Clinical practice guidelines for the management of liver metastases from extrahepatic primary cancers 2021.

BACKGROUND: Hepatectomy is standard treatment for colorectal liver metastases; however, it is unclear whether liver metastases from other primary cancers should be resected or not. The Japanese Society of Hepato-Biliary-Pancreatic Surgery therefore created clinical practice guidelines for the management of metastatic liver tumors. METHODS: Eight primary diseases were selected based on the number of hepatectomies performed for each malignancy per year. Clinical questions were structured in the population, intervention, comparison, and outcomes (PICO) format. Systematic reviews were performed, and the strength of recommendations and the level of quality of evidence for each clinical question were discussed and determined. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to assess evidence and make recommendations. RESULTS: The eight primary sites were grouped into five categories based on suggested indications for hepatectomy and consensus of the guidelines committee. Fourteen clinical questions were devised, covering five topics: (1) diagnosis, (2) operative treatment, (3) ablation therapy, (4) the eight primary diseases, and (5) systemic therapies. The grade of recommendation was strong for one clinical question and weak for the other 13 clinical questions. The quality of the evidence was moderate for two questions, low for 10, and very low for two. A flowchart was made to summarize the outcomes of the guidelines for the indications of hepatectomy and systemic therapy. CONCLUSIONS: These guidelines were developed to provide useful information based on evidence in the published literature for the clinical management of liver metastases, and they could be helpful for conducting future clinical trials to provide higher-quality evidence.

Dilemmas in Diagnosis and Management of Gastroenteropancreatic Mixed Neuroendocrine Non-neuroendocrine Neoplasms: First Single-Centre Report from India.

PURPOSE: Mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) is a rare neoplasm comprising of exocrine and neuroendocrine elements, each representing ≥ 30% lesion. It is commonly misdiagnosed as adenocarcinoma or grade-3 neuroendocrine neoplasm (NEN). Management is not well-defined. METHODS: Retrospective analysis of prospectively entered data at our centre from January 2011 to January 2018 revealed 16 MiNENs off 130 neuroendocrine neoplasms (NENs). These were analysed for demographics, clinicopathological characteristics, management strategies and prognosis. RESULTS: Four out of 16 patients, metastatic at presentation, were started on chemotherapy. Eleven of remaining 12 patients had pre-operative biopsy. Only two were diagnosed MiNEN. Four patients (33.34%) received 5-fluorouracil (5-FU)-based neoadjuvant chemotherapy and underwent curative surgery with adjuvant cisplatin+etoposide (Cis-Eto). Out of these, two patients (16.6%) developed metastasis and were shifted to capecitabine+temozolomide (Cap-Tem). Six patients (50%) with neuroendocrine-dominant MiNEN received adjuvant Cis-Eto after surgery. Two (16.6%) developed metastases for which Cap-Tem was started. One of them developed locoregional and liver metastasis. Three patients (25%) have succumbed to progressive disease, three (25%) are on treatment, and six (50%) are disease-free at 4-30 months. CONCLUSION: Preoperative diagnosis of MiNEN is challenging, and it needs quality histopathological examination and immunohistochemistry. The 30% criteria is therapeutically insignificant, and treatment based on most aggressive component is prognostically more relevant. Neoadjuvant 5-FU-based regimens may downstage adenocarcinoma-dominant tumours. There are no guidelines on adjuvant Cis-Eto. Cap-Tem can be considered second-line chemotherapy. Poor survival is reported irrespective of site of origin and adjuvant therapy.

Multimodality management, recurrence patterns, and long-term outcome of gastroenteropancreatic neuroendocrine neoplasms: Progress over 17 years.

