Duodenal Ampulla Neuroendocrine Tumor with GISTs of the Proximal Jejunum: A Case Report.

Neuroendocrine tumors (NEN) are a type of heterogenous, slow-growing tumors, that only in about half of the cases can be found in the gastrointestinal tract. Half of these is in the small intestine. The ampullary NENs are rare, accounting for less than 1% of gastroenteropancreatic NENs. Gastrointestinal stromal tumors (GIST) are a more common type of tumors of the gastrointestinal tract that consist of pacemaker cells. The occurrence of both tumors simultaneously is rare, but in patients with neurofibromatosis type 1, the co-existence of NEN and GIST is more often. Here we report a case of simultaneous occurrence of a well-differentiated NEN and a GIST in a patient without neurofibromatosis. Also, we provide a short review of the current knowledge and treatment strategies regarding these tumors.

Biological and Histological Assessment of the Hepatoportoenterostomy Role in Biliary Atresia as a Stand-Alone Procedure or as a Bridge toward Liver Transplantation.

Background and objectives: In patients with biliary atresia (BA), hepatoportoenterostomy (HPE) is still a valuable therapeutic tool for prolonged survival or a safer transition to liver transplantation. The main focus today is towards efficient screening programs, a faster diagnostic, and prompt treatment. However, the limited information on BA pathophysiology makes valuable any experience in disease management. This study aimed to analyze the evolution and survival of patients with BA referred for HPE (Kasai operation) in our department. Materials and Methods: A retrospective analysis was performed on fourteen patients with BA, diagnosed in the pediatric department and further referred for HPE in our surgical department between 2010 and 2016. After HPE, the need for transplantation was assessed according to patients cytomegalovirus (CMV) status, and histological and biochemical analysis. Follow-up results at 1-4 years and long term survival were assessed. Results: Mean age at surgery was 70 days. Surgery in patients younger than 60 days was correlated with survival. Jaundice's clearance rate at three months was 36%. Total and direct bilirubin values had a significant variation between patients with liver transplants and native liver (p = 0.02). CMV was positive in eight patients, half with transplant need and half with native liver survival. Smooth muscle actin (SMA) positivity was proof of advanced fibrosis. The overall survival rate was 79%, with 75% for native liver patients and an 83% survival rate for those with liver transplantation. Transplantation was performed in six patients (43%), with a mean of 10 months between HPE and transplantation. Transplanted patients had better survival. Complications were diagnosed in 63% of patients. The mean follow-up period was six years. Conclusions: HPE, even performed in advanced cirrhosis, allows a significant survival, and ensures an essential time gain for patients requiring liver transplantation. A younger age at surgery is correlated with a better outcome, despite early CMV infection.

Extension of Hepatic Resection Ameliorates Survival in Patients with Type IIIa or IIIb Klatskin Tumors Despite Surgical Complications.

Background: Major hepatectomies for hilar cholangiocarcinoma (HC) are associated with high rates of morbidity and mortality. We aimed to evaluate how and if surgical complications related to extended hepatectomies for HC type III and IV according to Bismuth-Corlette classification influence patients long-term survival. Methods: The files of all patients with major hepatectomy for HC and postoperative complications were retrospectively reviewed. Only patients with a complete postoperative follow up have be taken into account for the study. Postoperative morbidity and mortality, length of hospital stay (LOS) as well as overall survival (OS) and disease free survival (DFS) were recorded. Results: Five patients have been found to respond to all inclusion criteria. Three of them required re-operation with one in hospital death. Two patients are still alive and two other died because of the tumor recurrence with a DFS of 36 and 49 months respectively. The actuarial mean OS for the group was 30 months and the actuarial DFS was 26 months. Conclusions: In patients with HC, extensive resections bring a clearly benefit in terms of survival, even though there is an increase in postoperative morbidity and mortality. However, postoperative complications, if managed susccesfully do not interfere with the long-term survival.

Wilkie's syndrome as a cause of anxiety-depressive disorder: A case report and review of literature.

