LARGE CELL METASTATIC PANCREATIC NEUROENDOCRINE CARCINOMA TREATED WITH SOMATOSTATIN ANALOGUES - CASE REPORT AND LITERATURE REVIEW.

We report the case of a 55-year-old-male with a large cell metastatic pancreatic neuroendocrine carcinoma treated for 14 months with lanreotide autogel having a stable disease (SD) and not responding to chemotherapy. The somatostatin analogues (SSA) were introduced after an episode of diarrhea and controlled the disease. Progression-free survival (PFS) as determined by Computerized Tomography (CT) scans was obtained for 14 months. After more than a year, the patient's health state deteriorated along with progressive disease. The capecitabine-temozolomide regimen was challenged, but after three cycles, a rapid clinical decline was noted. CONCLUSION: This unexpected event (diarrhea) in the course of the disease could represent the beginning of carcinoid syndrome. While the lanreotide autogel helped the episode of diarrhea pass, it also helped gain control over the disease itself.

PROGNOSTIC FACTORS IN PATIENTS WITH SURGICAL RESECTION OF PANCREATIC NEUROENDOCRINE TUMOURS.

CONTEXT: Pancreatic neuroendocrine tumours (PanNETs) are rare pancreatic neoplasms. PanNETs can be treated by multimodal approach including surgery, locoregional and systemic therapy. OBJECTIVE: The aim of the present study is to evaluate predictive factors of overall survival in patients with PanNETs surgically treated at a single center. SUBJECTS AND METHODS: The study group consisted of 120 patients with PanNETs who had undergone surgery at the Center of Digestive Diseases and Liver Transplantation of Fundeni Clinical Institute, Bucharest, Romania. Surgical resection of the primary tumor was performed in 110 patients. RESULTS: Tumor size > 2 cm (p=0.048) (90% CI) lymph node involvement (p=0.048), ENET grade (p<0.001), distant metastases (p<0.001), Ki 67 index (<2%, 2-5%, 5-10%, 10-20%, >20%) (p<0.001) were identified as significant prognostic factors for OS on univariate analysis. Using multivariate Cox proportional regression model we found that distant metastases and Ki 67 index were independent risk factors for the survival outcome. CONCLUSIONS: Surgery with curative intent should be considered in all cases if clinically appropriate and technically feasible. High grade (Ki67 index ≥10%) tumours were associated with a 2- fold increase in risk of death as compared to those with a Ki67 <10%.

Extensive Left Iliac Veins and Inferior Vena Cava Thrombosis Revealing a Giant Uterine Myoma.

A deep vein thrombosis was rarely associated with uterine myomas. Hereby, it is presented the case of a 40-year-old woman in which the clinical manifestation of the deep vein thrombosis revealed the further diagnosis of a large uterine myoma. The diagnosis, management and clinical outcome of the patient are emphasized and discussed. The management of a patient with a uterine myoma and deep vein thrombosis is challenging and implies a multidisciplinary team.

Metachronous Ampulla of Vater Carcinoma after Curative-Intent Surgery for Klatskin Tumor.

Resection represents the single hope for long-term survival in a patient diagnosed with a hilar cholangiocarcinoma (Klatskin tumor). However, the largest part of these patients develops a recurrent disease. Second metachronous periampullary cancers after a curative-intent surgery for a Klatskin tumor represent an exceptional pathology, and the management of these patients was poorly documented. Hereby, it is presented a 32-year-old patient with bile duct resection, left hemi-hepatectomy and loco-regional lymph nodes dissection, for a type IIIB Bismuth-Corlette Klatskin tumor, which, furthermore, 6 years later, underwent a pancreaticoduodenectomy for a metachronous carcinoma of the ampulla of Vater. The management and outcomes were discussed in the reported case, along with a literature review of the previously published patients. In conclusion, a metachronous periampullary carcinoma after resection of a Klatskin tumor should be distinguished from a loco-regional recurrent disease. While most of the patients with recurrences are suitable to only chemotherapy and or radiotherapy, a second curative-intent surgery (i.e., pancreaticoduodenectomy) is feasible in the largest part of the patients with a metachronous cancer, with good long-term outcomes.

