Adenomas of the Ampulla of Vater: A comparison of Outcomes of Operative and Endoscopic Resections

Background

Data comparing operative and endoscopic resection of adenomas of the ampulla of Vater are limited. Our aims were to evaluate and compare the long-term results and outcomes of endoscopic and operative resections of benign tumors of the ampulla of Vater as well as to determine which features of benign periampullary neoplasms would predict recurrence or failure of endoscopic therapy and therefore need for operative treatment.

Methods

Retrospective review of all patients treated for adenomas of ampulla of Vater at our institution from 1994 to 2009.

Results

Over a 15-year span, 180 patients (mean age 59 years) were treated for benign adenomas of the ampulla of Vater with a mean follow-up of 4.4 years. Obstructive jaundice was more common in the operative resection group (p?=?0.006). The adenomas were tubular in 83 patients (44 %), tubulovillous in 77 (45 %) and villous in 20 (11 %). Endoscopic resection alone was performed in 130 patients (78 %). Operative resection was performed in 50 patients (28 %), with pancreatoduodenectomy in 40, transduodenal local resection in 9, and pancreas-sparing total duodenectomy in 1. Nine patients who underwent endoscopic resection initially were endoscopic treatment failures. Fifty-eight percent of endoscopically treated patients required one endoscopic resection, while 58 (42 %) required two or more endoscopic resections (range 2–8). Patients who underwent operative resection had larger tumors with a mean size of 3.7?±?2.8 versus 1.8?±?1.5 cm in those treated by endoscopic resection (p?p?=?0.02). Intraductal extension and ulceration had no effect on recurrence if completely resected endoscopically (p?=?0.41 and p?=?0.98, respectively). Postoperative complications occurred in 58 % of patients, and post-endoscopic complications in 29 % (p?p?=?0.006); 4 % of recurrences had invasive carcinomas. When comparing patients who underwent local resections only (endoscopic and operative), there was no difference in the recurrence rate between endoscopic resection and operative transduodenal resection (32 versus 33 %; p?=?0.49). The need for two or more endoscopic resections for complete tumor removal was associated with 13-fold greater risk of recurrence (p?Conclusion There is no significant difference between endoscopic and local operative resections of benign adenomas of ampulla of Vater; recurrences are more common when two or more endoscopic resections are required for complete tumor removal. Appropriate adenomas for endoscopic resection included tumors <3.6 cm that do not extend far enough intraductally (on EUS) to preclude an endoscopic snare ampullectomy.     

Lymph Node Involvement and Not the Histophatologic Subtype Is Correlated with Outcome After Resection of Adenocarcinoma of the Ampulla of Vater

Background

Intestinal and pancreaticobiliary types of Vater’s ampulla adenocarcinoma have been considered as having different biologic behavior and prognosis. The aim of the present study was to determine the best immunohistochemical panel for tumor classification and to analyze the survival of patients having these histological types of adenocarcinoma.

Method

Ninety-seven resected ampullary adenocarcinomas were histologically classified, and the prognosis factors were analyzed. The expression of MUC1, MUC2, MUC5AC, MUC6, CK7, CK17, CK20, CD10, and CDX2 was evaluated by using immunohistochemistry.

Results

Forty-three Vater’s ampulla carcinomas were histologically classified as intestinal type, 47 as pancreaticobiliary, and seven as other types. The intestinal type had a significantly higher expression of MUC2 (74.4% vs. 23.4%), CK20 (76.7% vs. 29.8%), CDX2 (86% vs. 21.3%), and CD10 (81.4% vs. 51.1%), while MUC1 (53.5% vs. 82.9%) and CK7 (79.1% vs. 95.7%) were higher in pancreatobiliary adenocarcinomas. The most accurate markers for immunohistochemical classification were CDX2, MUC1, and MUC2. Survival was significantly affected by pancreaticobiliary type (p?=?0.021), but only lymph node metastasis, lymphatic invasion, and stage were independent risk factors for survival in a multivariate analysis.

Conclusion

The immunohistochemical expression of CDX2, MUC1, and MUC2 allows a reproducible classification of ampullary carcinomas. Although carcinomas of the intestinal type showed better survival in the univariate analysis, neither histological classification nor immunohistochemistry were independent predictors of poor prognosis.     

