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Lung cancer (LC) is an aggressive malignancy with early metastatic spread and poor prognosis. Gastrointestinal metastases from primary LC are extremely rare with highly variable presentations. In this report, we review the case of a patient who presented with peritonitis secondary to perforated sigmoid mass as the first manifestation of metastatic squamous cell LC.

Hepatic angiosarcoma (AS) and hepatic epithelioid hemangioendothelioma (HEHE) are rare primary hepatic vascular malignancies (PHVM) that remain poorly understood. To guide management, we sought to identify factors and trends predicting survival after surgical intervention using a national database.In a retrospective analysis of the National Cancer Database patients with a diagnosis of PHVM were identified. Clinicopathologic factors were extracted and compared. Overall survival (OS) was estimated and predictors of survival were identified.Three hundred ninty patients with AS and 216 with HEHE were identified. Only 16% of AS and 36% of HEHE patients underwent surgery. The median OS for patients who underwent surgical intervention was 97 months, with 5-year OS of 30% for AS versus 69% for HEHE patients (P0.001). Tumor biology strongly impacted OS, with AS histology (Hazard Ratio [HR] of 3.61 [1.55-8.42]), moderate/poor tumor differentiation (HR = 3.86 [1.03-14.46]) and tumor size (HR = 1.01 [1.00-1.01]) conferring worse prognosis. The presence of metastatic disease in the surgically managed cohort (HR = 5.22 [2.01-13.57]) and involved surgical margins (HR = 3.87 [1.59-9.42]), were independently associated with worse survival.In this national cohort of PHVM, tumor biology, in the form of angiosarcoma histology, tumor differentiation and tumor size, was strongly associated with worse survival after surgery. Additionally, residual tumor burden after resection, in the form of positive surgical margins or the presence of metastasis, was also negatively associated with survival. Long-term clinical outcomes remain poor for patients with the above high-risk features, emphasizing the need to develop effective forms of adjuvant systemic therapies for this group of malignancies.

Meckel’s diverticulum (MD) is an anomaly of the small intestine from which malignancy may arise. Among MD neoplasms, neuroendocrine tumors (NETs) are considered the most common. However, their metastatic potential and optimal surgical therapy remain ill-defined. In a retrospective analysis of the National Cancer Database (2004–2015), patients with a diagnosis of MD malignancy were identified. Clinicopathologic factors were extracted and tumors arising in MD were compared. In the subgroup of patients with NET, the association between tumor factors and node involvement was investigated. Three hundred twenty primary MD malignancies were captured in the National Cancer Database, consisting of 280 (87.5%) NET. The median age at diagnosis was 64 years. Patients were predominantly male (207, 73.9%) and white (269, 96.1%). Most tumors were well-differentiated (118, 42.1%) and sub-centimeter (median size, 0.7 cm). Distant metastasis was present in a minority (16, 5.7%), and the median overall survival was 114 months in the entire cohort. The regional lymph node status was known in 87 NET patients, out of which 39 (44.8%) harbored node metastasis. Although the risk of node involvement increased with larger tumor size, it remained significant even among sub-centimeter (9 out of 34, 26.5%) and well-differentiated (18 out of 44, 41%) tumors. Regional node involvement was associated with the presence of distant metastasis (p

Although hepatocellular carcinoma remains the most common form of primary hepatic malignancy, a number of other liver neoplasms are being seen and managed by clinicians with increasing frequency. This diverse group of liver tumors includes cholangiocarcinoma, biliary cystadenocarcinoma, squamous cell carcinoma, and mesenchymal and vascular tumors, among others. In particular, intrahepatic cholangiocarcinoma accounts for up to 20% of primary cancers of the liver and is increasing in incidence in the United States and worldwide. The other tumor types are uncommon and vary in their etiology, biology, and management, adding further challenges for the clinical surgeon. As with most other cancer management, a multidisciplinary team approach offers the optimal care for these patients, including medical and radiation oncology, interventional and diagnostic radiology, gastroenterology, as well as an experienced surgeon. Herein, an overview is provided of the important clinical issues relating to the management of patients with these primary hepatic malignancies.

Hepatoid adenocarcinoma is a rare type of extrahepatic cancer characterized by hepatocellular carcinoma-like histology. We present a case of a large solitary mass in the peripancreatic region found to be an isolated lymph node metastasis from an unknown primary hepatoid adenocarcinoma masquerading as an acinar carcinoma of the pancreas.

