor gigante neuroendócrino não-funcionante em cauda de pâncreas / Monitorament of patient treated by huge non-functioning neuroendocrine tumor in the body and tail the pancreas

Os tumores neuroendócrinos do pâncreas não-funcionantes são raros. Os sintomas são crônicos, o diagnóstico pré-operatório é difícil e o tratamento cirúrgico exige a presença de um cirurgião de larga experiência clínico-cirúrgica. O objetivo é apresentar um paciente com tumor gigante de corpo e cauda de pâncreas, com acometimento de órgãos adjacentes e com metástases hepáticas. O paciente submeteu-se à ressecção de múltiplos órgãos e colostomia terminal, com boa evolução pós-operatória. Recebeu tratamento quimioterápico e mesmo com a remanescência da lesão após 14 meses, sobreviveu por 21 meses. A apresentação justifica-se pela baixa incidência de tumores dessa natureza, pela contribuição ao conhecimento da evolução clínica desses pacientes e para aumentar a casuística da doença, impossível de ser estabelecida de outra forma senão com relatos de casos.

The nonfunctioning neuroendocrine tumors of the pancreas are rare. The symptoms are chronic, the preoperative diagnosis is difficult and the surgical treatment demands an experienced surgeon with clinical surgical practice. The objective is to present a patient with a huge tumor in the body and tail of the pancreas with extension to adjacent organs and liver metastases. The patient was submitted to multiple organs resection and terminal colostomy, and made good postoperative progress. The patient received chemotherapy treatment and, even with the lesion remaining for the past 14 months, survived for 21 months. The presentation is justified by the low-incidence of this kind of tumor, the contribution to knowledge of the clinical evolution of these patients and the contribution to increasing the casuistry of the disease, which is only possible to be established by reporting of cases.

Carcinoma suprarrenal gigante / Giant suprarenal carcinoma

OBJETIVO: presentar un caso de carcinoma suprarrenal de gran tamaño, su diagnóstico y tratamiento. DESCRIPSIÓN: se presenta un paciente del sexo masculino, de 69 años de edad, con antecedentes de diabetes mellitus tipo II que refería palparse un tumor abdominal. Al examen físico tenía un tumor palpable en la fosa lumbar y flanco izquierdos de aproximadamente 15 a 16 cm de diámetro. El tumor se corroboró en el ultrasonido, por lo que fue necesario realizar la tomografía axial computadorizada simple y contrastada para establecer el origen suprarrenal de este. Se comprobaron niveles elevados de cortisol en plasma. INTERVENSIÓN: se realizó la suprarrenalectomía más la nefrectomía izquierdas. La evolución posoperatoria resultó satisfactoria. El informe histopatológico de la pieza quirúrgica fue carcinoma suprarrenal con infiltración renal. Se indicó tratamiento de quimioterapia con mitotano con posterioridad a la cirugía. En los controles semestral y anual realizados, el paciente se encontró asintomático y con niveles de cortisol sanguíneo normales. El ultrasonido, la tomografía axial computadorizada del abdomen y la radiografía del tórax no mostraron recidiva tumoral ni metástasis. CONCLUSIONES: los carcinomas suprarrenales pueden evolucionar de forma asintomática hasta alcanzar grandes dimensiones. El tratamiento combinado, quirúrgico y con quimioterapia, mejora el pronóstico de estos pacientes.

OBJECTIVE: this is the presentation of a case of a very large suprarenal carcinoma. DESCRIPTION: a male patient aged 69 with a history of type 2 diabetes mellitus mentioning the palpation of a abdominal tumor. At physical examination we noted the presence of a palpable tumor in lumbar fossa and left flanks of approximately 15 to 16 cm diameter. Ultrasound corroborated the tumor being necessary to perform a single and contrasted computed tomography to establish its suprarenal origin. There were high levels of cortisol in plasma. INTERVENTION: a left suprarenalectomy and nephrectomy were performed. Postoperative course was satisfactory. The histopathology report of the surgical specimen was a suprarenal carcinoma with renal infiltration. Chemotherapy with mitotane was prescribed after surgery. In half-yearly and yearly controls the patient was asymptomatic and with normal blood cortisol levels. Ultrasound, computerized axial tomography of abdomen and thorax X-rays not showed tumor relapse neither metastasis. CONCLUSIONS: suprarenal carcinomas may to evolve in an asymptomatic way and to have bid dimensions. Combined treatment of surgery and chemotherapy, improves the prognosis of these patients.

