Characteristics and malignancy rates of adult patients diagnosed with choledochal cyst in the West: a systematic review.

BACKGROUND: The approach to patients with choledochal cysts (CCs) remains varied and subject to institutional practices. Owing to the rarity of the disease, the optimal treatment remains poorly defined, particularly in the adult population. This study aimed to review the literature on adult patients with CCs to evaluate trends of diagnosis and management in Western countries. METHODS: A literature search of 3 electronic databases was performed on adult patients diagnosed with CCs in Western institutions. A review of published literature was completed with comprehensive screening by 2 independent reviewers. Studies were analyzed, and data on surgical approach, malignancies, and follow-up were collected. Findings are presented in concordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. RESULTS: Of the 3488 articles retrieved, 21 studies evaluated Western adults with CCs for a combined population of 1337 patients. The most common Todani subtypes included types I (64%) and IV (22%). Symptoms at presentation included abdominal pain and jaundice, although many were asymptomatic. Ultrasound was used most frequently for diagnosis, followed by computed tomography and endoscopic cholangiopancreatography. The combined malignancy rate was 10.9%, with cholangiocarcinoma being the most prevalent. Complete extrahepatic cyst resection was standard for type I and IV CCs. Among malignancies, 18.5% and 16.4% were observed in patients with prior resection and internal drainage, respectively. CONCLUSIONS: A significant proportion of patients who undergo resection of CC disease harbor malignancy. Cancer risk seems reduced but not eliminated with complete resection, which remains the standard treatment. Additional studies are needed to standardize guidelines for the diagnosis and postoperative care of patients in Western countries.

Patterns of Failure Following Preoperative Chemotherapy and Stereotactic Body Radiation Therapy and Resection for Patients with Borderline Resectable or Locally Advanced Pancreatic Cancer.

BACKGROUND: The role of neoadjuvant stereotactic body radiation therapy (SBRT) in the treatment of pancreatic adenocarcinoma (PDAC) is controversial and the optimal target volumes and dose-fractionation are unclear. The aim of this study is to report on treatment outcomes and patterns of failure of patients with borderline resectable (BL) or locally advanced (LA) pancreatic cancer following preoperative chemotherapy and SBRT. METHODS: We conducted a single-institution, retrospective study of patients with BL or LA PDAC. Patients received neoadjuvant chemotherapy and SBRT was prescribed to 30 Gy over 5 fractions to the pancreas planning tumor volume (PTV). A subset of patients received a simultaneous integrated boost to the high risk vascular PTV and/or elective nodal irradiation (ENI). Following neoadjuvant chemoradiation, all patients underwent subsequent resection. Overall survival (OS), progression-free survival (PFS), locoregional recurrence-free survival (LRRFS), distant metastasis-free survival (DMPFS), and locoregional control (LRC) estimates were obtained using Kaplan-Meier analysis. RESULTS: Twenty-two patients with BL (18) or LA (4) PDAC were treated with neoadjuvant chemotherapy and SBRT followed by resection from 2011-2022. Following neoadjuvant treatment, 5 patients (23%) achieved a pathologic complete response (pCR) and 16 patients (73%) had R0 resection. At 24 months, there were no isolated locoregional recurrences (LRRs), 9 isolated distant recurrences (DRs), and 5 combined LRRs and DRs. Two LRRs were in-field, 2 LRRs were marginal, and 1 LRR was both in-field and marginal. 2-year median LRC, LRRFS, DMPFS, PFS, and OS were 77.3%, 45.5%, 31.8%, 31.8%, and 59.1%, respectively. For BL and LA cancers, 2-year LRC, DMPFS, and OS were 83% vs. 75%, (p = 0.423), 39% vs. 0% (p = 0.006), and 61% vs. 50% (p = 0.202), respectively. ENI was associated with improved LRC (p = 0.032) and LRRFS (p = 0.033). Borderline resectability (p = 0.018) and lower tumor grade (p = 0.027) were associated with improved DMPFS. CONCLUSIONS: Following preoperative chemotherapy and SBRT, locoregional failure outside of the target volume occurred in 3 of 5 recurrences; ENI was associated with improved LRC and LRRFS. Further studies are necessary to define the optimal techniques for preoperative radiation therapy.

Surgical resection criteria and neoadjuvant therapies for intrahepatic cholangiocarcinoma.