BACKGROUND: Many advances in the management of gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) happened in the last two decades. This study highlights the progress in its management over 17 years, outcomes, recurrence patterns, and follow up protocols. METHODS: This retrospective analysis of prospectively maintained database at a single tertiary center included GEP-NEN patients from January 2001 to August 2017. Management protocols were based on European Neuroendocrine Tumor Society guidelines. Recurrences were categorized as follows: localized nodal, regional, distant hepatic, or combined. Patients were divided into cohorts: cohort 1 (2001-2006), cohort 2 (2007-2011), and cohort 3 (2012-2017). Survival patterns were analyzed. RESULTS: One hundred and ninety-two patients were included with 98 (51.04%) grade (G) 1, 64 (33.34%) G2, and 30 (15.63%) G3. One hundred and four (54.16%) underwent curative surgery (58 G1, 27 G2, and 19 G3). Overall follow up ranged from 3 to 276 months; 39 were lost to follow up. Ninety-six patients had recurrences: 44 regional + distant and 40 liver-limited recurrences. One-, 3-, and 5-year survivals show significant differences among different treatment groups (p < 0.05). Significant increase in curative resections, chemotherapy utilization, and reduced recurrences were noted in cohort 3. Curative (R0) resection offered 1- and 3-year overall survival of 93.3% and 66.7% in cohort 1; 95.8% and 83.1% in cohort 2; and 100% and 92.9% in cohort 3. CONCLUSION: Curative resection is the most significant factor for improved survival. Debulking surgerical procedure have a role whereas upfront peptide receptor radionuclide therapy is questionable. Chemotherapy improves overall survival in inoperable/metastatic setting. Recurrence patterns indicate that a long-term follow up greater than 10 years is necessary.

Tokyo Guidelines 2018: surgical management of acute cholecystitis: safe steps in laparoscopic cholecystectomy for acute cholecystitis (with videos).

In some cases, laparoscopic cholecystectomy (LC) may be difficult to perform in patients with acute cholecystitis (AC) with severe inflammation and fibrosis. The Tokyo Guidelines 2018 (TG18) expand the indications for LC under difficult conditions for each level of severity of AC. As a result of expanding the indications for LC to treat AC, it is absolutely necessary to avoid any increase in bile duct injury (BDI), particularly vasculo-biliary injury (VBI), which is known to occur at a certain rate in LC. Since the Tokyo Guidelines 2013 (TG13), an attempt has been made to assess intraoperative findings as objective indicators of surgical difficulty; based on expert consensus on these difficulty indicators, bail-out procedures (including conversion to open cholecystectomy) have been indicated for cases in which LC for AC is difficult to perform. A bail-out procedure should be chosen if, when the Calot's triangle is appropriately retracted and used as a landmark, a critical view of safety (CVS) cannot be achieved because of the presence of nondissectable scarring or severe fibrosis. We propose standardized safe steps for LC to treat AC. To achieve a CVS, it is vital to dissect at a location above (on the ventral side of) the imaginary line connecting the base of the left medial section (Segment 4) and the roof of Rouvière's sulcus and to fulfill the three criteria of CVS before dividing any structures. Achieving a CVS prevents the misidentification of the cystic duct and the common bile duct, which are most commonly confused. Free full articles and mobile app of TG18 are available at: http://www.jshbps.jp/modules/en/index.php?content_id=47. Related clinical questions and references are also included.

Tokyo Guidelines 2018: diagnostic criteria and severity grading of acute cholangitis (with videos).

Although the diagnostic and severity grading criteria on the 2013 Tokyo Guidelines (TG13) are used worldwide as the primary standard for management of acute cholangitis (AC), they need to be validated through implementation and assessment in actual clinical practice. Here, we conduct a systematic review of the literature to validate the TG13 diagnostic and severity grading criteria for AC and propose TG18 criteria. While there is little evidence evaluating the TG13 criteria, they were validated through a large-scale case series study in Japan and Taiwan. Analyzing big data from this study confirmed that the diagnostic rate of AC based on the TG13 diagnostic criteria was higher than that based on the TG07 criteria, and that 30-day mortality in patients with a higher severity based on the TG13 severity grading criteria was significantly higher. Furthermore, a comparison of patients treated with early or urgent biliary drainage versus patients not treated this way showed no difference in 30-day mortality among patients with Grade I or Grade III AC, but significantly lower 30-day mortality in patients with Grade II AC who were treated with early or urgent biliary drainage. This suggests that the TG13 severity grading criteria can be used to identify Grade II patients whose prognoses may be improved through biliary drainage. The TG13 severity grading criteria may therefore be useful as an indicator for biliary drainage as well as a predictive factor when assessing the patient's prognosis. The TG13 diagnostic and severity grading criteria for AC can provide results quickly, are minimally invasive for the patients, and are inexpensive. We recommend that the TG13 criteria be adopted in the TG18 guidelines and used as standard practice in the clinical setting. Free full articles and mobile app of TG18 are available at: http://www.jshbps.jp/modules/en/index.php?content_id=47. Related clinical questions and references are also included.

Tokyo Guidelines 2018: diagnostic criteria and severity grading of acute cholecystitis (with videos).