Background Determining the biologic behavior of neuroendocrine liver metastases (NELM) is important when managing patients with this disease. We sought to define the intraoperative ultrasound (IOUS) characteristics of NELM and correlate with tumor biology and prognosis. Methods Prospective data on patients who underwent IOUS and surgical intervention for NELM were collected, with images digitally recorded, blindly reviewed, and scored for echogenicity. Association between sonographic appearance, clinicopathologic factors and long-term outcomes was analyzed. Results A total of 216 lesions from 65 patients were analyzed, with IOUS identifying at least one additional metastasis than preoperative imaging in 41 patients (63.1%) with subsequent change of surgical strategy in 14 patients (21.5%). Most NELM appeared hypoechoic (49.1%) on IOUS, while 38.9% demonstrated hyperechogenicity and 12% isoechogenicity. Hypoechoic lesions were associated with poorly-differentiated tumor (p = 0.005) and smaller tumor size (p = 0.004). Patients with hypoechoic metastases demonstrated significantly shorter median disease-free survival compared with isoechoic or hyperechoic lesions (9 vs 20 vs 18 months, p = 0.049). Discussion In addition to improved tumor detection of NELM, IOUS was found to be associated with features of tumor biology, specifically tumor grade and risk-of-recurrence. Echogenicity should be considered a potential prognostic factor in the management of patients with neuroendocrine tumors.

Unlike other neuroendocrine tumors of the gastrointestinal tract, management of duodenal and periampullary carcinoids remains controversial. We aimed to determine the metastatic potential and optimal choice of therapy for these neoplasms.A retrospective review of all patients treated at the Johns Hopkins Hospital between 1996 and 2012 was conducted. Clinicopathologic factors associated with lymph nodal involvement and clinical outcomes were evaluated.A total of 101 patients were identified. Eighty (79.2%) tumors arose from the duodenum and 21 (20.8%) from the periampullary area. Thirty-five (34.7%) patients underwent pancreaticoduodenectomy (PD), 12 (11.9%) local resection, 38 (37.6%) endoscopic excision, and 16 (15.8%) patients harbored incidental tumors identified in the specimen after PD for another indication. Lymph node (LN) pathologic evaluation was done in 56 patients, among which 27 (48%) had positive LN. Specifically, LN positivity (LN+) for tumors1 cm in size was 4.5% (1/22), for tumors 1-2 cm 72% (13/18), and for tumors2 cm 81% (13/16). Tumor size was the only factor associated with LN+ (p = 0.029).Lymph nodal involvement is common for duodenal and periampullary carcinoid tumors, particularly among those1 cm in size; therefore, resection with lymphadenectomyfor these larger tumors is recommended.

Background Postoperative complications may have an adverse effect not only on short-term but also long-term outcome among patients having surgery for cancer. A retrospective series of patients who had surgery for colorectal liver metastases (CLM) was used to assess this association. Methods Patients who had surgery with curative intent for CLM from 2000 to 2009 were included. The impact of postoperative complications, patient characteristics, disease stage and treatment on long-term survival was analysed using multivariable Cox regression models. Results A total of 251 patients were included. The median age was 58 (interquartile range 51–68) years and there were 87 women (34·7 per cent). A minor or major postoperative complication developed in 41 and 14 patients respectively, and five patients (2·0 per cent) died after surgery. The 5-year recurrence-free (RFS) and overall survival rates were 19·5 and 41·9 per cent respectively. Multivariable analysis revealed that postoperative complications independently predicted shorter RFS (hazard ratio (HR) 2·36, 95 per cent confidence interval 1·56 to 3·58) and overall survival (HR 2·34, 1·46 to 3·74). Other independent predictors of shorter RFS and overall survival included lymph node metastasis, concomitant extrahepatic disease, a serum carcinoembryonic antigen level of at least 100 ng/dl, and the use of radiofrequency ablation (RFS only). The severity of complications also correlated with RFS (P = 0·006) and overall survival (P = 0·001). Conclusion Postoperative complications were independently associated with decreased long-term survival after surgery for CLM with curative intent. The prevention and management of postoperative adverse events may be important oncologically.