Enucleation of Pancreatic Endocrine Tumor Following Pancreatic Duct Stenting: A Case Report

Pancreatic fistula is the most frequent complication after pancreatic resection regardless of the extent of the resection. A 68-year-old woman with B-viral hepatitis was referred with an incidentally detected pancreatic head mass that was diagnosed 4 months previously when performing following up of her liver cirrhosis. She had no specific symptoms, but she had a 1.2 cm sized solitary mass that was suspected to be a pancreatic endocrine tumor and it was located very close to the main pancreatic duct in the pancreas uncinate process on the imaging workup. Preoperative endoscopic pancreatic stenting was prepared to guide the enucleation of the mass while identifying the pancreatic duct using intraoperative ultrasonography. Precise intraoperative estimation of the mass and the pancreatic duct was possible and the enucleation was successful without injury to the duct. We recommend this operative approach and especially when planning local pancreatic resection for tumors in the pancreatic head or uncinate process, as these tumors make the pancreatic duct injury vulnerable to injury.

On development of pancreatic endocrine surgery in China / 中华肝胆外科杂志

The paper summarized the current situation of diagnosis and treatment of pancreatic endocrine tumors (PETs)in China.In recent sixty years, we understand PETs more deeply, and have all kinds of imaging technique to localize the tumors.It makes more cases having been diagnosed.Operation is the most important and the first choice of treatment.The concept of minimal-invasion runs through the surgical procedure.Multi-model strategy is used in therapy for malignant PETs with liver metastasis.Basic research is focused in mechanism and early diagnosis and it helps the clinical practice.Specialization and co-operation are emphasized in the development of PETs diagnosis and treatment.

Ampullary Carcinoid Tumor

The ampullary carcinoid tumor is a rare lesion accounting for less than 0.3% of all carcinoid tumors of the gastrointestinal tract. We recently experienced a case of an ampullary carcinoid tumor at our institution. A 70-year-old man was admitted to our hospital on January 2006 for right upper quadrant pain with jaundice. He underwent a pyloruspreserving pancreaticoduodenectomy for an ampullary carcinoid tumor. Abdominal computed tomography showed an oval shaped mass at the ampulla of Vater with abrupt obstruction of the distal end of the common bile duct. The patient underwent a percutaneous transhepatic bile drainage for a cholangiogram, in which was noted a filling defect with a smooth margin at the ampullary portion. During surgery, a firm, grayish-pink, and fairly well circumscribed tumor measuring 2.5 cm was found in the ampulla of Vater. Histopathologically, the tumor with invasion to the muscular layers consisted of cells with an eosinophillic cytoplasm and uniform, oval hyperchromatic nuclei. Immunohistochemical staining of the tumor was positive for chromogranin, synaptophysin and neuron-specific enolase. The patient had an unremarkable hospital course. Carcinoids of the ampulla of Vater are very rare. Only 105 cases have been reported in the literature, most as single case reports. We report here a case of an ampullary carcinoid tumor with a discussion based on the literature.

Chemoembolization in Synchronous Liver Metastases from Nonfunctioning Endocrine Tumor of Pancreas / 한국간담췌외과학회지

Pancreatic endocrine tumors are rare and represent a heterogeneous group of benign or malignant lesions. In nonfunctioning tumors, lacking the features of an endocrine tumor syndrome, they seldom present until there is advanced local disease. Endocrine tumors of the pancreas, even in case of liver involvement, are generally characterized by a slower evolution and a better prognosis, if compared with ductal carcinoma. This fact gives reason to a radical surgical approach and to the research of any effective adjuvant treatment including hormonal suppression, systemic chemotherapy and hepatic transarterial chemoembolization (TACE). We report a case of synchronous liver metastases from nonfunctioning pancreatic endocrine tumor in 66 year old female patient.

