Faculty Bibliography

PURPOSE/OBJECTIVE:To evaluate the impact of a multidisciplinary clinic on the clinical care recommendations of patients with pancreatic cancer compared with the recommendations the patients received prior to review by the multidisciplinary tumor board. METHODS:The records of 203 consecutive patients referred to the Johns Hopkins pancreatic multidisciplinary clinic were prospectively collected from November 2006 to October 2007. Cross-sectional imaging, pathology, and medical history were evaluated by a panel of medical/radiation oncologists, surgical oncologists, pathologists, diagnostic radiologists, and geneticists. Alterations in treatment recommendations between the outside institution and the multidisciplinary clinic were recorded and compared. RESULTS:On presentation, the outside computed tomography (CT) report described locally advanced/unresectable disease (34.9%), metastatic disease (17.7%), and locally advanced disease with metastasis (1.1%). On review of submitted imaging and imaging performed at Hopkins, 38 out of 203 (18.7%) patients had a change in the status of their clinical stage. Review of the histological slides by dedicated pancreatic pathologists resulted in changes in the interpretation for 7 of 203 patients (3.4%). Overall, 48 out of 203 (23.6%) patients had a change in their recommended management based on clinical review of their case by the multidisciplinary tumor board. Enrollment into the National Familial Pancreas Tumor Registry increased from 52 out of 106 (49.2%) patients in 2005 to 158 out of 203 (77.8%) with initiation of the multidisciplinary clinic. CONCLUSION/CONCLUSIONS:The single-day pancreatic multidisciplinary clinic provided a comprehensive and coordinated evaluation of patients that led to changes in therapeutic recommendations in close to one-quarter of patients.

PURPOSE/OBJECTIVE:To examine the efficacy of adjuvant chemoradiotherapy after pancreaticoduodenectomy (PD) for pancreatic adenocarcinoma (PC) in patients undergoing resection at Johns Hopkins Hospital (JHH; Baltimore, MD). PATIENTS AND METHODS/METHODS:Between August 30, 1993, and February 28, 2005, a total of 908 patients underwent PD for PC at JHH. A prospective database was reviewed to determine which patients received fluorouracil (FU) -based CRT. Excluded patients had metastatic disease, died 60 or fewer days after PD, received preoperative therapy, an experimental vaccine, adjuvant chemotherapy or radiation alone. The final cohort includes 616 patients. RESULTS:The median follow-up was 17.8 months (interquartile range, 9.7 to 33.5 months). Overall median survival was 17.9 months (95% CI, 16.3 to 19.5 months). Groups were similar with respect to tumor size, nodal status, and margin status, but the CRT group was younger (P < .001), and less likely to present with a severe comorbid disease (P = .001). Patients with carcinomas larger than 3 cm (P = .001), grade 3 and 4 (P < .001), margin-positive resection (P = .001), and complications after surgery (P = .017) had poor long-term survival. Patients receiving CRT experienced an improved median (21.2 v 14.4 months; P < .001), 2-year (43.9% v 31.9%), and 5-year (20.1% v 15.4%) survival compared with no CRT. After controlling for high-risk features, CRT was still associated with improved survival (relative risk = 0.74; 95% CI, 0.62 to 0.89). CONCLUSION/CONCLUSIONS:These data suggest that adjuvant concurrent FU-based CRT significantly improves survival after PD for PC when compared with patients not receiving CRT. These data support the use of combined adjuvant CRT for PC.

INTRODUCTION/BACKGROUND:Acinar cell carcinoma (ACC) is a rare, malignant neoplasm with a generally poor prognosis. We report our institutional series of 14 patients with ACC to determine current guidelines for their evaluation and treatment. MATERIALS AND METHODS/METHODS:The Johns Hopkins pathology prospective database was reviewed from 1988 to 2006 to identify patients with pancreatic neoplasms possessing features of acinar cell differentiation. Retrospective review and follow-up was performed for each patient. RESULTS:Fourteen patients with ACC were identified with a median age of 57 years. All patients presented with abdominal pain or discomfort with none showing evidence of lipase hypersecretion syndrome. Each patient underwent surgical resection, including nine pancreaticoduodenectomies and five distal pancreatectomies. Median tumor size was 3.9 cm with 12 patients found to have stage IIB disease or worse. Four patients underwent neoadjuvant chemoradiation. Eight of the fourteen patients developed recurrent disease. Overall median survival and disease-free survival were 33 and 25 months, respectively, as compared to a median survival of 18 months for pancreatic adenocarcinoma. CONCLUSION/CONCLUSIONS:Acinar cell carcinomas are rare, aggressive neoplasms that are difficult to diagnose and treat. Operative resection represents the best first-line treatment. These lesions have a better prognosis than the more common pancreatic adenocarcinomas.

