Search

Hepatic haemangiomas are the most common benign hepatic tumour; they are usually solitary, less than 5 cm in size, and clinically silent. Giant cavernous haemangiomas are a minor subset of hepatic haemangiomas, often symptomatic due to a mass lesion, complications or compression of adjacent structures. Few of these are pedunculated—often difficult to diagnose because of their exophytic development and greater tendency towards complications through the twisting of their peduncle. We report the case of a 55-year-old woman with a giant pedunculated hepatic cavernous haemangioma causing abdominal pain, with epigastralgia mimicking a gastric stromal tumour. We discuss the diagnostic workup, focusing on the useful of computed tomography and magnetic resonance imaging findings and giving a brief report of the therapeutic management and literature review.

Introduction In the selection of non-small cell lung cancer (NSCLC) patients for immunotherapy, specimen processed as cell blocks (CBs) may be the only available material to assess PD-L1 expression. Therefore, optimal CB preparation becomes paramount. In this context, here we assessed whether inadequate fixation time might be one of the pre-analytical factors affecting PD-L1 expression. Methods Ex vivo CBs from placental (n = 3) and NSCLC (n = 8) resection specimens were obtained. PD-L1 staining was performed on CBs prepared at increasing fixation times (12 hours, 48 hours, 72 hours, 96 hours, 168 hours and 504 hours) using the companion diagnostic SP263 Assay and a validated 22C3 laboratory developed test (LDT). Staining intensity and percentage of positive cells were evaluated. Results All placental CBs showed moderate to strong PD-L1 positivity in most cells, regardless of the fixation time. Likewise, the percentage of SP263-stained NSCLC cells was similar at all fixation times except for one case, which showed less intense SP263 staining at 168 hours. Conversely, in 5/8 cases, the 22C3 LDT percentage of positive cells and staining intensity decreased at 168 hours and 504 hours. Conclusions Our results show that fixation time influences the performance of 22C3 LDT on CBs. Thus, we recommend that the fixation time of cytological materials be carefully checked, especially when PD-L1 testing is delayed until the oncology request. Indeed, delays in tissue processing and paraffin embedding may lead to sub-optimal performance of PD-L1 staining on CBs.

Background Thyroid fine-needle aspirates (FNAs) with undetermined morphology can be outsourced to centralized laboratories for comprehensive molecular profiling. When a local, rapid screening rules out easily detectable BRAF and NRAS mutations outsourcing is minimized, leading to cost savings. The fully automated Idylla technology, that does not require trained staff, is an emerging option. However, Idylla platform has only been validated to process formalin fixed paraffin embedded (FFPE) sections. Here we investigate whether also the FNA needle rinse could be genotyped by the same cytopathologist who performs the FNA, a procedure that can be termed rapid on site molecular evaluation (ROME). Methods To validate this approach, the Idylla BRAF and NRAS Test was performed on the rinses from 25 simulated (bench-top) FNAs, in a first part of the study. Genotyping data were compared with those obtained on matched histological FFPE blocks. The second part of the study was carried out on 25 prospectively collected routine FNAs to assess the performance of the Idylla BRAF and NRAS assay against a gold standard real time polymerase chain reaction method. Results Idylla NRAS-BRAF Mutation Test was performed on needle rinse as well as histological FFPE blocks. A sensitivity of 88.9%, a specificity of 100.0% were obtained comparing the Idylla NRAS-BRAF Mutation Test on needle rinse to the reference method. Conclusions The FNA needle rinse can be directly genotyped. This obviates the need of cell block preparation, making possible a rapid combined morphological and molecular evaluation. Since DNA extraction is no longer necessary, the cytopathologist can perform ROME him/herself.

Given the increasing incidence of neuroendocrine neoplasms (NENs) over the past few decades, a more comprehensive knowledge of their pathophysiological bases and the identification of innovative NEN biomarkers represents an urgent unmet need. There is still little advance in the early diagnosis and management of these tumors, due to the lack of sensible and specific markers with prognostic value and ability to early detect the response to treatment. Chronic systemic inflammation is a predisposing factor for multiple cancer hallmarks, as cancer proliferation, progression and immune-evading. Therefore, the relevance of inflammatory biomarkers has been identified as critical in several types of tumours, including NENs. A bidirectional relationship between chronic inflammation and development of NENs has been reported. Neuroendocrine cells can be over-stimulated by chronic inflammation, leading to hyperplasia and neoplastic transformation. As the modulation of inflammatory response represents a therapeutic target, inflammatory markers could represent a promising new key tool to be applied in the diagnosis, the prediction of response to treatment and also as prognostic biomarkers in NENs field. The present review provides an overview of the pre-clinical and clinical data relating the potentially usefulness of circulating inflammatory markers: neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), cytokines and tissue inflammatory markers (PD-1/PD-L1), in the management of NENs. (1) NLR and PLR have both demonstrated to be promising and simple to acquire biomarkers in patients with advanced cancer, including NEN. To date, in the context of NENs, the prognostic role of NLR and PLR has been confirmed in 15 and 4 studies, respectively. However, the threshold value, both for NLR and PLR, still remains not defined. (2) Cytokines seem to play a central role in NENs tumorigenesis. In particular, IL-8 levels seems to be a good predictive marker of response to anti-angiogenic treatments. (3) PD-1 and PD-L1 expression on tumour cells and on TILs, have demonstrated to be promising predictive and prognostic biomarkers in NENs. Unfortunately, these two markers have not been validated so far and further studies are needed to establish their indications and utility.