The Tokyo Guidelines 2013 (TG13) for acute cholangitis and cholecystitis were globally disseminated and various clinical studies about the management of acute cholecystitis were reported by many researchers and clinicians from all over the world. The 1st edition of the Tokyo Guidelines 2007 (TG07) was revised in 2013. According to that revision, the TG13 diagnostic criteria of acute cholecystitis provided better specificity and higher diagnostic accuracy. Thorough our literature search about diagnostic criteria for acute cholecystitis, new and strong evidence that had been released from 2013 to 2017 was not found with serious and important issues about using TG13 diagnostic criteria of acute cholecystitis. On the other hand, the TG13 severity grading for acute cholecystitis has been validated in numerous studies. As a result of these reviews, the TG13 severity grading for acute cholecystitis was significantly associated with parameters including 30-day overall mortality, length of hospital stay, conversion rates to open surgery, and medical costs. In terms of severity assessment, breakthrough and intensive literature for revising severity grading was not reported. Consequently, TG13 diagnostic criteria and severity grading were judged from numerous validation studies as useful indicators in clinical practice and adopted as TG18/TG13 diagnostic criteria and severity grading of acute cholecystitis without any modification. Free full articles and mobile app of TG18 are available at: http://www.jshbps.jp/modules/en/index.php?content_id=47. Related clinical questions and references are also included.

Tokyo Guidelines 2018: flowchart for the management of acute cholecystitis.

We propose a new flowchart for the treatment of acute cholecystitis (AC) in the Tokyo Guidelines 2018 (TG18). Grade III AC was not indicated for straightforward laparoscopic cholecystectomy (Lap-C). Following analysis of subsequent clinical investigations and drawing on Big Data in particular, TG18 proposes that some Grade III AC can be treated by Lap-C when performed at advanced centers with specialized surgeons experienced in this procedure and for patients that satisfy certain strict criteria. For Grade I, TG18 recommends early Lap-C if the patients meet the criteria of Charlson comorbidity index (CCI) ≤5 and American Society of Anesthesiologists physical status classification (ASA-PS) ≤2. For Grade II AC, if patients meet the criteria of CCI ≤5 and ASA-PS ≤2, TG18 recommends early Lap-C performed by experienced surgeons; and if not, after medical treatment and/or gallbladder drainage, Lap-C would be indicated. TG18 proposes that Lap-C is indicated in Grade III patients with strict criteria. These are that the patients have favorable organ system failure, and negative predictive factors, who meet the criteria of CCI ≤3 and ASA-PS ≤2 and who are being treated at an advanced center (where experienced surgeons practice). If the patient is not considered suitable for early surgery, TG18 recommends early/urgent biliary drainage followed by delayed Lap-C once the patient's overall condition has improved. Free full articles and mobile app of TG18 are available at: http://www.jshbps.jp/modules/en/index.php?content_id=47. Related clinical questions and references are also included.

The role of preoperative C-reactive protein and procalcitonin as predictors of post-pancreaticoduodenectomy infective complications: A prospective observational study.

INTRODUCTION: The common causes of morbidity after pancreaticoduodenectomy (PD) are infective complications. Till date, no specific preoperative markers have been identified to determine the probability of developing infective complications. We have studied the factors predicting the occurrence of the infective complication/s in the present study. METHODS: The present prospective observational study included 133 consecutive patients who underwent PD from January 2011 to June 2016 at a specialized hepatopancreaticobiliary surgical oncology unit. The surgeries were done using a standardized technique. Postoperative complications were segregated into two categories-(a) infective (e.g. cholangitis) and (b) non-infective (e.g. delayed gastric emptying). Increased age, preoperative serum albumin levels, preoperative biliary stenting, pre-stenting serum bilirubin levels, duration of common bile duct stenting, preoperative C-reactive protein [CRP], and procalcitonin [PCT] were evaluated. RESULTS: Overall morbidity rate was 48.8%. Morbidity associated with infective complications was 21.8%. Increased age, preoperative serum albumin levels, and pre-stenting serum bilirubin levels did not increase the rate of the infective complications. The association between preoperative PCT and preoperative CRP with the infective complications was significant with a p-value of <0.01 (6.75E-07) and <0.01 (4.80E-10), respectively. In the multivariate analysis, only the elevated preoperative procalcitonin was a statistically significant predictor of postoperative infective complications. CONCLUSION: Preoperative PCT and CRP levels done 48 h before surgery are sensitive, specific, easily available, and cost-effective predictors of infective complications after PD.