Emphysematous cystitis: an unreported complication after pancreaticoduodenectomy.

Pancreaticoduodenectomy was associated with an increased morbidity, and septic complications are the primary cause of death in these patients. However, severe sepsis, caused by a postoperative urinary tract infection, is uncommon. It is presented the case of a 72 years old man, with a pylorus-preserving pancreaticoduodenectomy for an ampullary adenocarcinoma. Postoperatively, the patient developed a chyle leak, and a severe urinary tract infection (i.e., emphysematous cystitis), with septic shock. The diagnosis, management and outcome are discussed. In conclusion, emphysematous cystitis is a potentially life-threatening complication, which may occur due to the postoperative immunodepression after pancreaticoduodenectomy.

A posterior approach pancreaticoduodenectomy with portal vein resection in a large adenocarcinoma of the uncinate process of the pancreas - case report.

A portal vein invasion is no longer a contraindication for resection in pancreatic cancer, but increased morbidity and mortality rates can be encountered. Hereby it is presented the case of a patient diagnosed with a large adenocarcinoma of the uncinate process of the pancreas, who underwent aposterior approach pancreaticoduodenectomy, with en bloctang ential resection of the portal vein, and total mesopan creasexcision. A posterior approach allows a negative resection margins pancreaticoduodenectomy, with a good local control of the disease, despite the in creas.

Impact of a portal/superior mesenteric vein resection during pancreatico-duodenectomy for pancreatic head adenocarcinoma.

AIM: The impact of venous resection (VR) in pancreatico-dudenectomy (PD) for pancreatic adenocarcinoma (PDAC) is controversial. The aim of the study is to comparatively assess the postoperative outcomes after PD with and without VR for PDAC and to identify predictors of morbidity and survival in the subgroup of PD with VR. METHODS: The data of 51 PD with VR were compared with those of 183 PD without VR. Binary logistic regression and Cox survival analyses were performed. RESULTS: Both the operative time and estimated blood loss was significantly higher in the VR group (P<0.001). A trend towards an increased 90-day mortality (9.8% vs. 5.5%) and severe morbidity (20% vs. 13%) was observed when a VR was performed (P ≥0.264). The median overall survival time after the PD with and without VR was 13 months and 17 months, respectively (P=0.845). The absence of histological tumor invasion of the VR was found as the only independent predictor for a better survival (HR=0.359; 95% CI 0.161-0.803; P=0.013). CONCLUSION: A PD with VR can be safely incorporated in a pancreatic surgeon armamentarium. However, the trend towards increased mortality and severe morbidity rates should be expected, along with higher operative time and blood loss, compared with PD without VR. Associated VR does not appear to significantly impair the prognosis after PD for PDAC; however, histological tumor invasion of the VR has a negative impact on the survival.

An unclassified congenital bile duct cyst.

Congenital bile duct cysts are rare in adulthood. The most frequently used classification was proposed by Todani in 1977. However, in rare cases, not all the bile duct cysts are suitable to this classification. Hereby, we describe the case of an unclassified and very rare form of congenital bile duct cyst--isolated cystic duct cyst. En-block resection of the cyst, along with gallbladder, is the treatment of choice. Although exceptional, cystic duct cysts should be included in Todani classification so that the surgeons to be aware for this variation.

The Todani classification for bile duct cysts: an overview.