Clinical and Immunohistochemical Characteristics of Pancreatic Neuroendocrine Tumor: Immunohistochemical Analysis of 7 Tumors

BACKGROUND: Neuroendocrine tumors of the pancreas are classified according to the endocrine function as insulinomas gastrinomas somatostatinomas, or nonfunctioning tumors. However, the morphologic features are not different from each other. Therefore, we tried to compare correlations among the morphologic features, endocrine function, and the immunohistochemical reaction with specific monoclonal antibodies to the tumors. METHOD: We reviewed the medical records of seven patients with pancreatic neuroendocrine tumors retrospectively, and analysed the clinical manifestations, the methods of diagnosis, the pathological characteristics and the results of surgery. Additionally, we compared the correlation between the clinical manifestations and the expression of immunohistochemical staining by using six different kinds of monoclonal antibodies to each tumor. RESULTS: The seven pancreatic neuroendocrine tumor patients were treated by surgical excision. Four patients had benign insulinomas, two had nonfunctioning malignant tumor and one patient had a benign nonfunctioning tumor associated with stomach cancer. The pattern of immunohistochemical stain of each tumor was not correlate with the clinical manifestations. CONCLUSION: The morphologic study with H & E stain, even with immunohistochemical staining of pancreatic neuroendocrine tumor, cannot support differentiation of the functional diagnoses, such as insulinoma, gastrinoma, somatostatinoma, nonfunctional tumors and so forth.

Experiences of Endoscopic Ultrasonography in the 5 cases with Pancreatic Insulinoma / 대한소화기내시경학회지

Of the various endocrine tumors affecting the pancreas, insulinomas and gastrinomas are the most common. In order to facilitate surgery, the localization of tumor is important. However, at the first time of presentation, the localization of tumor was often difficult by CT or ultrasonography because the tumor was relatively too small. The introduction of endoscopic ultrasonography has allowed high-resolution imaging of the pancreas that distinguishes structures as small as 2 to 3 mm in diameter. Thus, it has became easier to detect the lesion site of pancreatic endocrine tumor by endoscopic ultrasonography. We report the 5 patients who had pancreatic insulinoma which were detected and localized by endoscopic ultrasonography.

Experiences of Endoscopic Ultrasonography in the 5 cases with Pancreatic Insulinoma / 대한소화기내시경학회지

Of the various endocrine tumors affecting the pancreas, insulinomas and gastrinomas are the most common. In order to facilitate surgery, the localization of tumor is important. However, at the first time of presentation, the localization of tumor was often difficult by CT or ultrasonography because the tumor was relatively too small. The introduction of endoscopic ultrasonography has allowed high-resolution imaging of the pancreas that distinguishes structures as small as 2 to 3 mm in diameter. Thus, it has became easier to detect the lesion site of pancreatic endocrine tumor by endoscopic ultrasonography. We report the 5 patients who had pancreatic insulinoma which were detected and localized by endoscopic ultrasonography.

Duodenal Somatostatinoma: A case report

Somatostatinoma is rare endocrine tumor that was first described in 1977 by Ganda et al. and Larsson et al. simultaneously. It seems nonfunctioning at clinical level. But it may present with diabetes, diarrhea, cholelithiasis, steatorrhea, indigestion, hypochlorhydria, and anemia. In contrast with pancreatic somatostatinoma, duodenal somatostatinoma, in general, is clinically silent. Duodenal endocrine tumors show similar histologic pattern. Therefore, the definite diagnosis is performed by immunohistochemistry and electron microscopic examination. We have experienced a case of somatostatinoma of duodenum in a 62-year-old male. He has complained generalized pruritus for one year and jaundice for 2 weeks. Grossly, the mass was a intraluminary protruding, polypoid lesion with focal mucosal erosion at immediately distal to Ampulla of Vater. Histologically, it showed tall, cylindrical cells with distinct cell membranes, having granular cytoplasm and small innocent looking nuclei. No mitosis was seen. The tumor cells were arraged in small solid groups and trabeculae, separated by fibrovascular stroma. Immunohistochemically, the tumor cells were strongly positive with somatostatin and negative with several other hormonal and neuroendocrine markers. Ultrastructurally, the cytoplasm contains numerous, homogeneous low electron dense secretory granules, which are essentially similar to those seen in normal delta cells.