Gavin A. Falk

Pancreatic Lesions in von Hippel–Lindau Disease? A Systematic Review and Meta-synthesis of the Literature

Backgroundvon Hippel–Lindau (vHL) disease is a rare condition that leads to characteristic lesions within many different body systems. Pancreatic manifestations of vHL cover a wide spectrum of pathologies, and thus, accurate characterization and management is critical.MethodsA comprehensive and systematic text word and MeSH search of the medical literature was performed to identify studies where information regarding the prevalence, clinical characteristics, and management recommendations could be extracted.ResultsEleven studies were identified but 2 studies utilized the same data set. Of the 10 remaining studies, a total of 1,442 patients with vHL were available for analysis. Four hundred and twenty patients were examined for any type of pancreatic lesion, 362 for simple cysts or serous cystadenomas (SCAs), and 1,442 for neuroendocrine tumors (NETs). Of the 420 assessed for any pancreatic manifestation of vHL, 252 (60%) had a pancreatic lesion identified. Simple cysts that present as the sole manifestation of pancreatic disease were common and found in 169 of 362 (47%) patients. These are usually asymptomatic and do not normally require intervention. SCAs were reported in 39 of 362 (11%) patients and followed a similar benign course; resection is acceptable in symptomatic patients. NETs were identified in 211 of 1,442 (15%) patients, and 27 of 1,442 (2%) lesions behaved malignantly. Management of NETs depends on size, doubling time, and underlying genetics. Renal cell carcinoma is a characteristic in vHL, but there were no cases of pancreatic metastases identified from the included studies. Adenocarcinomas of the pancreas are not pathogenically linked to vHL.ConclusionsThis review highlights the wide spectrum and high prevalence of pancreatic lesions in vHL. Simple cysts and SCAs are benign, but NETs require careful observation due to their malignant potential.

Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen: Case report and review of the literature

INTRODUCTION Sclerosing Angiomatoid Nodular Transformation of the spleen (SANT) is a rare benign vascular lesion of the spleen with extensive sclerosis and unknown etiology. PRESENTATION OF CASE We report a new case of SANT of the spleen found in a 53-year-old female following detection of a splenic mass on a routine computed tomography (CT). The patient underwent an uncomplicated laparoscopic splenectomy and the specimen was sent for histopathologic examination. DISCUSSION A review of the 97 reported cases of SANT found in the literature was undertaken. There were 43 males and 54 females with a median age of 46 years (range: 11-82 years). SANT is classically considered to be a female predominant disease, however 44.3% of reported case were male and the gender predilection may soon be neutralized as more cases are reported. 65 of the 97 (67%) patients were in 30-60 year age group. The majority of lesions (n=50) were incidentally found on imaging, and for those patients presenting with symptoms, abdominal pain (n=18) was the predominant symptom. CONCLUSION The diagnosis of SANT should be considered in any patient presenting with a splenic lesion that contains an angiomatoid or inflammatory component. As the differential diagnosis for SANT includes malignant pathologies, and currently no reliable diagnostic radiological feature has been identified to differentiate between these conditions, SANT will continue to be diagnosed on the basis of surgical histopathology.

Robotic surgery of the pancreas

Pancreatic surgery is one of the most challenging and complex fields in general surgery. While minimally invasive surgery has become the standard of care for many intra-abdominal pathologies the overwhelming majority of pancreatic surgery is performed in an open fashion. This is attributed to the retroperitoneal location of the pancreas, its intimate relationship to major vasculature and the complexity of reconstruction in the case of pancreatoduodenectomy. Herein, we describe the application of robotic technology to minimally invasive pancreatic surgery. The unique capabilities of the robotic platform have made the minimally invasive approach feasible and safe with equivalent if not better outcomes (e.g., decreased length of stay, less surgical site infections) to conventional open surgery. However, it is unclear whether the robotic approach is truly superior to traditional laparoscopy; this is a key point given the substantial costs associated with procuring and maintaining robotic capabilities.