Clinical Outcomes for Neuroendocrine Tumors of the Duodenum and Ampulla of Vater:A Population-Based Study

Background

Previous case series report that neuroendocrine tumors (NETs) of the ampulla of Vater have worse overall survival (OS) than NETs in the duodenum. We aimed to compare the OS of patients with ampullary NETs to patients with duodenal NETs.

Methods

This retrospective comparative cohort study used the Surveillance, Epidemiology, and End Results (SEER) registry from 1988 to 2009. OS was evaluated using Kaplan–Meier estimates and Cox proportional hazard regression.

Results

Ampullary NETs (n?=?120) were larger (median size 18 vs. 10 mm, p?<?0.001), higher grade (poorly and undifferentiated tumor 42 % vs. 12 %, p?<?0.001), higher SEER historic stage (distant metastasis 16 % vs. 7 %, p?<?0.001), and more often resected (78 % vs. 60 %, p?<?0.001) than duodenal NETs (n?=?1,360). Median OS was significantly worse for patients with ampullary NETs than with duodenal NETs (98 vs. 143 months, p?=?0.037). Local resection was performed for 50.5 % of the resected ampullary NETs and resulted in similar OS compared to locally resected duodenal NETs (HR 1.37, 95 % CI 0.76–2.48, p?=?0.291).

Conclusions

While ampullary NETs are more advanced at presentation and have worse OS than duodenal NETs, long-term survival is possible with proximal small bowel NETs. For locally resected NETs, OS is similar between ampullary and duodenal NETs.     

Preoperative Cholangitis and Metastatic Lymph Node Have a Negative Impact on Survival After Resection of Extrahepatic Bile Duct Cancer

Background

The significance of the presence of preoperative inflammation for the prognosis of patients with extrahepatic bile duct cancer (BDCA) was evaluated.

Methods

The clinical data of 84 patients who underwent surgery for BDCA from August 2003 to May 2009 were reviewed, and survival analysis was performed. The patients were classified into two groups according to the presence of preoperative cholangitis: Group A had no cholangitis (n?=?59), and group B had cholangitis (n?=?25).

Results

There were no differences in sex, mean age, TNM stage, biliary drainage, type of resection, or radicality between the two groups (p?>?0.05). The 3-year disease-specific survival (DSS) and disease-free survival (DFS) rates for the group B patients (21.5 and 11.9?%, respectively) were significantly lower than those for the group A patients (66.1 and 57.3?%, respectively; p?=?0.013 and 0.001, respectively). The multivariate analysis showed that preoperative inflammation and lymph node metastasis were the independent prognostic factors for both overall survival (OS) [p?=?0.021, relative risk (RR)?=?2.224 and p?=?0.015, RR?=?2.367, respectively] and DFS (p?=?0.014; RR?=?2.192 and p?=?0.013; RR?=?2.240, respectively). The rates of angiolymphatic and perineural invasion were higher for group B than those for group A (p?=?0.016 and 0.030, respectively).

Conclusions

The presence of preoperative inflammation is an independent poor prognostic factor for OS and DFS for patients with BDCA.     

Clinical Outcome of IBD-Associated versus Sporadic Colorectal Cancer: A Matched-Pair Analysis

Purpose

This study assesses the perioperative course and long-term survival of inflammatory bowel disease (IBD)-associated vs. sporadic colorectal cancer (IBD-CRC vs. SCRC) after elimination of known confounders.

Methods

Between 1991 and 2007, n?=?3,299 patients underwent surgery for CRC at our institution. Thirty-three IBD patients were identified and compared to 165 SCRC using a matched-pair analysis (1:5 scenario). As matching parameters were used: age, gender, Union Internationale Contre le Cancer (UICC) stage, site of primary lesion, and date of surgery. After univariate analysis of the perioperative course, a multivariate survival analysis (Cox) of all patients (n?=?198) was performed.