The determination of the primary tumor origin in patients with neuroendocrine tumor liver metastases (NELM) can pose a considerable management challenge. Recent studies have shown that the alternative lengthening of telomeres (ALT) is prevalent in some human tumors, including pancreatic neuroendocrine tumors (PanNET), and can be useful in predicting tumor biology. In this study, we aimed to evaluate the use of ALT as a biomarker in patients with NELM, in particular to predict the site of origin of metastases.Tissue microarrays (TMAs) were constructed using tumor tissue from NELM patients undergoing liver resection between 1998 and 2010. These included 43 PanNET and 47 gastrointestinal carcinoid tumors. The TMAs were tested for ALT using telomere-specific fluorescent in situ hybridization. The association between ALT positivity and clinicopathologic features and long-term outcomes was investigated.Alternative lengthening of telomeres was positive (ALT+) in 26 (29%) of the 90 tumors included in the TMAs. Pancreatic neuroendocrine tumors were ALT+ in 56% of patients, compared with only 4% ALT+ among gastrointestinal carcinoid tumors (p0.001). The specificity of ALT for detecting pancreatic origin was 96% and the positive predictive value was 92%, and sensitivity was 56% and the negative predictive value was 70%. Additionally, ALT was associated with the pattern of metastatic disease: ALT+ NELM were more likely to have oligometastases (p = 0.001) and less likely to be bilateral in distribution (p = 0.05) than were ALT tumors. In addition, ALT+ was associated with improved prognosis in the PanNET patient population.Alternative lengthening of telomeres was found to be a useful biomarker in patients with NELM. This marker can be helpful in guiding therapy by identifying the site of origin in patients in whom the primary site is unknown.

316 Background: The metastatic potential and choice of therapy of duodenal and ampullary carcinoid tumors are poorly understood. We evaluated the local management and outcomes in patients with these uncommon tumors and determined factors predicting risk of nodal involvement. Methods: 117 patients were identified with duodenal or ampullary carcinoid tumors between 1996 and 2012, who were treated in a single high-volume center. Clinicopathologic data and overall survival by local treatment modality were analyzed. Results: Among all patients, 64 (55%) were treated surgically, including 34 (29%) who underwent pancreaticoduodenectomy (PD), 14 (12%) who underwent local resection (partial duodenectomy), and 16 (13.6%) where a carcinoid tumor was found incidentally after PD for another indication. The remaining 53 patients (45%) underwent endoscopic excision. The average tumor size was 1.8 cm (0.1-8.5) and the majority were of duodenal origin (n=93, 80%). Surgical management was more commonly performed for ampullary tumors compared to tumors of duodenal origin (83% vs. 47%, p=0.002), and endoscopic excision was more common with smaller tumors (p=0.001). Most carcinoids were well-differentiated (94%) and 55% were T1/T2. Yet, among the 55 patients in whom lymph nodes were histologically assessed (PD or lymph node sampling), 51% had positive nodes (N+). In addition, on multivariate analysis nodal involvement was strongly associated with tumor size (OR: 9.9, p=0.001). Specifically, tumors larger than 1-cm had positive nodes in more than 70% of cases whereas those ≤1cm had a 5% risk of nodal involvement (1 of 21 patients) (Table). Similar rates of N+ were observed for both duodenal and ampullary carcinoids. With long-term follow-up, only one recurrence was observed (1%). The overall survival was similar among all treatment groups (median=139 months). Conclusions: Lymph node involvement is common in patients with duodenal and ampullary carcinoid tumor, particularly among tumors >1 cm in size. When possible, surgical resection with lymphadenectomy is recommended for such tumors. [Table: see text]

INTRODUCTIONPrimary pancreatic neuroendocrine tumors are a well-established disease entity, however, neuroendocrine metastases to the pancreas from other sites have been scarcely documented. Specifically, pancreatic metastases from a pulmonary carcinoid tumor have only previously been described in a single case report.PRESENTATION OF CASEWe sought to outline our institutional experience of two patients with pulmonary neuroendocrine tumors that developed metastases to the pancreas, confirmed by gross pathology and immunohistochemistry. In both cases, the pancreatic metastases were surgically resected and their pulmonary origin were discovered post-operatively.DISCUSSIONOur findings should raise awareness to the possibility of metastatic disease when evaluating a pancreatic mass in a patient with a clinical history of pulmonary carcinoid tumor. Expert opinion on immunohistochemically differentiating a primary pancreatic neuroendocrine malignancy from a metastasis should be employed in these cases.CONCLUSIONEstablishing this diagnosis pre-operatively could affect the decision to proceed with surgical resection, given the morbidity of pancreatectomy and the unknown long-term clinical outcome of patients with pulmonary carcinoid tumors metastatic to the pancreas.