During the 5th NIKE (Neuroendocrine tumors Innovation in Knowledge and Education) meeting, held in Naples, Italy, in May 2019, discussions centered on the understanding of pathology reports of gastroenetropancreactic neuroendocrine neoplasms. In particular, the main problem concerned the difficulty that clinicians experience in extrapolating relevant information from neuroendocrine tumor pathology reports. During the meeting, participants were asked to identify and rate issues which they have encountered, for which the input of an expert pathologist would have been appreciated. This article is a collection of the most rated questions and relative answers, focusing on three main topics: 1) morphology and classification; 2) Ki67 and grading; 3) immunohistochemistry. Patient management should be based on multidisciplinary decisions, taking into account clinical and pathology-related features with clear comprehension between all health care professionals. Indeed, pathologists require clinical details and laboratory findings when relevant, while clinicians require concise and standardized reports. In keeping with this last statement, the minimum requirements in pathology datasets are provided in this paper and should be a baseline for all neuroendocrine tumor professionals.

Background: The new concept of disease remission for pediatric inflammatory bowel diseases (IBD) implies the achievement of mucosal healing. Aims: We aimed to evaluate endoscopic and histologic healing in children with Ulcerative Colitis (UC) and Crohn's disease (CD) in clinical remission after 52 weeks of Azathioprine. Methods: From December 2012 to July 2015 we prospectively enrolled IBD children starting Azathioprine. Enrolled patients in clinical remission underwent colonoscopy after 52 weeks. Macroscopic assessment was described with Mayo score and the simplified endoscopic score for UC and CD, respectively. For microscopic assessment, an average histology score was used. Data on inflammatory markers and fecal calprotectin were also collected. Results: Fourty-seven patients were included in the analysis. Endoscopic healing was detected in 20/26 (76.9%) UC children and 10/21 (47.6%) CD patients. Median Mayo score and simplified endoscopic score were significantly decreased at week 52 (p < 0.001; p = 0.005). Median average histology score was not significantly different at week 52 in both diseases. Fecal calprotectin was directly correlated with simplified endoscopic score (T0: r = 0.4, p = 0.05; T52: r = 0.5, p = 0.01), but not with Mayo score. No correlation was found between endoscopic and histologic scores. Conclusions: IBD children under Azathioprine reach endoscopic healing, but not histological remission.

Background: Pancreatic ductal adenocarcinoma (PDAC) is one of the most lethal human malignancies: novel therapeutic approaches beyond conventional chemotherapy are still lacking and prognosis remains poor, even for resectable patients (pts). Furthermore, there is an almost complete absence of validated predictive factors. Consequently, robust biomarkers for the early diagnosis and the prognostic stratification are urgently needed in clinical practice, especially in the context of neoadjuvant and adjuvant settings. In the last years, evidence revealed the crucial role of miRNAs in cancer initiation and progression, as well as in the chemo-resistance mechanisms, suggesting their use as clinical biomarkers. Material and methods: In this pilot study, we performed a microarray analysis to characterize global miRNA expression profile from surgical tissue samples collected from 20 resected PDAC pts pooled into 4 groups according to different clinico-pathological features: nodal metastases (N+/N-) and tumor grading (G2/G3). Results: According to expression patterns, we identified, among 384 miRNAs, a significant different modulation for 11 miRNAs associated to G2 vs G3 and for 7 miRNAs in N+ vs N- disease, suggesting a possible specific signature reflecting histological grade and nodal metastasis occurrence, respectively. We focused on 2 up-regulated (miR-138-5p and miR-518-3p) and 3 down-regulated (miR-215-5p, miR-519a-3p and miR-576-5p) miRNAs in N+ pts, and on 3 up-regulated (miR-1-3p, miR-31-5p and miR-205-5p) in G3 pts, in order to verify their possible implication in the molecular changes behind tumor differentiation and spread, as well as their potential use for prognostic and therapeutic purpose. A bio-informatic analysis was also performed, using different in silico tools, to study both high affinity miRNA targets and cross-regulated pathways among the upand down-regulated miRNAs. The results identified several associated targets involved in multiple signaling pathways commonly dysregulated in cancer. Finally, BRCA1/2 and RB1 miRNAs-mediated-modulation is actually ongoing, considering the pivotal role of these genes in some PDAC pts. Conclusion: These preliminary data provide a strong rationale to further investigate miRNAs expression in larger cohorts of PDAC pts, possibly integrating validated tissue miRNAs data with circulating miRNAs, in order to identify strong (and easily accessible) potential biomarker(s) with prognostic and/or predictive significance. Citation Format: Luca Pompella, Michela Falco, Carlo Caputo, Anna Grimaldi, Giuseppe Tirino, Severo Campione, Francesca Sparano, Maria Lucia Iacovino, Chiara Carmen Miceli, Carlo Molino, Marco Montella, Renato Franco, Gennaro Galizia, Giovanni Conzo, Vincenzo Napolitano, Annamaria Auricchio, Francesca Cardella, Fortunato Ciardiello, Michele Caraglia, Angela Lombardi, Gabriella Misso, Ferdinando De Vita. A pilot study of miRNA expression profile in surgically resected pancreatic ductal adenocarcinoma: Initial report from a bi-institutional cohort [abstract]. In: Proceedings of the AACR Virtual Special Conference on Pancreatic Cancer; 2021 Sep 29-30. Philadelphia (PA): AACR; Cancer Res 2021;81(22 Suppl):Abstract nr PO-052.