Results

Significant differences were shown for preoperative symptoms (p?=?0.022), transfusion rate (p?=?0.01), ileostomy construction rate (p?=?0.001), total complication rate (p?=?0.042), and hospital stay (15 vs. 11 days, p?<?0.001). Local tumor recurrence was three times higher in IBD-CRC (p?=?0.004), and the 5-year survival rate was lower (49 % vs. 67 %, p?=?0.03). IBD, advanced UICC stage, and synchronous liver metastasis were identified as independent prognostic factors.

Conclusion

We demonstrate for the first time survival differences between IBD-CRC and SCRC after elimination of five known confounders. This might be caused by a difference in tumor biology resulting in a higher local recurrence rate in IBD-CRC.     

Surgical Outcomes and Clinical Characteristics of Elderly Patients Undergoing Curative Hepatectomy for Hepatocellular Carcinoma

Background

The aim of this study was to investigate the clinical characteristics and outcomes of elderly patients (≥70 years old) undergoing curative hepatectomy for hepatocellular carcinoma (HCC).

Methods

Clinicopathological data and treatment outcomes in 100 elderly patients (≥70 years old) and 120 control patients (≤70 years old) with HCC who underwent curative hepatectomy between 2000 and 2011 were retrospectively collected and compared.

Results

The overall survival rate was similar between the two groups, but the disease-free survival rate was worse in the elderly group when compared with the control group. Prognostic factors for overall and disease-free survival were the same when comparing the two groups. The elderly group had higher rate of females (p?=?0.0230), higher hepatitis C virus infection rate (p?=?0.0090), higher postoperative pulmonary complication rate (p?=?0.0484), lower rate of response to interferon (IFN) therapy (p?=?0.0203) and shorter surgical time (p?=?0.0337) when compared with the control group. The overall recurrence rate was higher in the elderly group than in the control group (p?=?0.0346), but the rate of recurrence within 2 years after the operation was similar when comparing the two groups.

Conclusion

The survival of elderly patients with HCC was similar to that of younger patients. However, the disease-free survival was worse in elderly patients than in younger patients. Aggressive antiviral therapy (e.g. IFN therapy) may be necessary to improve the disease-free survival, even in elderly patients. Additionally, clinicians should be aware of the risk of pulmonary complications in elderly patients after hepatectomy.     

Patients with Multiple Hepatocellular Carcinomas Within the UCSF Criteria Have Outcomes After Curative Resection Similar to Patients Within the BCLC Early-Stage Criteria

Background

Surgical strategies for the treatment of multiple hepatocellular carcinomas (HCC) remain controversial. This study compared the prognostic power of the University of California, San Francisco (UCSF) criteria with the Barcelona Clinic Liver Cancer (BCLC) early-stage criteria.

Methods

Clinical and survival data of 162 multiple-HCC patients in Child-Pugh class A who underwent curative resection were retrospectively reviewed. Prognostic risk factors were analyzed using univariate and multivariate analyses.

Results

UCSF criteria were shown to independently predict overall and disease-free survival. In patients within the UCSF criteria, 3-year overall and disease-free survivals were significantly better than in those exceeding the UCSF criteria (68 vs. 34?% and 54 vs. 26?%, respectively; both p?p?=?0.506 and 57 vs. 50?%, p?=?0.666, respectively). Tumors within the UCSF criteria were associated with a lower incidence of high-grade tumor (p?=?0.009), microvascular invasion (p?=?0.005), 3-month death (p?=?0.046), prolonged Pringle’s maneuver (p?=?0.005), and surgical margin <0.5?cm (p?Conclusions Multiple HCC patients within the UCSF criteria benefit from curative resection. Expansion of curative treatment is justified.     

Selective Reoperation for Locally Recurrent or Metastatic Pancreatic Ductal Adenocarcinoma Following Primary Pancreatic Resection

Background

Resection of certain recurrent malignancies can prolong survival, but resection of recurrent pancreatic ductal adenocarcinoma is typically contraindicated because of poor outcomes.

Methods

All patients from 1992 to 2010 with recurrent pancreatic cancer after intended surgical cure were retrospectively evaluated. Clinicopathologic features were compared from patients who did and did not undergo subsequent reoperation with curative intent to identify factors associated with prolonged survival.

