Surgical management of large, connected perineal and pelvic epidermal inclusion cysts mimicking a dumbbell-shaped lesion in an adult male.

INTRODUCTION AND IMPORTANCE: Epidermal inclusion cysts are a common benign finding, and they are predominantly asymptomatic. They can rarely form in the pelvis or abdomen, however, and may cause symptoms secondary to mass effect. This case highlights management of an anterectal epidermal inclusion cyst connected to the perineal cyst, mimicking a dumbbell-shaped lesion, found in a male. CASE PRESENTATION: This is a unique case of a 21-year-old Caucasian male with a palpable perineal mass, lower extremity hypoesthesia, and constipation who was found to have a complex-shaped cyst on computed tomography and magnetic resonance imaging. This was ultimately managed with a two-stage perineal and transabdominal resection. CLINICAL DISCUSSION: This case highlights that perineal epidermal inclusion cysts may have pelvic extension, especially in patients with additional new-onset neurologic, gastrointestinal, or urologic symptoms. These symptoms should completely resolve after resection. Additionally, resection is recommended to prevent complications including malignant degeneration and fistulization. CONCLUSION: This is the first reported case of an anterectal, epidermal inclusion cyst connected to a perineal cyst found in a male. Perineal and pelvic cysts may be synchronous and may be connected through the pudendal canal. These masses can be safely removed via a combined perineal and transabdominal resection. The connecting portion of lesions that have both pelvic and perineal components should be meticulously identified and dissected because even a thin, patent segment - if left unresected - may result in lesion recurrence.

Management of Rectal Cancer.

Rectal cancer is often presented with a dizzying array of treatment recommendations. This article clarifies and simplifies this common clinical problem from the surgical perspective. Treatment of rectal cancer requires an understanding of presenting stage (early or advanced) and location (high or low) to provide oncologic sound treatment decisions. Surgical treatment requires a minimum of 1 cm distal margin, careful clearance of the mesorectum and radial margin using total mesorectal excision technique, and 12 or more regional lymph nodes harvested and analyzed. Appropriate and effective multimodality treatments exist and must be used based on sound guidelines as outlined.

Neoadjuvant Therapy for Rectal Cancer Affects Lymph Node Yield and Status Without Clear Implications on Outcome: The Case for Eliminating a Metric and Using Preoperative Staging to Guide Therapy.

BACKGROUND: Nodal status has long been considered pivotal to oncologic care, staging, and management. This has resulted in the establishment of rudimentary metrics regarding adequate lymph node yield in colon and rectal cancers for accurate cancer staging. In the era of neoadjuvant treatment, the implications of lymph node yield and status on patient outcomes remains unclear. PATIENT AND METHODS: This study included 1,680 patients with locally advanced rectal cancer from the NCCN prospective oncology database stratified into 3 groups based on preoperative therapy received: no neoadjuvant therapy, neoadjuvant chemoradiation, and neoadjuvant chemotherapy. Clinicopathologic characteristics and survival were compared between the groups, with univariate and multivariate analyses undertaken. RESULTS: The clinicopathologic characteristics demonstrated statistically significant differences and heterogeneity among the 3 groups. The neoadjuvant chemoradiation group demonstrated the statistically lowest median lymph node yield (n=15) compared with 17 and 18 for no-neoadjuvant and neoadjuvant chemotherapy, respectively (P<.0001). Neoadjuvant treatment did impact survival, with chemoradiation demonstrating increased median overall survival of 42.7 compared with 37.3 and 26.6 months for neoadjuvant chemotherapy and no-neoadjuvant therapy, respectively (P<.0001). Patients with a yield of fewer than 12 lymph nodes had improved median overall survival of 43.3 months compared with 36.6 months in patients with 12 or more lymph nodes (P=.009). Multivariate analysis demonstrated that neither node yield nor status were predictors for overall survival. DISCUSSION: This analysis reiterates that nodal yield in rectal cancer is multifactorial, with neoadjuvant therapy being a significant factor. Node yield and status were not significant predictors of overall survival. A nodal metric may not be clinically relevant in the era of neoadjuvant therapy, and guidelines for perioperative therapy may need reconsideration.