The staging of intrahepatic cholangiocarcinoma (ICC) is complex, and there is no consensus among international cancer groups on how to most appropriately select candidates with nonmetastatic disease for surgical resection. Factors contributing to a higher stage of disease include larger tumor size, multiple tumors, vascular invasion (either portal venous or arterial), biliary invasion, involvement of local hepatic structures, serosal invasion, and regional lymph node metastases. For patients selected to undergo surgery, it is well-documented that R0 resection translates to a survival benefit. Estimating the risk of post-hepatectomy liver failure and post-surgical residual liver function is vital and may preclude some patients with significant tumor burden from undergoing surgery. Numerous serum and biliary biomarkers of the disease can help detect recurrence in patients undergoing surgical resection. Systemic and locoregional neoadjuvant treatments to facilitate better surgical outcomes have yielded mixed results regarding improving resectability and overall survival. Additional research is needed to identify optimal neoadjuvant treatment approaches and to evaluate which patients will benefit most from these strategies. Therapies targeting genetic mutations and protein aberrations found by tumor molecular profiling may offer additional options for future neoadjuvant treatment.

Impact of adjuvant therapy in patients with invasive intraductal papillary mucinous neoplasms of the pancreas: an international multicenter cohort study.

BACKGROUND: Adjuvant therapy prolongs survival in patients with pancreatic ductal adenocarcinoma. However, no clear guidelines are available regarding the oncologic effects of adjuvant therapy (AT) in resected invasive intraductal papillary mucinous neoplasms (IPMN). The aim was to investigate the potential role of AT in patients with resected invasive IPMN. MATERIALS AND METHODS: From 2001 to 2020, 332 patients with invasive pancreatic IPMN were retrospectively reviewed in 15 centres in eight countries. Propensity score-matched and stage-matched survival analyses were conducted. RESULTS: A total of 289 patients were enroled in the study after exclusion (neoadjuvant therapy, unresectable disease, uncertain AT status, and stage IV). A total of 170 patients were enroled in a 1:1 propensity score-matched analysis according to the covariates. In the overall cohort, disease-free survival was significantly better in the surgery alone group than in the AT group ( P =0.003), but overall survival (OS) was not ( P =0.579). There were no significant differences in OS in the stage-matched analysis between the surgery alone and AT groups (stage I, P =0.402; stage II, P =0.179). AT did not show a survival benefit in the subgroup analysis according to nodal metastasis (N0, P =0.481; N+, P =0.705). In multivariate analysis, node metastasis (hazard ratio, 4.083; 95% CI, 2.408-6.772, P <0.001), and cancer antigen 19-9 greater than or equal to 100 (hazard ratio, 2.058; 95% CI, 1.247-3.395, P =0.005) were identified as adverse prognostic factors in resected invasive IPMN. CONCLUSION: The current AT strategy may not be recommended to be performed with resected invasive IPMN in stage I and II groups, unlike pancreatic ductal adenocarcinoma. Further investigations of the potential role of AT in invasive IPMN are recommended.

The Microbiome and Metabolomic Profile of the Transplanted Intestine with Long-Term Function.

We analyzed the fecal microbiome by deep sequencing of the 16S ribosomal genes and the metabolomic profiles of 43 intestinal transplant recipients to identify biomarkers of graft function. Stool samples were collected from 23 patients with stable graft function five years or longer after transplant, 15 stable recipients one-year post-transplant and four recipients with refractory rejection and graft loss within one-year post-transplant. Lactobacillus and Streptococcus species were predominant in patients with stable graft function both in the short and long term, with a microbiome profile consistent with the general population. Conversely, Enterococcus species were predominant in patients with refractory rejection as compared to the general population, indicating profound dysbiosis in the context of graft dysfunction. Metabolomic analysis demonstrated significant differences between the three groups, with several metabolites in rejecting recipients clustering as a distinct set. Our study suggests that the bacterial microbiome profile of stable intestinal transplants is similar to the general population, supporting further application of this non-invasive approach to identify biomarkers of intestinal graft function.

Robotic Hepaticojejunostomy for Late Anastomotic Biliary Stricture After Liver Transplantation: Technical Description and Case Series.