Results

Twenty-one of 426 patients (5?%) with recurrent pancreatic cancer underwent potentially curative reoperation for solitary local-regional (n?=?7) or distant (n?=?14) recurrence. The median disease-free interval after initial resection among reoperative patients was longer for those with lung or local-regional recurrence (52.4 and 41.1?months, respectively) than for those with liver recurrence (7.6?months, p?=?0.006). The median interval between reoperation and second recurrence was longer in patients with lung recurrence (median not reached) than with liver or local-regional recurrence (6 and 9?months, respectively, p?=?0.023). Reoperative patients with an initial disease-free interval >20?months had a longer median survival than those who did not (92.3 versus 31.3?months, respectively; p?=?0.033).

Conclusion

Patients with a solitary pulmonary recurrence of pancreatic cancer after a prolonged disease-free interval should be considered for reoperation, as they are more likely to benefit from resection versus other sites of solitary recurrence.     

Clinical features and surgical outcomes of primary cauda equina tumours

Background

To identify clinical features, radiological findings and surgical outcomes of primary cauda equina tumours.

Methods

A consecutive series of 64 operations in 60 patients with primary cauda equina tumours from April 1999 to May 2009 at one institution comprised the study. The cases were divided into tumours of neural sheath origin (TNS, n?=?48) and tumours of non-neural sheath origin (TNNS, n?=?22). We analysed pain intensity, neurological abnormalities, MRI findings, surgical extent and functional outcome.

Results

The TNS group showed more leg pain (76 % vs. 44 %, p?=?0.019) with higher intensity (6.1?±?1.5 vs. 4.6?±?1.9, p?=?0.04). Motor weakness and bladder dysfunction were more common in the TNNS group (p?=?0.028 and p?=?0.00 in each). Flow voids of MRI were more frequently observed in TNNS (50 % vs. 4 %, p?=?0.01). The TNS group achieved total removal in all operations compared with total removal in 77 % in the TNNS group (p?=?0.001). The TNNS group showed higher recurrence rates (18 % vs. 0 %, p?=?0.009). The TNS group showed higher improvement of JOA scores postoperatively (p?=?0.049). Surgical complications were observed less frequently in the TNS group (19 % vs. 78 %, p?=?0.000).

Conclusions

TNS differs from TNNS by causing more frequent leg pain, higher pain intensity and more frequent flow voids. TNS has better surgical outcomes than TNNS in terms of higher rates of total removal, fewer surgical complications, better functional outcomes and less recurrence.     

Ezrin Expression Is an Independent Prognostic Factor in Gastro-intestinal Cancers

Background

Ezrin, a member of the ezrin–radixin–moesin (ERM) family of plasma membrane–cytoskeleton linker proteins, has been associated with metastatic behavior.

Methodology

Microarrayed pathological tissues of surgically resected colorectal cancer liver metastasis (CRLM) and whole tissue sections of cancer of the ampulla of Vater (CAV) were analyzed to determine ezrin expression levels and correlation with survival. The requirement of ezrin in invasive capability was assessed using in vitro assays.

Results

Surgically resected CAV showing a low ezrin score have a better 5-year disease-specific survival than those showing a high ezrin score (P?<?0.0001). Similarly, high ezrin expression at the invasive front of CRLM resulted in poor disease-free survival (P?=?0.05). Multivariate analysis demonstrated high ezrin expression to be an independent adverse prognostic factor for CAV (hazard ratio (HR) 15.22 (95 % confidence interval (CI) 1.98–117.03), P?<?0.01) and CRLM (HR 6.42 (95 % CI 1.01–52.43), P?=?0.05), among other clinically relevant variables such as lymph node metastasis (for CAV) and the presence of extrahepatic disease, large hepatic metastases (>5 cm), and close surgical resection margins (<5 mm) (all for CRLM). In vitro experiments indicated that ezrin expression was vital for cellular processes such as adhesive and invasive activity.

Significance

High ezrin expression indicates an adverse prognosis in primary CAV and CRLM.     