Natural history of asymptomatic pancreatic cystic neoplasms

BACKGROUND The management of asymptomatic pancreatic cysts is controversial and indications for excision are based on pathology and natural history. OBJECTIVES This study aimed to examine outcomes of asymptomatic lesions using a protocol based on size and cyst fluid analysis. METHODS Asymptomatic cysts were identified from a prospectively maintained database. Sequential cross-sectional imaging studies were assessed, and results of endoscopic ultrasound-guided aspiration were co-analysed. RESULTS A total of 338 asymptomatic patients underwent evaluation. Overall, 84 cysts were <1.5 cm and 254 were ≥1.5 cm in diameter. Median patient follow-up was 5.1 years [interquartile range (IQR): 4.1-6.9 years]. In the group in which cysts measured <1.5 cm in diameter, median cyst size was 1.0 cm (IQR: 0.6-1.2 cm) at presentation and increased to 1.2 cm (IQR: 0.7-1.6 cm) during follow-up. Five (6.0%) patients underwent resection, all within 2 months of presentation. In the group in which cysts measured ≥1.5 cm in diameter, median cyst size was 2.5 cm (IQR: 2.0-3.4 cm) at presentation and increased to 2.7 cm (IQR: 3.0-4.2 cm). A total of 63 (24.8%) patients underwent resection. Surgery was performed with 2 months in 53 (84.1%) patients, within 12 months in four (6.3%) patients and at >12 months post-presentation in six (9.5%) patients. A total of 70.6% of resected specimens were identified as malignancies or mucinous lesions. CONCLUSIONS Asymptomatic cysts of <1.5 cm in diameter can safely be followed by imaging and are expected to undergo little change. A quarter of all asymptomatic cysts measuring ≥1.5 cm are appropriately resected based on imaging and cyst fluid analysis.

A 10-Step Intraoperative Surgical Checklist (ISC) for Laparoscopic Cholecystectomy—Can It Really Reduce Conversion Rates to Open Cholecystectomy?

IntroductionThe recent introduction of a Surgical Safety Checklist has significantly reduced the morbidity and mortality of surgery. Such a simple measure that can impact so highly on surgical outcomes causes all surgeons to pause for thought. This paper documents the introduction of a 10-step intraoperative surgical checklist (ISC) to standardize performance, decision-making, and training during laparoscopic cholecystectomy (LC). The checklist’s impact on conversion rates to open cholecystectomy (OC) is presented.MethodsIn 2004, a 10-step ISC was introduced by a single consultant surgeon for the performance of LCs. Data were collected comparing LCs between 1999–2003 (period 1) and 2004–2008 (period 2). Data on sex, age, American Society of Anesthesiology grade, previous abdominal surgery, severity of gallbladder pathology, and conversion to OC were recorded. The chi-squared test with Yates correction was used to compare groups.ResultsIn total, 637 LCs were performed, 277 during period 1 and 360 during period 2. Risk factors for conversion (gender, age, previous abdominal surgery, and severity of gallbladder pathology) were not significantly different in the two periods studied. The overall conversion rate to OC fell significantly in period 2 (p = 0.001). Subgroup analysis also showed a significant reduction in conversion rates in female patients (p = 0.002) and patients with grades III and IV gallbladder disease (p = 0.001).ConclusionsThe introduction of a 10-step ISC was temporally related to reduced conversion rates to OC. The standardization of a frequently performed operation such as a LC that could potentially lead to an impact as great the one we observed warrants further attention in prospective, appropriately designed studies.

Adenoid cystic carcinoma of the base of the tongue: Late metastasis to the pancreas.

Adenoid cystic carcinoma (ACC) is a relatively rare epithelial tumor of the salivary glands. We present a 64-year-old gentleman with ACC of the tongue who following resection and radiotherapy, presented 10 years later with a lung metastasis and underwent operative intervention and further radiotherapy. Five years later he presented with obstructive jaundice found to be metastatic ACC. We believe this to be the first report of an ACC metastasizing to the pancreas.