Bile duct cysts are a rare medical condition and are more frequent in children. However, the disease is becoming increasingly common in adults. The modified Todani classification, which is based on anatomical characteristics, is the current standard classification method. However, this classification does not take the following factors into consideration: different epidemiology, pathogenesis, risk of malignant transformation, clinical and imaging aspects, and different therapeutical approaches for all the bile duct cysts. Thus, some clinicians denied its clinical significance and viability. Moreover, some rare variants (i.e., cystic duct cysts) of bile duct cysts were initially not included and were subsequently categorized as type VI. Although it clusters different diseases, the Todani classification of bile duct cysts should also be used in clinical practice because it is simple, reproducible and widely agreed upon, thereby allowing an appropriate comparative analysis between different series of patients who are classified based on this scheme. Exceptional, cystic duct cysts should be included in the Todani classification (as a subtype of type II BDC rather than as a "new" type VI) so that the gastroenterologists, radiologists and surgeons are aware of this variation.

POSSUM, a potentially useful tool for prediction of morbidity in patients undergoing central pancreatectomy.

AIM: Central pancreatectomy is a pancreas-sparing alternative to standard pancreatic resections, and it is associated with substantial morbidity. The aim of the present study is to assess the utility of the POSSUM scoring system in the prediction of the postoperative complications after central pancreatectomy, which would help identify the patients who are at the highest risk of developing complications. METHODS: A retrospective analysis of 24 patients who underwent central pancreatectomies (2002-2010) was performed. The POSSUM score was calculated for each patient and was correlated with observed morbidity. RESULTS: The mean POSSUM score was 32, thus predicting morbidity in 9 out of 24 patients. This risk assessment proved to be quite accurate, as 13 patients (54%) actually developed postoperative complications (chi-squared = 3.2101, p = 0.073). The predictive value of the POSSUM was strongest for the < or = 20%, 60-80% and > or = 80% morbidity risk cohorts (O/E ratio 1). CONCLUSIONS: The identification of a scoring system to predict the development of severe complications after central pancreatectomy may stratify the patients' risk and lead to a tailored approach of this surgical procedure. Although POSSUM seems to predict morbidity after central pancreatectomy, further studies involving larger numbers of patients should be conducted to confirm this effect.

Dorsal pancreas agenesis and ductal adenocarcinoma: surgical implications of an extremely rare association.

It is presented the case of a 44-year-old woman with ductal adenocarcinoma of the pancreatic head with associated dorsal pancreas agenesis. In this case, curative intent surgery implies removal of the whole pancreas with its consequences--parental insulin requirement and exocrine pancreatic insufficiency. A review of the literature on the previously reported cases was performed.

Pathological characteristics and clinical specifications in gastroenteropancreatic neuroendocrine tumors: a study of 68 cases.

INTRODUCTION: Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) represent a group of tumors, having their origin in cells of diffuse endocrine system, with particular clinical course, diagnosis and treatment. PATIENTS AND METHODS: In our study, were included 68 patients with neuroendocrine digestive tumors admitted, diagnosed and treated in Fundeni Clinical Institute, Bucharest, in the last ten years--2000-2010 (retrospective study). RESULTS: Thirty-three (49%) patients were males, 35 (51%) females, and the main age was 58.9 years. In 62 (90.3%) cases was possible to find the primary tumor. The examined tumors had different localizations: pancreas--32 (47.04%) cases (head--17 (24.99%) cases, and body and tail--15 (22.05%) cases), stomach--7 (10.29%) cases, small intestine--7 (10.29%) cases, 6 (8.82%) cases--unknown primary site (diagnosis was established on metastases), right colon--6 (8.82%) cases, liver--6 (8.82%) cases, rectum--2 (2.94%) cases, and retroperitoneum--2 (2.94%) cases. Microscopic examination revealed 59 (86.8%) malignant tumors and 9 (13.2%) benign tumors. Using WHO 2000 Classification, 28 cases of malignant tumors were well-differentiated neuroendocrine carcinomas, and 31 cases were poor differentiated neuroendocrine carcinomas. From malignant cases, 25 (42.3%) have distant metastases and 15 (25.9%) lymph node metastases. CONCLUSIONS: Cases of gastroenteropancreatic neuroendocrine tumors included in our study had clinical and histopathological features in correspondence with data from literature--slight predominance in women, predominance in 5th and 6th decades of life, the most frequent localizations were at pancreatic level--both head and body and tail, but the rarest were in colon and retroperitoneum. Most of the cases studied, were malignant tumors, from these more than a half were poor differentiated, and a quarter of them having lymph node or distant metastases.