Carcinoma of the Ampulla of Vater: Patterns of Failure Following Resection and Benefit of Chemoradiotherapy

Background

Ampullary carcinoma is a rare malignancy. Despite radical resection, survival rates remain low with high rates of local failure. We performed a single-institution outcomes analysis to define the role of concurrent chemoradiotherapy (CRT) in addition to surgery.

Methods

A retrospective analysis was performed of all patients undergoing potentially curative pancreaticoduodenectomy for adenocarcinoma of the ampulla of Vater at Duke University Hospitals between 1976 and 2009. Time-to-event analysis was performed comparing all patients who underwent surgery alone to the cohort of patients receiving CRT in addition to surgery. Local control (LC), disease-free survival (DFS), overall survival (OS), and metastases-free survival (MFS) were estimated using the Kaplan?CMeier method.

Results

A total of 137 patients with ampullary carcinoma underwent Whipple procedure. Of these, 61 patients undergoing resection received adjuvant (n?=?43) or neoadjuvant (n?=?18) CRT. Patients receiving chemoradiotherapy were more likely to have poorly differentiated tumors (P?=?.03). Of 18 patients receiving neoadjuvant therapy, 67% were downstaged on final pathology with 28% achieving pathologic complete response (pCR). With a median follow-up of 8.8?years, 3-year local control was improved in patients receiving CRT (88% vs 55%, P?=?.001) with trend toward 3-year DFS (66% vs 48%, P?=?.09) and OS (62% vs 46%, P?=?.074) benefit in patients receiving CRT.

Conclusions

Long-term survival rates are low and local failure rates high following radical resection alone. Given patterns of relapse with surgery alone and local control benefit in patients receiving CRT, the use of chemoradiotherapy in selected patients should be considered.     

Peri-Operative Bowel Perforation in Early Stage Colon Cancer is Associated with an Adverse Oncological Outcome

Background

The presence of an inflammatory response resulting from bowel perforation or anastomotic leakage has been suggested to enhance recurrence rates in colorectal cancer patients. Currently, it is unknown if bowel perforation or anastomotic leakage has prognostic significance in early stage colon cancer patients. In this study, the impact of peri-operative bowel perforation including anastomotic leakage on disease-free survival of stage I/II colon cancer patients was investigated.

Methods

Prospective follow up data of 448 patients with stages I/II colon cancer that underwent resection were included. Patients who died within 3?months after initial surgery were excluded.

Results

Median follow up was 56.0?months. Patients with peri-operative bowel perforation (n?=?25) had a higher recurrence rate compared to patients without perforation (n?=?423), 36.0?% vs. 16.1?% (p?=?0.01). Disease-free survival was significantly worse for the perforation group compared to patients without perforation (p?=?0.004). Multivariate analysis including T-stage, histological grade, and adjuvant chemotherapy showed peri-operative bowel perforation to be an independent factor significantly associated with disease recurrence (odds ratio, 2.7; 95?% CI, 1.1?C6.7).

Conclusion

Peri-operative bowel perforation is associated with increased recurrence rates and impaired disease-free survival in early-stage colon cancer patients.     

Resection for Hilar Cholangiocarcinoma: Analysis of Prognostic Factors and the Impact of Systemic Inflammation on Long-term Outcome

Introduction

Resection for hilar cholangiocarcinoma is the single hope for long-term survival.

Methods

Ninety patients underwent curative intent surgery for hilar cholangiocarcinoma between 1996 and 2012. The potential prognostic factors were assessed by univariate (Kaplan–Meier curves and log-rank test) and multivariate analyses (Cox proportional hazards model).

Results

The median overall and disease-free survivals were 26 and 17 months, respectively. The multivariate analysis identified R0 resection (HR?=?0.03, 95 % CI 0–0.19, p?<?0.001), caudate lobe invasion (HR?=?6.33, 95 % CI 1.31–30.46, p?=?0.021), adjuvant gemcitabine-based chemotherapy (HR?=?0.38, 95 % CI 0.15–0.94, p?=?0.037), and the neutrophil-to-lymphocyte ratio (HR?=?0.78, 95 % CI 0.62–0.98, p?=?0.036) as independent prognostic factors for disease-free survival. The independent prognostic factors for overall survival were R0 resection (HR?=?0.03, 95 % CI 0–0.22, p?<?0.001), caudate lobe invasion (HR?=?11.75, 95 % CI 1.65–83.33, p?=?0.014), and adjuvant gemcitabine-based chemotherapy (HR?=?0.19, 95 % CI 0.06–0.56, p?=?0.003).