Biliary strictures after liver transplantation are common and when refractive to endoscopic and percutaneous intervention require surgical revision. Robotic technology facilitates minimally invasive biliary reconstruction and has not previously been described after liver transplantation. Robotic biliary revisions were retrospectively compared to all the historical open cases over a time period from May 2013 to October 2020. During the study period there were 3 robotic and 4 open surgical biliary revisions with a follow-up of at least 6months. All cases were hepaticojejunostomies for late choledocho-choledochostomy anastomotic strictures presenting > 4 weeks after transplant and refractive to at least 3 endoscopic and/or percutaneous interventions. Median (range) case time was longer in the robotic group, 373 minutes (286-373) compared to open group, 280 minutes (163-321). The median length of stay was shorter in the robotic group, 4 days (1--4) compared to open group 7 days (4-10). Morbidity included 2 wound infections in the open group (grade II), 1 infected hematoma in the robotic group (grade Ilia), and 1 bile leak on the open group (grade Ilia). There was no biliary stricture recurrence or mortality in either group. Robotic biliary revision is a safe alternative to traditional open biliary revision for refractive biliary strictures after liver transplantation.

Improving safety of robotic major hepatectomy with extrahepatic inflow control and laparoscopic CUSA parenchymal transection: technical description and initial experience.

BACKGROUND: Blood loss is a major determinant of outcomes following hepatectomy. Robotic technology enables hepatobiliary surgeons to mimic open techniques for inflow control and parenchymal transection during major hepatectomy, increasing the ability to minimize blood loss and perform safe liver resections. METHODS: Initial experience of 20 consecutive major robotic hepatectomies from November 2018 to July 2020 at two co-located institutions was reviewed. All cases were performed with extrahepatic inflow control and parenchymal transection with the laparoscopic cavitron ultrasonic surgical aspirator (CUSA), and a technical description is illustrated. Clinical characteristics, operative data, and surgical outcomes were retrospectively analyzed. RESULTS: The median (range) patient age was 58 years (20-76) and the majority of 14 (70%) patients were ASA III-IV. There were 12 (60%) resections for malignancy and the median tumor size was 6.2 cm (1.2-14.6). Right or extended right hepatectomy was the most common procedure (12 or 60% of cases). There were 7 (35%) left or extended left hepatectomies and 1 (5%) central hepatectomy. The median operative time was 420 (177-622) minutes. Median estimated blood loss was 300 mL (25-800 mL). One (5%) case was converted to open. Two (10%) patients required blood transfusion. The median length of stay was 3 (1-6) days. Major complications included 1 (5%) Clavien-Dindo IIIa bile leak requiring percutaneous drainage placement. There was no 90-day mortality. CONCLUSION: Advanced techniques to reduce blood loss in robotic hepatectomy may optimize safety and minimize morbidity in these complex minimally invasive procedures.

Robot-assisted combined pancreatectomy/hepatectomy for metastatic pancreatic acinar cell carcinoma: case report and review of the literature.

Acinar cell carcinoma (ACC) of the pancreas is a rare neoplasm with less aggressive behavior than ductal carcinoma. As a result, surgical resection for metastatic ACC is a therapeutic option which can result in long-term survival. There is a paucity of data describing institutional approaches to these challenging patients, and therefore, we herein describe our institution's approach to a patient with a distal pancreatic ACC and isolated liver metastasis. The patient underwent neoadjuvant chemotherapy (FOLFIRINOX), followed by a robot-assisted distal pancreatectomy/splenectomy and non-anatomic segment 6 resection. He was discharged to home post-operative day 2. Final pathology revealed complete tumor response of the liver metastasis and a margin negative resection of the primary tumor. He remains disease free and without complications at 3 months. We highlight that combined modality therapy for metastatic ACC can yield long-term survival in selected patients. Similarly, the robotic platform enables performance of complex multivisceral resections with rapid recovery. Future research investigating precision medicine for metastatic ACC is warranted given widely variable tumor biology in this disease.

Robotic video-assisted thoracoscopy: minimally invasive approach for management of mediastinal tumors.