BACKGROUND: Superior mesenteric artery syndrome is a disease with a complex diagnosis, and it is associated with complications that make it even harder to identify. Currently, a frequent association with psychiatric disorders has been noted. Despite numerous case reports and case series, the variability of the disease has not allowed the development of protocols regarding diagnosis and management. CASE SUMMARY: A 33-year-old woman presented with abdominal pain, nausea, and bile vomiting over the last 15 mo, associated with a 15-kg weight loss over the last three months. After the onset of the symptoms, the patient was diagnosed with anxiety-depressive disorder and treated appropriately. Standard examinations excluded an organic cause, and the cause of the symptoms was considered psychogenic. The persistence of symptoms, even under treatment, prompted a computer tomography angiography examination of the abdomen and pelvis. The examination identified emergence at a sharp angle of 13.7° of the superior mesenteric artery, with a reduced distance between the artery and the anterior wall of the aorta up to a maximum of 8 mm. A diagnosis of aortomesenteric clamp was established. Surgical treatment by laparoscopic duodenojejunostomy was performed. Postoperative evolution was marked by a patent anastomosis at 1 mo, with a 10-kg weight gain and improvement of the associated anxiety. CONCLUSION: This case report underlines two major aspects. One aspect refers to the predisposition of patients with superior mesenteric artery syndrome to develop psychiatric disorders, with an excellent outcome when proper treatment is administered. The second aspect underlines the key role of a multidisciplinary approach and follow-up.

Difficulties in diagnosing anorectal melanoma: A case report and review of the literature.

BACKGROUND: Anorectal melanoma is a tumour that is difficult to identify due to its rarity and variability of presentation. Insufficient data published in the literature do not allow for diagnostic and treatment guidelines to be established. Anorectal melanoma has the worst prognosis among mucosal melanomas and is frequently misdiagnosed by standard identification methods. CASE SUMMARY: A 66-year-old woman presented with intermittent anal bleeding, pain, and tenesmus in the past month, with no associated weight loss. Colonoscopy revealed a cauliflower-like tumour with a diameter of 1.5 cm, with exulcerated areas and an adherent clot but without obstruction. Biopsy results identified an inflammatory rectal polyp with nonspecific chronic rectitis. Tumour markers CA 19-9 and CEA were within the normal range. After 6 mo, due to the persistence of symptoms, a pelvic magnetic resonance imaging scan was performed. A lesion measuring 2.8 cm × 2.7 cm × 2.1 cm was identified at the anorectal junction, along with two adjacent lymphadenopathies. No distant metastases were detected. Immunohistochemistry was performed on the second set of biopsies, and a diagnosis of anorectal melanoma was established. Surgical treatment by abdominoperineal resection was performed. Evolution was marked by the appearance of lung metastases at 1 mo postoperatively, detected on a positron emission tomography-computer tomography scan, and perineal recurrence after 5 mo. After molecular testing, the patient was included in an immunotherapy trial. CONCLUSION: This case highlights the difficulty of establishing a definitive early diagnosis of anorectal melanoma, the importance of performing histological analysis on a well-represented biopsy specimen, and the poor prognosis, even with radical surgery.

Neutrophil-to-lymphocyte ratio: a hidden gem in predicting neoadjuvant treatment response in locally advanced rectal cancer?

PURPOSE: The link between the pro-inflammatory status, tumor aggressiveness and treatment response has been well established in multiple cancers. Various hematologic and biochemical variables representing surrogates for inflammation have been used as predictive markers. Our primary aim was to assess the prognostic value of neutrophil-to-lymphocyte ratio (NLR) in evaluating neoadjuvant treatment response in locally advanced rectal cancer (LARC). METHODS: We included 60 consecutive patients with LARC, admitted for surgery, after completing a standard full-course neoadjuvant radio-chemotherapy regimen. NLR and other hematologic parameters were collected one day prior to surgery. Treatment response was assessed on the resection specimens. RESULTS: On univariate analysis, poor responders had a significantly higher NLR value when compared with good responders: 5.81 (5.40-7.28) vs. 3.51 (2.36-4.04), p<0.0001. NLR retained its significance on multivariate analysis, with an OR of 3.51 (1.54-6.57), p=0.001. A NLR cut-off value of 4.50 had the best predictive value for poor response, with an area under the curve (AUC) of 0.85, sensitivity of 83.3% and specificity of 83.3% (p<0.001). Other hematologic ratios, such as the derived NLR (dNLR) and platelet-to-lymphocyte ratio (PLR) were also significant predictors for poor response, although to a lesser extent when compared to NLR. CONCLUSION: NLR is a simple and cost-effective predictor for neoadjuvant treatment response in LARC. As more data is generated, clear cut-off values could provide valuable insight regarding the management of LARC.