Pancreatic cancer metastatic to a limited number of lymph nodes has no impact on outcome.

BACKGROUND: The purpose of this study was to determine the association of the extent of metastatic lymph node involvement with survival in pancreatic cancer. METHODS: This is a retrospective review of a prospectively maintained database of patients who underwent resection for pancreatic adenocarcinoma, 1999-2011. RESULTS: 165 patients were identified and divided into 3 groups based on the number of positive lymph nodes - 0 (group A), 1-2 (B), >3 (C). Each group had 55 patients. Those in group C were more likely to have a higher T stage, poorly differentiated grade, lymphovascular invasion (LVI), higher mean intraoperative blood loss, positive margins, tumor location involving the uncinate process, and a higher likelihood of undergoing a pancreaticoduodenectomy. Median overall survival (OS) for group A, B and C was 25.5 months (mo), 21 mo and 12.3 mo, respectively (p < 0.001). No survival difference was noted for survival between groups A and B (p = 0.86). The ratio of involved lymph nodes <0.2 was predictive of improved survival (p < 0.001). CONCLUSIONS: Resected pancreatic cancer patients with only 1-2 positive lymph nodes or less than 20% involvement have a similar prognosis to patients without nodal disease. Current staging should consider stratification based on the extent of nodal involvement.

Duodenal Adenocarcinoma: Profile and Predictors of Survival Outcomes.

Duodenal adenocarcinoma is rare. We aimed to evaluate survival outcome and prognostic factors for survival in patients with duodenal adenocarcinoma in recent years, marked by advancement in chemotherapy for gastrointestinal cancers. All patients treated for duodenal adenocarcinoma at our institution between January 2000 and July 2013 were reviewed. Thirty-nine patients were identified: 27 operative patients [21(53.8%) curative and 6 (15.4%) palliative operations] and 12 nonoperative patients [primary systemic chemotherapy, 4 (10.3%), palliative radiotherapy, 1 (2.6%), and no treatment, 7 (17.9%)]. Curative resections included 13 pancreaticoduodenectomies and eight segmental resections. Median overall survival (OS) for entire cohort was 14.4 months. Median OS and one-, three-, and five-year OS were operative group (41.4 months; 79.1%, 50.6%, and 10.6%, respectively); nonoperative group (7.4 months; 25.0%, 8.3%, and 0%, respectively); curative surgery (45.4 months; 92.9%, 62.5%, and 16.7%, respectively) and palliative surgery (5.4 months; 33.3%, 16.7%, and 0%, respectively). Female gender (P = 0.04), curative resection (P = 0.03), nodal metastasis (P = 0.047) and advanced T stage (P = 0.047) were predictive of OS. Two factors were independently predictive of OS--female gender and curative resection. Overall survival still hinges on curative resection. This favors early detection. Adjuvant treatment modalities such as chemotherapy and radiation require further investigation.

Surgical Management of Bile Duct Strictures.

Biliary strictures can arise from either benign or malignant diseases. Both are amenable to surgical treatment if the surgeon has a clear understanding of the inciting patho-physiology and appropriate training and skill. This review article focuses on the key aspects of surgical management of biliary strictures. The decision to perform a biliary bypass or radical resection of a biliary stricture depends upon the pathology (benign or malignant) and whether there is curative or palliative intent. Endoscopic findings and brushings can often be non-diagnostic and clinical judgment is required. Final pathology ranges from a delayed stricture years following cholecystectomy to cholangiocarcinoma. Performing the correct operation safely requires clinical experience and knowledge of multiple surgical approaches. Surgical options must maximize cure when possible and relieve biliary obstructive and infectious complications.