In spite of difficult anatomic access for tumors of mediastinum, surgical resection remains the best diagnostic and therapeutic approach. Widespread acceptance of video-assisted thoracoscopy (VATS) is restricted by the limiting nature of instruments and suboptimal visualization. Robotic assisted minimally invasive surgery seems to hold most promise in remote, narrow anatomical regions. After obtaining approval from Institutional Review Board (IRB), a retrospective review of prospectively collected database on patients that underwent Robotic VATS between 2009 and 2013 was conducted. Forty-eight patients underwent RVATS resection of mediastinal tumor. One procedure (2.1%) was converted to open. The size of the mass ranged from 0.6 to 12.5 cm in greatest dimension (mean 5.16 cm). The mean duration of procedure was 127.96 min (60-240 min). Five patients (10.4%) had early postoperative complications including chylothorax (1 patient), new onset atrial fibrillation (1 patient), pleural effusion (1 patient), empyema (1 patient), and bleeding (1 patient). Mean follow-up time was 186 days (10-1300 days). Two patients (4%) with invasive thymoma developed local recurrence. The present study documents the feasibility of RVATS in the management of mediastinal tumors irrespective of the location in various mediastinal compartments. The role for careful and complete excision of the tumor, and surveillance afterward on invasive thymoma, was noted in our study, as in literature.

Adenoid cystic carcinoma of the breast, 20 years of experience in a single center with review of literature.

BACKGROUND: Adenoid cystic carcinoma (ACC) of the breast is a rare type of breast cancer, which presents inconsistencies in the optimal management strategy. METHODS: A retrospective review of prospectively collected data, spanning the last 20 years, was performed using the cancer registry database at our institution. RESULTS: Six patients were diagnosed with ACC of the breast, out of 5,813 total patients diagnosed with breast cancer (0.1%). Our identified patients had a median age of 66, all with the early stage cancer (Stage I/II). The average size of the breast lesion was 1.62 cm, and nodal status was negative for all cases. All patients had resection as primary therapy (partial or total mastectomy), with one patient also undergoing external beam radiation and tamoxifen hormonal therapy. Median follow-up was 85 months, with all patients being disease-free at last follow-up. CONCLUSIONS: ACC of the breast has an indolent course, despite triple negative status. Our study suggests that radiation may not be warranted and confirms the rarity of axillary node metastases, indicating that sentinel node excision may also not be necessary. Ultimately, the hope is that our findings along with the reviewed literature will aid in determining the most appropriate options for management of ACC of the breast.

Impact of intraoperative blood loss on the short-term outcomes of laparoscopic liver resection.

BACKGROUND: Intraoperative blood loss is one of the predictors of outcome of open hepatectomy. But the impact of blood loss in laparoscopic hepatectomy (LH) on postoperative outcomes is poorly understood. The aim of this study is to analyze the association between blood loss and postoperative outcomes after LH. METHODS: A retrospective analysis of prospectively maintained database of patients undergoing LH from 1995 to 2016 was performed. The data were divided into two groups based on the extent of blood loss: Group 1 (<250 ml) and Group 2 (≥250 ml). The basic characteristics and postoperative outcomes were compared between these groups. RESULTS: A total of 504 patients underwent 611 LH (Group 1: 414 and Group 2: 197). The mean age was 62.4 years. The most common indication was liver secondaries (71.7%). Major hepatectomy was performed in 37% cases. Mean operative time was 225 ± 110.5 min and estimated blood loss was 239 ± 399.4 ml (range 0-4500 ml). Group 2 had significantly higher number of patients with malignant lesions undergoing major hepatectomy, anatomical resection with higher requirement for blood transfusion, and longer hospital stay. The incidence of conversion rate, overall complications including liver failure, renal failure, and postoperative mortality, was significantly higher in Group 2. However, the bile leak rate was similar in the two groups. CONCLUSION: Intraoperative blood loss is most frequent in patients undergoing major LH. Blood loss ≥250 ml during LH may adversely affect the postoperative outcomes.

Robotic video-assisted thoracoscopic lung resection for lung tumors: a community tertiary care center experience over four years.

INTRODUCTION/BACKGROUND: After its initial description in 1990, video-assisted thoracoscopic surgery (VATS) has emerged as the minimally invasive approach for lung resection in early lung cancer. METHODS: A retrospective review of prospectively collected data on patients who underwent robotic pulmonary resection for cancer by a single surgeon, between years 2009 and 2013, was performed. Age, gender, type and duration of surgery, length of stay, estimated blood loss, early and late complications, follow-up time, and local recurrence were reviewed and analyzed descriptively. RESULTS: Three hundred and thirty-one patients underwent the procedure for pulmonary neoplasm. Two hundred and fifty-nine (79%) patients underwent anatomic lobectomies, 56 (17%) patients had wedge resection, while five (1.5%) patients underwent pneumonectomy. In 11 patients, no pulmonary resection was performed for different reasons. Most common neoplasm was adenocarcinoma (185, 56%). All procedures involved a systematic mediastinal and hilar lymph node exploration and removal of suspicious nodes. Twenty-six (6.9%) procedures were converted to open thoracotomy. Mean duration of surgery was 185.63 min. Mean length of hospital stay was 5.52 days. Mean estimated blood loss (EBL) was 47.85 ml. Mean follow-up was 249.41 days (20-1550 days), and five (1.5%) patients developed local recurrence. Early complications were seen in 29 patients (8.8%), most commonly cardiac arrhythmias (20, 6%). CONCLUSION: Robotic video-assisted thoracoscopic surgery is feasible in lung lesions, with all the advantages of VATS in terms of decreased length of stay and decreased blood loss with local recurrence rate and complication rate comparable to open procedures. There is a clear need for more studies comparing the apparent advantages of robotic-assisted surgery with increased cost of technology.