Left-sided duplication of inferior vena cava: clinical implications in a patient with sigmoid adenocarcinoma.

Double inferior vena cava is a rare congenital anomaly, usually detected by computed tomography or magnetic resonance imaging. Although asymptomatic, it may have a clinical significance, as it may mimic a para-aortic lymphadenopaty. A case of left-sided duplication of the inferior vena cava in a patient with sigmoid colon cancer is presented. The diagnostic pitfalls and clinical implications are discussed. Accurate preoperative assessment of such an anatomical variant is of utmost importance, this way potentially life-threatening surgical complications, particularly when a minimally invasive approach is planned, are prevented.

[Pancreatoduodenectomy--past, present and future]. / Duodenopancreatectomia cefalica--trecut, prezent si viitor.

Pancreatico-duodenectomy represents one of the most important and complex surgical digestive procedure. Although rarely performed in the past, nowadays has become a routine surgery. Moreover, postoperative mortality significantly decreased (from over 30% to less than 5%), while the number of severe, life-threatening complications were reduced. This outcome was possible due to technical innovations acquired in time, and also due to a better per operative management of these patients, in tertiary surgical centers, with experienced operative teams. Some modifications of the standard procedure of resection, like posterior or artery first approach contributed to better results, facilitating en-block resection of the portal/ superior mesenteric vein, where needed. Moreover, posterior approach facilitates complete mesopancreas excision, involved in local recurrence after resection of pancreatic head adenocarcinoma. Regarding reconstruction after pancreaticoduodenectomy, there is no optimal type of anastomosis (pancreatico-gastrostomy vs pancreatico-jejunostomy), results being related mostly with the expertise of the operative team, as like the postoperative pancreatic fistula rate. Future studies are deemed to look on the real clinical impact of the total mesopancreas excision in achieving negative resection margins, decreasing local recurrence and increasing the long-term survival of patients resected for pancreatic head cancer.

Emergency pancreaticoduodenectomy in a patient with an ampullary adenocarcinoma associated with severe acute pancreatitis: a case report.

Emergency pancreatico-duodenectomy is an infrequent surgical procedure. Herein, it is reported the case of a 58-year old man with ampullary carcinoma revealed by acute necrotizing pancreatitis. Due to clinical features, an emergency two-step pancreatico-duodenectomy was performed, accomplished by pancreatic and peripancreatic necrosectomy. The distal remnant pancreas was drained as an external pancreatic fistula. A time later, after complete remittance of local inflammation, the pancreas was anastomosed to the jejunum. The postoperative outcome was uneventful, the patient being disease-free at more than 5 years after surgery. Thus, pancreatico-duodenectomy is a feasible and safe operation when performed in tertiary hepato-bilio-pancreatic centers, in selected cases.

[Diagnosis and surgical management in gastrointestinal neuroendocrine tumors]. / Managementul diagnosticului si tratamentul chirurgical în tumorile neuroendocrine gastrointestinale.

Neuroendocrine tumors, known as carcinoid tumors constitute a heterogeneous group of neoplasms that present many clinical challenges. They secrete peptides and neuroamines that cause specific clinical syndromes. Assessment of specific or general tumors markers offers high sensitivity in establishing the diagnosis and they also have prognostic significance. Management strategies include curative surgery, whenever possible-that can be rarely achieved, palliative surgery, chemotherapy, radiologic therapy, such as radiofrequency ablation and chemoembolisations and somatostatin analogues therapy in order to control the symptoms. The aim of this paper is to review recent publications in this field and to give recommendations that take into account current advances in order to facilitate improvement in management and outcome.