Incidence of minimally invasive colorectal cancer surgery at National Comprehensive Cancer Network centers.

BACKGROUND: Laparoscopic colectomy has been shown to have equivalent oncologic outcomes to open colectomy for the management of colon cancer, but its adoption nationally has been slow. This study investigates the prevalence and factors associated with laparoscopic colorectal resection at National Comprehensive Cancer Network (NCCN) centers. METHODS: Data on patients undergoing surgery for colon and rectal cancer at NCCN centers from 2005 to 2010 were obtained from chart review of medical records for the NCCN Outcomes Project and included information on socioeconomic status, insurance coverage, comorbidity, and physician-reported Eastern Cooperative Oncology Group (ECOG) performance status. Associations between receipt of minimally invasive surgery and patient and clinical variables were analyzed with univariate and multivariable logistic regression. All statistical tests were two-sided. RESULTS: A total of 4032 patients, diagnosed between September 2005 and December 2010, underwent elective colon or rectal resection for cancer at NCCN centers. Median age of colon cancer patients was 62.6 years, and 49% were men. The percent of colon cancer patients treated with minimally invasive surgery (MIS) increased from 35% in 2006 to 51% in 2010 across all centers but varied statistically significantly between centers. On multivariable analysis, factors associated with minimally invasive surgery for colon cancer patients who had surgery at an NCCN institution were older age (P = .02), male sex (P = .006), fewer comorbidities (P ≤ .001), lower final T-stage (P < .001), median household income greater than or equal to $80000 (P < .001), ECOG performance status = 0 (P = .02), and NCCN institution (P ≤ .001). CONCLUSIONS: The use of MIS increased at NCCN centers. However, there was statistically significant variation in adoption of MIS technique among centers.

Systemic therapy for advanced appendiceal adenocarcinoma: an analysis from the NCCN Oncology Outcomes Database for colorectal cancer.

Appendiceal malignancies are rare and represent 1% of intestinal tumors in the United States. The role and efficacy of modern systemic therapy in advanced appendiceal adenocarcinoma has not been established. This study analyzed patients with recurrent or metastatic appendiceal adenocarcinoma in the database for Colorectal Cancer (CRC; 2005-2012). This database tracks longitudinal care for patients treated at 8 specialty centers across the Unites States. Study objectives were to describe and evaluate the efficacy of systemic therapy and investigate relationships with clinicopathologic features. Cox regression analysis was performed to identify predictors of progression-free survival (PFS) and overall survival (OS). Of 248 patients with advanced appendiceal carcinoma, 112 (45%) received systemic therapy for measurable disease and are the focus of this report. The most common chemotherapy regimens included FOLFOX with or without bevacizumab (n=39 and n=37, respectively), FOLFIRI (n=15), and single-agent fluoro-pyrimidine (n=10). Among 99 patients evaluable for best response, 39 experienced a response (response rate [RR], 39%) and 36 (36%) had stable disease. The median PFS was 1.2 years (95% CI, 1.0-1.8) and median OS was 2.1 years (95% CI, 1.6-2.3). Patients with non-mucinous histology or high-grade tumors and those who underwent nondebulking surgery had worse PFS and OS. Treatment of advanced appendiceal adenocarcinoma at NCCN Member Institutions commonly incorporates agents used for CRC. RR, PFS, and OS are comparable to those achieved in the treatment of metastatic CRC. Poor prognostic factors include nonmucinous histology or high-grade tumors and history of nondebulking surgery.

Morbidity and mortality associated with gastrectomy for gastric cancer.