Conclusions

The negative resection margin represents the most important prognostic factor. Adjuvant gemcitabine-based chemotherapy appears to benefit survival. The neutrophil-to-lymphocyte ratio may potentially be used to stratify patients for future clinical trials.     

Definition of T3/4 and Regional Lymph Nodes in Gallbladder Cancer: Which Is More Valid, the UICC or the Japanese Staging System?

Background

The Union for International Cancer Control (UICC) and Japanese Society of Biliary Surgery (JSBS) staging systems differ in their staging of gallbladder cancer: they define hepatic invasion with or without invasion of another organ as T3 and either T3 or T4, respectively, and posterosuperior pancreatic lymph node (PSPLN) metastases as M1 and N2, respectively.

Methods

We retrospectively evaluated the survival of 224 patients who had undergone macroscopically curative resection for gallbladder cancer and assessed the influence of the differences between the two staging systems on survival.

Results

JSBS staging stratified the survival curves better for stages III or IV. Fifty-seven patients were classified as UICC-T3 but JSBS-T4. These patients had better survival than did 43 patients with UICC-T4/JSBS-T4 and comparable survival to 17 patients with UICC-T3/JSBS-T3. UICC stage IIIB is composed of two subgroups: U-T2N1 (18 patients) and U-T3N1 (21 patients). Their 5-year survivals were 85 and 41?%, respectively (P?=?0.01). The latter was comparable to that of 28 T3N0 patients (35?%, P?=?0.93). The survival of the UICC-M1 patients with disease restricted to PSPLNs was significantly better than that of those with involvement beyond PSPLNs (5-year survival 35 vs. 17?%; P?=?0.04).

Conclusions

Although UICC staging more accurately defines the T category, JSBS staging better stratifies the prognosis of patients with gallbladder cancer, mainly because UICC stage IIIB includes T1/2N1M0, which is associated with significantly better survival than T3N0M0. It would be appropriate to classify PSPLNs as regional lymph nodes.     

Prognostic impact of lymph node involvement and the extent of lymphadenectomy (LAD) in adenocarcinoma of the esophagogastric junction (AEG)

Background

The prognostic importance of lymph node (LN) involvement for patients with adenocarcinoma of the esophagogastric junction (AEG) is well-known. In the latest edition of the UICC staging system, the number of metastatic LNs was taken into account, while the extent of lymphadenectomy (LAD) remains unaddressed. Removal of at least six LNs is recommended, but there is no defined minimum number as to classify as (y)pN0. We examined the prognostic value of the number of positive LNs, number of LNs removed, and LN ratio (LNR) in order to determine the influence of an adequate LAD on overall survival (OS).

Methods

We analyzed data of 316 patients with AEG treated in our institution (2001–2011) regarding clinicopathological data, treatment, morbidity, mortality, and long-term prognosis. Univariate and multivariate analysis was performed using Cox regression to evaluate the prognostic impact of(y)pN category, number of LNs removed and LNR.

Results

OS decreased with higher count of positive LNs (p?<?0.001) and higher LNR (p?<?0.001). Whether >6, >15, or >30 LNs were removed did not influence OS, neither in the entire study population nor within individual (y)pT or (y)pN categories. Multivariate analysis revealed LNR (p?<?0.001) besides M category (p?=?0.015) and tracheotomy during the postoperative course (p?=?0.005) as independent predictors of OS.

Conclusion

The classification according to the number of involved LNs in the latest edition of the UICC staging system improves prognostication in patients with AEG. The importance of an adequate LAD is shown by the high prognostic relevance of the LNR rather than the absolute number of LNs removed.     

Combined resection of colorectal hepatic–pulmonary metastases shows improved outcome over chemotherapy alone

Background

The objective of this retrospective study was to assess the survival of patients after resection of hepatic and pulmonary colorectal metastases to identify predictors of long-term survival.