Prognostic value of the lymph node ratio after resection of periampullary carcinomas

BACKGROUND Data have indicated that the lymph node ratio (LNR) may be a better prognostic indicator than lymph node status in pancreatic cancer. OBJECTIVES To analyse the value of the LNR in patients undergoing resection for periampullary carcinomas. METHODS A cut off value of 0.2 was assigned to the LNR in accordance with published studies. The impact of histopathological factors including a LNR was analysed using Kaplan-Meier and Cox regression methods. RESULTS In total, 551 patients undergoing a resection (January 2000 to December 2010) were analysed. The median lymph node yield was 15, and 198 (34%) patients had a LNR > 0.2. In patients with a LNR of > 0.2, the median overall survival (OS) was 18 versus 33 months in patients with an LNR < 0.2 (P < 0.001). Univariate analysis demonstrated a LNR > 0.2, T and N stage, vascular or perineural invasion, grade and resection margin status to be significantly associated with OS. On multivariate analysis, only a LNR > 0.2, vascular or perineural invasion and margin positivity remained significant. In N1 disease, a LNR was able to distinguish survival in patients with a similar lymph node burden, and correlated with more aggressive tumour pathological variables. CONCLUSION A LNR > 0.2, and not lymph note status, is an independent prognostic factor for OS indicating the LNR should be utilized in outcome stratification.

Lymphoepithelial cysts of the pancreas:a management dilemma.

Pancreatic lymphoepithelial cysts (LECs) are rare, benign lesions that are typically unexpected post-operative pathological findings. We aimed to review clinical, radiological and pathological features of LECs that may allow their pre-operative diagnosis. Histopathology databases of two large pancreatic units were searched to identify LECs and notes reviewed to determine patient demographic details, mode of presentation, investigations, treatment and outcome. Five male and one female patients were identified. Their median age was 60 years. Lesions were identified on computed tomography performed for abdominal pain in two patients, and were incidentally observed in four patients. Five LECs were located in the tail and one in the body of the pancreas, with a median cyst size of 5 cm. Obtaining cyst fluid was difficult and a largely acellular aspirate was yielded. The pre-operative diagnosis was mucinous cystic neoplasm in all patients. This series of patients were treated distal pancreatectomy and splenectomy. A retrospective review of radiological examinations suggested that LECs have a relatively low signal on T2 imaging and a high signal intensity on T1 weighted images. LECs appear more common in elderly males, and are typically incidental, large, unilocular cysts. Close attention to signal intensity on MRI may allow pre-operative diagnosis of these lesions.

Jejunal cavernous lymphangioma.

Cavernous lymphangiomas are usually identified in infants and children with the majority of lesions found around the head and neck, trunk or extremities. Tumours affecting the intra-abdominal organs are rare. The authors report a case of small bowel cavernous lymphangioma arising within the jejunum of a 34-year-old woman presenting with dyspnoea and anaemia, and review the existing literature relating to this uncommon tumour.

Simultaneous Clear Cell Sarcomas of the Duodenum and Jejunum

Clear cell sarcoma (CCS) is an uncommon tumor that usually presents as an extremity mass but can rarely manifest as a gastrointestinal tumor with a diverse spectrum of symptoms, most commonly related to a mass effect or ulceration. Herein we report a case in which two separate tumors, one in the duodenum and the other in the jejunum, present concurrently. The subject presented with symptomatic anemia and underwent imaging and endoscopic studies that culminated in the discovery of the two lesions. He subsequently underwent operative treatment with resection of both tumors and made an unremarkable recovery. The resection specimen consisted of two separate clear cell sarcomas with negative margins. Under microscopic evaluation, they demonstrated nested growths of epithelioid cells with scattered spindled cells infiltrating the enteric wall. The neoplastic cells were positive for S100 with scattered expression of Melan A. Florescence in situ hybridization revealed a translocation at the EWRS1 locus. He was disease-free for 30 months following the procedure; then he developed a rapidly progressing metastatic disease with subsequent death 4 months later.