Reporting the margin in pancreaticoduodenectomies: R0 versus R1.

AIM: This study was conducted to analyze the changes in margin positivity in pancreaticoduodenectomies, on using a standardized protocol, which bread loafs the pancreas and duodenum in the axial plane for adenocarcinomas arising in the pancreatic head, ampulla, terminal common bile duct (CBD), and duodenum, and then to assess whether these tumor subsets involve the margins in different ways. METHODS: The analysis was performed on 70 consecutive specimens, the pre-protocol specimens serving as the control group. RESULTS AND CONCLUSIONS: Tumors originating from the pancreatic head, ampulla, terminal CBD, and duodenum showed a consistent increase in their R1 incidence, post-protocol. Ampullary tumors showed the greatest upward change in R1 positivity. The highest incidence of margin positivity was seen in pancreatic head adenocarcinomas (80%), then distal CBD tumors (60%), and finally the ampullary tumors (39%). In pancreatic head adenocarcinomas, R1 increased from 55% to 80%, distal CBD from 50% to 60%, and ampullary from 17% to 39%. Duodenal adenocarcinomas had no R1 in both pre- and post-protocol groups. The tumors also had different patterns of margin involvement. Ampullary tumors involved only the posterior margin, pancreatic adenocarcinomas involved the superior mesenteric vein (SMV) groove more often than the posterior margin, and distal CBD tumors involved the posterior margin and SMV groove equally. The size of the tumor made a significant difference in pancreatic head carcinomas with tumor size less than or equal to 2 cm, showing an R1 incidence of 38%, while those above 2 cm had an R1 incidence of 68%.

Neoadjuvant chemotherapy in patients with locally advanced gallbladder cancer.

AIM: Surgery is the only curative option for patients with gallbladder cancer (GBC). This study looks at the outcome of patients treated with neoadjuvant chemotherapy (NACT). PATIENTS & METHODS: This is retrospective analysis of the prospectively maintained database of patients with locally advanced GBC treated between February 2009 and September 2013 with NACT. Patients received gemcitabine-platinum based regimen. RESULTS: A total of 37 patients (median age: 54 years, 64.9% females) received NACT. Overall response rate was 67.5%. In total, 17 patients (46%) underwent R0 resection. Median overall survival/progression-free survival of the whole group was 13.4/8.1 months, respectively. Patients who underwent surgery had a significantly better overall survival (median not reached vs 9.5 months) and progression-free survival (25.8 vs 5.6 months), respectively. CONCLUSION: NACT increases resectability and survival in patients with locally advanced GBC.

Recommendations for laparoscopic liver resection: a report from the second international consensus conference held in Morioka.

The use of laparoscopy for liver surgery is increasing rapidly. The Second International Consensus Conference on Laparoscopic Liver Resections (LLR) was held in Morioka, Japan, from October 4 to 6, 2014 to evaluate the current status of laparoscopic liver surgery and to provide recommendations to aid its future development. Seventeen questions were addressed. The first 7 questions focused on outcomes that reflect the benefits and risks of LLR. These questions were addressed using the Zurich-Danish consensus conference model in which the literature and expert opinion were weighed by a 9-member jury, who evaluated LLR outcomes using GRADE and a list of comparators. The jury also graded LLRs by the Balliol Classification of IDEAL. The jury concluded that MINOR LLRs had become standard practice (IDEAL 3) and that MAJOR liver resections were still innovative procedures in the exploration phase (IDEAL 2b). Continued cautious introduction of MAJOR LLRs was recommended. All of the evidence available for scrutiny was of LOW quality by GRADE, which prompted the recommendation for higher quality evaluative studies. The last 10 questions focused on technical questions and the recommendations were based on literature review and expert panel opinion. Recommendations were made regarding preoperative evaluation, bleeding controls, transection methods, anatomic approaches, and equipment. Both experts and jury recognized the need for a formal structure of education for those interested in performing major laparoscopic LLR because of the steep learning curve.

Experience with the technique of pancreas-sparing distal duodenectomy.

Pancreas-sparing distal duodenectomy (PSDD) is a novel surgical technique for tumors of distal duodenum below the ampulla to achieve oncologically free margins and avoid multiple anastomoses. We report PSDD performed in five cases, three duodenal adenocarcinoma, and two neuroendocrine tumors (NETs). Three patients had adenocarcinoma of D3 and D4 with free ampulla. PSDD was performed with total excision of regional nodes. In the two patients with NETs, one had a mass lesion close to the pancreatic head. The mass was excised followed by PSDD. There were four small primary NETs in the duodenum, and the mass was metastatic lymph node. The second patient had primary duodenal NET with liver metastases. After transarterial chemoembolization, PSDD with liver metastatectomy was performed. Specimens in all five cases showed clear margins. The patients had a smooth recovery and were well at a median follow up of 10 months.