Methods

Patients receiving chemotherapy alone were compared to patients receiving surgery and chemotherapy in a matched-pair analysis with the following criteria: UICC stage, grading, and date of initial primary tumor occurrence.

Results

A total of 30 patients with liver and lung metastases of colorectal carcinoma underwent resection. In 20 cases, complete resection was achieved (median survival, 67 months). Incomplete resection and preoperatively elevated carcinoembryonic antigen (CEA) levels are independent risk factors for reduced survival. Patients developing pulmonary metastases prior to hepatic metastases had the worst prognosis. Surgical resection significantly increased survival compared to chemotherapy alone in matched-pair analysis (65 vs. 30 months, p?=?0.03).

Conclusions

Incomplete resection and elevated CEA levels are predictors of poor outcome. Matched-paired analysis confirmed that surgical resection in combination with chemotherapy appears to be superior to chemotherapy alone.     

Clinical Experience with Primary Hepatic Epithelioid Hemangioendothelioma: Retrospective Study of 33 Patients

Background

This multicenter-based retrospective study aimed to investigate the prognostic factors and report our experiences with the diagnosis and treatment of hepatic epithelioid hemangioendothelioma (HEHE), a rare malignant vascular tumor.

Methods

A total of 33 patients with HEHE from two centers between 2004 and 2011 were retrospectively reviewed with respect to their clinical, radiologic, and pathologic characteristics; treatment modalities and outcomes; and potential prognostic factors.

Results

A total of 17 patients underwent liver resections (LRs) alone, 12 patients had transcatheter arterial chemoembolization (TACE) alone, three patients had LR followed by TACE, and one patient underwent liver transplantation (LT). The difference of overall survival (OS) between LR and TACE was not significant (p?=?0.499). Older patients [≥47?years, n?=?17; p?=?0.035, hazard ratio (HR)?=?7.0), those with symptoms (n?=?17; p?=?0.001, HR?=?86.5], and those with an elevated serum CA19-9 level (>37?U/ml, n?=?5; p?=?0.018, HR?=?5.0) had a poorer OS, according to univariate analysis. The presence of symptoms was validated as a prognostic factor (p?=?0.012) by multivariate analysis.

Conclusions

Liver resection and TACE have comparable outcomes in HEHE patients. The presence of symptoms indicates a poor prognosis. Older age and elevated serum CA19-9 are potential negative impact factors on outcome.     

Increasing Tumor Thickness is Associated with Recurrence and Poorer Survival in Patients with Merkel Cell Carcinoma

Background

Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous neuroendocrine tumor usually occurring on sun-exposed skin in elderly patients. Clinical and pathologic factors associated with disease progression and mortality in patients with MCC are poorly defined. Recently, it has been reported that p63 expression in primary MCC is strongly associated with clinical outcome.

Methods

MCC patients diagnosed between July 1, 1993 and July 31, 2009 were identified from the surgical pathology records of the Sydney South West Area Health Service. Clinical, pathologic, treatment, and survival data were obtained and immunohistochemical analyses for p53, p63, and Ki-67 were performed. The associations of clinical and pathologic features with disease-free and disease-specific survival were analyzed.

Results

Ninety-five patients were identified (67 males, 28 females; median age at diagnosis of primary MCC 76 [range, 42?C93] years). Increasing primary tumor thickness was significantly associated with poorer disease-free survival (5-year survival 18?% in tumors >10?mm thick compared with 69?% for patients with tumors ??10?mm thick, p?=?0.002) and disease-specific survival (5-year survival 74?% in tumors >10?mm thick compared with 97?% for patients with tumors ??10 mm thick, p?=?0.006). There was a strong positive correlation between the Ki-67 index (proportion of Ki-67-positive tumor nuclei) and tumor thickness (r?=?0.39, n?=?45, p?=?0.008). Positive staining for p63 in MCC was infrequent (9?% of primary MCC) and showed no significant association with disease outcome.

Conclusions

Tumor thickness is significantly associated with disease-free survival in MCC. We recommend that primary tumor thickness be routinely recorded in the pathology reports of patients with primary MCC.