BACKGROUND:The reported incidence of choledochal cyst (CC) disease varies greatly based on geography. Most large series on CC originate from East Asia. So our understanding of the presentation and natural history of CC disease in western societies is less well characterized. Recognition and surgical treatment are important because of the significant longterm risks of developing cholangiocarcinoma. We report here the largest single-institution western experience with CC disease. STUDY DESIGN/METHODS:Ninety-two patients with CC disease who were seen at our institution between 1976 and 2006 were included in this study. Potential differences between children and adults (defined as 16 years old or older) were specifically evaluated. RESULTS:There were 19 children and 73 adults; 90% were girls/women with type I cysts. Adults were more likely to present with abdominal pain (97% versus 63%, p < 0.001), and children were more likely to present with jaundice (71% versus 25%, p=0.001). Surgical management most commonly involved cyst excision and Roux-en-Y hepaticojejunostomy reconstruction. Four adults (three with cholangiocarcinoma and one with gall bladder cancer) and one child (with embryonal rhabdomyosarcoma) had malignancies associated with their CC at the time of excision. Two patients (with type IV and type V disease) developed cholangiocarcinoma after surgical management. Another patient died of pancreatic adenocarcinoma 21 years after excision of her type I cyst. There was no operative or hospital mortality, and no patient who underwent complete cyst excision developed cholangiocarcinoma during a mean followup of 10 years. CONCLUSIONS:Western CC disease has a similar demographic profile as that seen in Asia. CCs are more frequently found in adults and girls/women and are associated with a longterm risk of developing cholangiocarcinoma. Presentation differs between adults and children. After complete cyst excision, no patients developed cholangiocarcinoma.

BACKGROUND:Based on data from other malignancies, the number of lymph nodes evaluated and the ratio of metastatic to examined lymph nodes (LNR) may be important predictors of survival. LNR has never been investigated in a large population-based study of patients with pancreatic adenocarcinoma. METHODS:The Surveillance, Epidemiology, and End Results (SEER) database was used to identify 4005 patients who underwent resection for pancreatic adenocarcinoma from 1988 to 2003. The effect of total lymph node count and LNR on survival was examined using univariate and multivariate analyses. RESULTS:The median number of lymph nodes examined was seven; 390 (10.1%) patients had no lymph nodes examined. Of those patients who had at least one lymph node examined, 1507 (43.3%) had no lymph node metastases (N0) and 1971 (56.7%) had metastatic nodal disease (N1). Overall median survival was 13 months, and 5-year survival was 6.8%. N1 disease was associated with a worse 5-year survival compared with N0 disease (4.3 vs 11.3%, respectively, P < .001). Patients with N0 disease could be further stratified based on the number of lymph nodes evaluated (median survival: 1-11 nodes, 16 months vs 12 or more nodes, 23 months; P < .001). For N1 patients, LNR was one of the most powerful factors associated with survival (LNR > 0-0.2, 15 months; LNR > 0.2-0.4, 12 months; LNR > 0.4, 10 months) (P < .001). CONCLUSIONS:Most patients have an inadequate number of lymph nodes evaluated following pancreatic surgery. N0 patients who have fewer than 12 lymph nodes examined may be understaged. In patients with N1 disease, LNR may better substratify patients with regard to prognosis.

BACKGROUND:To date, no consensus has been reached regarding which primary tumor subtypes are managed appropriately with hepatic metastectomy. Specifically, the role of hepatic resection for metastatic periampullary or pancreatic adenocarcinoma remains controversial. METHODS:Between 1995 and 2005, 1563 patients underwent surgical resection for periampullary carcinoma (n=608 patients) or pancreatic adenocarcinoma (head, n=905 patients; tail, n=50 patients). Data on demographics, operative details, primary tumor status, and-when indicated-extent of hepatic metastasis were collected. RESULTS:Of the 1563 patients who underwent resection of periampullary or pancreatic adenocarcinoma, 22 patients (1.4%) underwent simultaneous hepatic resection for synchronous liver metastasis. The primary tumor site was ampullary (n=1 patient ), duodenal (n=2 patients), distal bile duct (n=2 patients), or pancreas (head, n=10 patients; tail, n=7 patients). The majority of patients (86.4%) had a solitary hepatic metastasis, and the median size of the largest lesion was 0.6 cm. Hepatic metastectomy included wedge resection (n=20 patients), segmentectomy (n=1 patient), and hemihepatectomy (n=1 patient). After matching patients on primary tumor histology and location, the median survival of patients who underwent hepatic resection of synchronous metastasis was 5.9 months compared with 5.6 months for patients who underwent palliative bypass alone (P=.46) and 14.2 months for patients with no metastatic disease who underwent primary tumor resection only (P<.001). Pancreatic (median, 5.9 months) versus nonpancreatic (median, 9.9 months) primary tumor histology was not associated with a difference in survival in patients who underwent resection of synchronous liver metastasis (P=.43). CONCLUSIONS:Even in well selected patients with low-volume metastatic liver disease, simultaneous resection of periampullary or pancreatic carcinoma with synchronous liver metastases did not result in long-term survival in the overwhelming majority of patients.

The goal of this article is to describe the different types of benign pancreatic neoplasms, methods to distinguish between them, and treatment options. Pancreatic adenocarcinoma is associated with specific neoplastic lesions that are similar in radiographic appearance to some benign lesions. The correct differentiation of these malignant and premalignant lesions from their benign counterpart is paramount to their proper management.