TG13 miscellaneous etiology of cholangitis and cholecystitis.

This paper describes typical diseases and morbidities classified in the category of miscellaneous etiology of cholangitis and cholecystitis. The paper also comments on the evidence presented in the Tokyo Guidelines for the management of acute cholangitis and cholecystitis (TG 07) published in 2007 and the evidence reported subsequently, as well as miscellaneous etiology that has not so far been touched on. (1) Oriental cholangitis is the type of cholangitis that occurs following intrahepatic stones and is frequently referred to as an endemic disease in Southeast Asian regions. The characteristics and diagnosis of oriental cholangitis are also commented on. (2) TG 07 recommended percutaneous transhepatic biliary drainage in patients with cholestasis (many of the patients have obstructive jaundice or acute cholangitis and present clinical signs due to hilar biliary stenosis or obstruction). However, the usefulness of endoscopic naso-biliary drainage has increased along with the spread of endoscopic biliary drainage procedures. (3) As for biliary tract infections in patients who underwent biliary tract surgery, the incidence rate of cholangitis after reconstruction of the biliary tract and liver transplantation is presented. (4) As for primary sclerosing cholangitis, the frequency, age of predilection and the rate of combination of inflammatory enteropathy and biliary tract cancer are presented. (5) In the case of acalculous cholecystitis, the frequency of occurrence, causative factors and complications as well as the frequency of gangrenous cholecystitis, gallbladder perforation and diagnostic accuracy are included in the updated Tokyo Guidelines 2013 (TG13). Free full-text articles and a mobile application of TG13 are available via http://www.jshbps.jp/en/guideline/tg13.html.

TG13 surgical management of acute cholecystitis.

BACKGROUND: Laparoscopic cholecystectomy is now accepted as a surgical procedure for acute cholecystitis when it is performed by an expert surgeon. There are several lines of strong evidence, such as randomized controlled trials (RCTs) and meta-analyses, supporting the introduction of laparoscopic cholecystectomy for patients with acute cholecystitis. The updated Tokyo Guidelines 2013 (TG13) describe the surgical treatment for acute cholecystitis according to the grade of severity, the timing, and the procedure used for cholecystitis in a question-and-answer format using the evidence concerning surgical management of acute cholecystitis. METHODS AND MATERIALS: Forty-eight publications were selected for a careful examination of their full texts, and the types of surgical management of acute cholecystitis were investigated using this evidence. The items concerning the surgical management of acute cholecystitis were the optimal surgical treatment for acute cholecystitis according to the grade of severity, optimal timing for the cholecystectomy, surgical procedure used for cholecystectomy, optimal timing of the conversion of cholecystectomy from laparoscopic to open surgery, and the complications of laparoscopic cholecystectomy. RESULTS: There were eight RCTs and four meta-analyses concerning the optimal timing of the cholecystectomy. Consequently, it was found that cholecystectomy is preferable early after admission. There were three RCTs and two meta-analyses concerning the surgical procedure, which concluded that laparoscopic cholecystectomy is preferable to open procedures. Literature concerning the surgical treatment according to the grade of severity could not be quoted, because there have been no publications on this topic. Therefore, the treatment was determined based on the general opinions of professionals. CONCLUSION: Surgical management of acute cholecystitis in the updated TG13 is fundamentally the same as in the Tokyo Guidelines 2007 (TG07), and the concept of a critical view of safety and the existence of extreme vasculobiliary injury are added in the text to call the surgeon's attention to the need to reduce the incidence of bile duct injury. Free full-text articles and a mobile application of TG13 are available via http://www.jshbps.jp/en/guideline/tg13.html.

Chronic heavy metal exposure and gallbladder cancer risk in India, a comparative study with Japan.