BACKGROUND: Surgery alone is often inadequate for advanced-stage gastric cancer. Surgical complications may delay adjuvant therapy. Understanding these complications is needed for multidisciplinary planning. MATERIAL AND METHODS: The American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) database was queried for patients who underwent gastrectomy for malignancy (ICD-9 code 151.x) from 2005 to 2010. Thirty-day mortality and morbidity were evaluated. RESULTS: Overall, 2,580 patients underwent gastrectomy for malignancy, divided as total gastrectomy 999 (38.7 %) and partial gastrectomy 1,581 (61.3 %). Overall, serious morbidity occurred in 23.6 %, and the 30-day mortality was 4.1 %. Patients receiving a total gastrectomy were younger and healthier than those receiving a partial gastrectomy for the following measured criteria: age, diabetes, chronic obstructive pulmonary disease and hypertension. Serious morbidity and mortality were significantly higher in the total gastrectomy group than the partial gastrectomy group (29.3 vs. 19.9 %, p < 0.001; and 5.4 vs. 3.4 %, p < 0.015, respectively). The inclusion of additional procedures increased the risk of mortality for the following: splenectomy (odds ratio [OR] 2.8; p < 0.001), pancreatectomy (OR 3.5; p = 0.001), colectomy (OR 3.6; p < 0.001), enterectomy (OR 2.7; p = 0.030), esophagectomy (OR 3.5; p = 0.035). Abdominal lymphadenectomy was not associated with increased morbidity (OR 1.1; p = 0.41); rather, it was associated with decreased mortality (OR 0.468; p = 0.028). CONCLUSIONS: Gastrectomy for cancer as currently practiced carries significant morbidity and mortality. Inclusion of additional major procedures increases these risks. The addition of lymphadenectomy was not associated with increased morbidity or mortality. Strategies are needed to optimize surgical outcomes to ensure delivery of multimodality therapy for advanced-stage disease.

Neoadjuvant radiotherapy use in locally advanced rectal cancer at NCCN member institutions.

Based on randomized data, neoadjuvant chemoradiotherapy has been incorporated into the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for stage II-III rectal cancer. Factors associated with nonadherence to evidence-based guidelines for neoadjuvant radiotherapy (RT) were examined at dedicated cancer centers. The prospective NCCN Oncology Outcomes Database for Colorectal Cancers was queried for patients with stage II-III rectal cancer who underwent a transabdominal surgical resection between September 2005 and June 2012. Multivariable logistic regression was used to identify factors associated with omission of RT. Among 1199 identified patients, 1119 (93%) received neoadjuvant RT, 51 (4%) did not receive RT, and 29 (2%) received adjuvant RT. Among 51 patients not receiving RT, only 19 (37%) were referred and evaluated by a radiation oncologist. On multivariable analysis, clinical factors associated with not receiving RT included a history of prior pelvic RT (adjusted odds ratio [aOR], 23.9; P=.0003), ECOG performance status of 2 or greater (aOR, 11.1; P=.01), tumor distance from the anal verge greater than 10 cm (aOR, 5.4; P=.009), age at diagnosis of 75 years or older (aOR, 4.43; P=.002), body mass index of 25 to 30 kg/m(2) and less than 25 kg/m(2) (aOR, 5.22 and 4.23, respectively; P=.03), and clinical stage II (aOR, 2.27; P=.02). No significant change was seen in RT use according to diagnosis year, nor was any correlation seen with distance to the nearest RT facility. Concordance with NCCN Guidelines for neoadjuvant RT is high among NCCN Member Institutions. After adjusting for clinical characteristics that increase the risk for RT toxicity, including history of pelvic RT and high comorbidity burden/low functional status, the authors found that non-obese patients of advanced age or those with more favorable clinical features were more likely to not receive RT.

Hepatobiliary cystadenocarcinoma.

Biliary cystadenocarcinomas are rare tumors that are poorly understood. Preoperative imaging is imprecise and treatment is not standardized. We describe the presentation and treatment of one of these rare tumors and review the limited published literature.

Case report of pneumatosis intestinalis secondary to sunitinib treatment for refractory gastrointestinal stromal tumor.