Primary breast lymphoma: 30 years of experience with diagnosis and treatment at a single medical center.

In our retrospective study covering the past 30 years at a tertiary cancer care institution, we review 30 patients with primary breast lymphoma (stage I or II) and analyze the different treatment modalities. All 30 patients had unilateral disease, and the median age was 67.5 years. Two patients refused treatment and, hence, were excluded from our study. Of the 28 remaining patients, 11 (39%) were treated with a single treatment modality, and 17 (61%) underwent different combinations of surgery, radiation therapy, and chemotherapy. Seventeen patients underwent a surgical resection as the primary modality or as part of a multimodality therapy. Seven patients (25%) experienced a local or distal recurrence of the disease. Of these 7 patients, 6 underwent surgery as the primary treatment or as part of a combined treatment modality, and 1 underwent radiation therapy only. Eighteen patients (64%) died during the follow-up period ranging from 6 to 230 months, but only 5 of these 18 patients (28% [18% of all patients]) died of disease-specific causes. Of the 2 patients who were only treated with chemotherapy, 1 had primary breast lymphoma that never went into remission. In our study, surgery as the primary modality therapy or as part of a multimodality therapy for primary breast lymphoma is associated with a higher rate of treatment failure, whereas a combination of chemotherapy and local radiation therapy provides the best results. However, because our sample size is small, for such a rare neoplasm, definitive treatment recommendations are difficult to determine.

Validation of the sentinel lymph node biopsy technique in head and neck cancers of the oral cavity.

The purpose of this study was to present our experience and validate the use of sentinel lymph node (SLN) mapping in patients with head and neck cancers. A retrospective review of a prospectively collected database of patients with a diagnosis of squamous cell carcinomas of the head and neck from 2008 to 2011 was done. The group consisted of a total of 20 patients. The first node(s) highlighted with blue, or identified as radioactive by Tc99-sulfur radioactive colloid, was (were) identified as the SLNs. In the first seven patients, formal modified neck dissection was performed. In the remaining 13 patients, only a SLN biopsy procedure was done. At least one SLN was identified in all 20 patients (100%). Only one patient (5%) had positive nodes. In this case, the SLN was also positive. In the remaining 19 cases, all lymph nodes were negative. After an average of 24 months of follow-up, there have been three local recurrences (15%) but no evidence of distant metastatic disease. SLN mapping in head and neck cancers is a feasible technique with a high identification rate and a low false-negative rate. Although the detection rate of regional metastatic disease compares favorably with published data as well as the disease-free and overall survival, further studies are warranted before considering this technique to be the "gold standard" in patients with oral squamous cell carcinoma and a negative neck by clinical examination and imaging studies.

A multidisciplinary approach for the treatment of GIST liver metastasis.

BACKGROUND: Advanced gastrointestinal stromal tumors (GISTs) can metastasize and recur after a long remission period, resulting in serious morbidity, mortality, and complex management issues. CASE PRESENTATION: A 67-year-old woman presented with epigastric fullness, mild jaundice and weight loss with a history of a bowel resection 7 years prior for a primary GIST of the small bowel. The finding of a heterogeneous mass 15.5 cm in diameter replacing most of the left lobe of the liver by ultrasonography and CT, followed by positive cytological studies revealed a metastatic GIST. Perioperative optimization of the patient's nutritional status along with biliary drainage, and portal vein embolization were performed. Imatinib was successful in reducing the tumor size and facilitating surgical resection. CONCLUSION: A well-planned multidisciplinary approach should be part of the standard management of advanced or metastatic GIST.