BACKGROUND: High incidence of gallbladder cancer (GBC) is reported from North India, with elevated concentrations of heavy metals in water and soil. This Indo-Japan collaborative study compared presence of heavy metals in gallbladder tissues. METHODS: Heavy metal concentrations were estimated in Indian GBC and cholecystitis tissues and compared with Japanese GBC and cholecystitis tissues. Spectrophotometry was done for 13 Indian gallbladder tissues (8 GBC, 5 cholecystitis) and 9 Japanese (5 GBC, 4 cholecystitis). Transmission electron microscopy (TEM) thin foil element analysis was done in 10 Indian samples (6 GBC, 4 cholecystitis). RESULTS: Chromium, lead, arsenic and zinc were significantly high in Indian GBC compared with Japanese GBC. Chromium, lead and arsenic were significantly high in the Indian cholecystitis tissues compared to the Japanese. TEM of Indian tissues demonstrated electron dense deposits in GBC. CONCLUSION: Heavy metals- chromium, lead, arsenic and zinc are potential carcinogens in Indian GBC from endemic areas. This preliminary study links presence of heavy metals in gallbladder cancer tissues in endemic areas.

Surgical treatment of liver metastases in neuroendocrine neoplasms.

Neuroendocrine neoplasms (NENs) are a distinctive entity, and nearly 10% of patients already have liver metastases at presentation. The management of neuroendocrine liver metastases (NEN-LM) is complex with differing patterns of metastatic presentation. An aggressive approach should be used to resect the primary tumor, to remove regional lymph nodes, and to resect or treat appropriate distant metastases (including liver tumors). Despite having an indolent course, NENs have a significantly reduced survival when liver metastases are untreated. Though a wide range of therapies are now available with a multimodal approach to the treatment, surgical treatment offers the only chance for a significant survival prolongation and/or improvement of symptoms and quality of life. A review of the existing surgical modalities for NEN-LM is discussed in this paper.

Postsurgical infections are reduced with specialized nutrition support.

OBJECTIVE: The objective was to examine the relationship between pre-, peri-, and postoperative specialized nutritional support with immune-modulating nutrients and postoperative morbidity in patients undergoing elective surgery. METHODS: Studies were identified by searching MEDLINE, review article bibliographies, and abstracts and proceedings of scientific meetings. All randomized clinical trials in which patients were supplemented by the IMPACT formula before and/or after elective surgery and the clinical outcomes reported were included in the meta-analysis. Seventeen studies (n=2,305), 14 published (n=2,102), and 3 unpublished (n=203), fulfilled the inclusion criteria. Ten studies (n=1,392) examined the efficacy of pre- or perioperative IMPACT supplementation in patients undergoing elective surgery, whereas 7 (n=913) assessed postoperative efficacy. Fourteen of the studies (n=2,083) involved gastrointestinal (GI) surgical patients. Postoperative complications, mortality, and length of stay in hospital (LOS) were major outcomes of interest. RESULTS: IMPACT supplementation, in general, was associated with significant (39%-61%) reductions in postoperative infectious complications and a significant decrease in LOS in hospital by an average of 2 days. The greatest improvement in postoperative outcomes was observed in patients receiving specialized nutrition support as part of their preoperative treatment. In GI surgical patients, anastomotic leaks were 46% less prevalent when IMPACT supplementation was part of the preoperative treatment. CONCLUSION: This study identifies a dosage (0.5-1 l/day) and duration (supplementation for 5-7 days before surgery) of IMPACT that contributes to improved outcomes of morbidity in elective surgery patients, particularly those undergoing GI surgical procedures. The cost effectiveness of such practice is supported by recent health economic analysis. Findings suggest preoperative IMPACT use for the prophylaxis of postoperative complications in elective surgical patients.

Microcystic serous cystadenoma of the pancreas: a report of two cases with one of diffuse presentation.

Microcystic adenoma or serous cystadenoma is an uncommon tumor and accounts for 1-2% of the exocrine neoplasms of the pancreas. Usually unifocal, they present as single, large, well-demarcated multiloculated cystic tumors, ranging in size from 1 to 25 cm. Multifocal variants or diffuse serous cystadenomas are extremely rare. We present 2 cases of which 1 is a diffuse variant affecting the body, tail and part of the neck of the pancreas. In both the patients the tumors were detected incidentally. We highlight on the diffuse variant in view of its rarity and present a review of literature. In this case the entire body and tail of the pancreas was spongy replaced by multicystic lobules and hyalinized fibrocollagenous stroma. The cysts were lined by low cuboidal glycogen containing bland cells. Such a unique presentation wherein the entire body and tail of the pancreas is replaced with multiple cysts is a diffuse presentation of microcystic adenoma and a search through literature revealed only 7 such cases among the 15 cases with multifocal presentation reported.