Pneumatosis intestinalis (PI) occurs when inter-luminal air enters the bowel wall of the gastrointestinal tract via a mucosal defect. The condition is caused by numerous disease states, direct trauma, and various drugs. When PI is secondary to drug therapy, discontinuation of the offending agent results in the resolution of PI. We report on the case of a 73-year-old male with a history of refractory gastrointestinal stromal tumor experiencing PI while on sunitinib treatment. PI was noted via computed tomography (CT) scans 68 days after starting sunitinib therapy and showed near complete resolution on a follow up CT performed one month after discontinuing sunitinib. Given that a CT scan performed five months prior to the initiation of sunitinib did not show PI, lack of abdominal symptoms in our patient, and resolution of PI after discontinuing sunitinib, the cause of PI in our patient was likely due to sunitinib treatment.

Intestinal lymphangiectasia mimicking primary peritoneal carcinoma.

Intestinal lymphangiectasia is an obstruction of the lymphatic system. We report on a patient with mesenteric adenopathy and an elevated CA125 level, which were suspicious for peritoneal carcinoma. Further evaluation and bowel resection identified intestinal lymphangiectasia. This disease should be considered in patients with mesenteric adenopathy and a small bowel mass.

Hepatocellular carcinoma masquerading as a large renal mass with hepatic invasion.

Large masses are evaluated with imaging to assess primary origin and tumor spread. We present the unusual case of a 53-year-old male with a 17-cm right upper quadrant mass suspected to be renal or adrenal in origin based on radiographic findings. After surgical excision, the mass was subsequently discovered to be primary hepatocellular carcinoma with direct extension to the kidney and adrenal gland. A diagnosis of chronic hepatitis B was made postoperatively. Primary hepatocellular carcinoma with direct renal extension is an exceedingly rare occurrence based on our experience and review of the published literature.

Long-term survival results of surgery alone versus surgery plus 5-fluorouracil and leucovorin for stage II and stage III colon cancer: pooled analysis of NSABP C-01 through C-05. A baseline from which to compare modern adjuvant trials.

BACKGROUND: The objective of this study is to conduct a pooled analysis of National Surgical Adjuvant Breast and Bowel Project (NSABP) colon trials involving surgery and surgery plus 5-fluorouracil and leucovorin (5-FU/LV) to compare survival and establish a baseline from which to evaluate future studies. METHODS: All patients enrolled in NSABP adjuvant trials C-01 through C-05 with stage II and III disease who were treated with surgery or with surgery plus 5-FU/LV were examined for overall survival (OS), disease-free survival (DFS), and recurrence-free interval (RFI). Time-to-event by treatment group was examined using adjusted Kaplan-Meier estimates and multivariable Cox regression analysis. RESULTS: There were 2,966 eligible patients: 693 (23%) surgery and 2,273 (77%) surgery plus 5-FU/LV; 1,255 (42%) stage II and 1,711 (58%) stage III. Age > or =60 years [hazard ratio (HR) = 1.36, P < 0.0001], male gender (HR = 1.20, P = 0.0012), and more nodes positive or fewer nodes examined (P < 0.0001) were associated with worse survival. At 5 years, the adjusted OS was 0.62 [confidence interval (CI) = 0.60-0.63] in the surgery group and 0.76 (CI = 0.74-0.78) in the surgery plus 5-FU/LV group. Treatment with 5-FU/LV was associated with improved outcome compared with surgery: OS (HR = 0.62, P < 0.0001), DFS (HR = 0.66, P < 0.0001) and RFI (HR = 0.64, P < 0.0001). Improved OS with adjuvant treatment was seen in both stage II (HR = 0.58, 95% CI = 0.48-0.71) and stage III disease (HR = 0.65, 95% CI = 0.55-0.75). CONCLUSIONS: This analysis demonstrates that treatment of colon cancer patients with 5-FU/LV following surgery provides benefit over surgery alone and can provide anticipated survival outcomes with which to compare modern adjuvant trials.