Objectives CCDC6 (coiled-coil domain containing 6) is a player of the HR response to DNA damage and has been predicted to interact with BAP1, another HR-DNA repair gene highly mutated in Malignant Pleural Mesothelioma (MPM), an aggressive cancer with poor prognosis. CCDC6 levels are modulated by the deubiquitinase USP7, and CCDC6 defects have been reported in several tumors determining PARP-inhibitors sensitivity. Our aim was to investigate the functional role of CCDC6 in MPM carcinogenesis and response to PARP-inhibitors. Materials and Methods The interaction between CCDC6 and BAP1 was confirmed in MPM cells, by co-immunoprecipitation. Upon USP7 inhibition, that induces CCDC6 degradation, the ability to repair the DSBs and the sensitivity to PARP inhibitors, was explored by HR reporter and by cells viability assays, respectively. A TMA including 34 MPM cores was immunostained for CCDC6, USP7 and BAP1 and the results correlated by statistical analysis. Results MPM cells depleted of CCDC6 showed defects in DSBs repair and sensitivity to PARP inhibitors. The silencing of CCDC6 when combined with the overexpression of BAP1-mutant (Δ221-238) enhanced the HR-DNA repair defects and the PARP inhibitors sensitivity. In the TMA of MPM primary samples, the staining of CCDC6 and of its de-ubiquitinase USP7 showed a significant correlation in the tested primary samples (p = 0.01). CCDC6 was barely detected in 30% of the tumors that also carried BAP1 defects. Conclusion The combination of CCDC6 and BAP1 staining may indicate therapeutic options for DDR targeting, acting in synergism with cisplatinum.

Aim Tumour neoangiogenesis is a key factor in tumour progression and metastatic spread and the possibility to assess tumour angiogenesis might provide prognostic information. The aim of this study was to establish the role of probe-based confocal laser endomicroscopy (p-CLE) in the identification of vascular architecture and specific morphological patterns in normal colorectal mucosa and malignant lesions during routine endoscopy. Method Fourteen consecutive patients with colorectal cancer were included. The following features were identified and then compared between normal and neoplastic mucosa on p-CLE images: vessel shape (straight vs irregular) vessel diameter the 'branching patterns' vessel permeability (fluorescein leakage) and blood flow (normal vs defective flux). Immunohistochemistry was used to confirm the presence and to study the morphology of vascular structures (CD-34 staining) and 'neo-vessels' (WT-1 staining) on tumour and normal mucosal sections. Results Tumour vessels appeared as irregular, ectatic and with a highly variable calibre and branching patterns on p-CLE images. The mean diameter of tumour vessels was significantly larger than those in normal mucosa (weighted mean difference 3.38, 95% CI 2.65-4.11, P = 0.01). Similarly, 'vessel branching' (OR 2.74, 95% CI 1.23-6.14, P = 0.01), fluorescent dye 'extravasation' (OR 3.46, 95% CI 1.39-8.57, P = 0.01) were significantly more frequent in colorectal cancer than in normal colorectal mucosa. Immunohistochemistry corroborated the p-CLE findings, showing higher vascularity in tumour sections due to neoformed vessels, presenting irregular patterns. Conclusion Probe-based confocal laser endomicroscopy provides a noninvasive characterization of the microvascular architecture of colonic mucosa. Different morphological patterns have been described, discriminating normal and malignant microvascular networks in colorectal mucosa.

BACKGROUND: To understand the role of ghrelin in the mechanism of action of laparoscopic sleeve gastrectomy (LSG), a prospective cohort case-control study to assess the expression of ghrelin-producing cells (GPC) in two groups of patients was designed. METHODS: Specimens of resected stomach from 26 obese patients who underwent LSG (group A), were compared by immunohistochemistry to control stomach samples from 26 non-obese patients (group B) resected for other pathologies or during autopsy; (GIST: 6 cases, inflammatory diseases: 4 cases, post-mortem autopsy cases with stomachs from healthy persons victims of traumatic accidents: 16 cases). Immunohistochemistry investigation was performed with the use of Ventana Benchmark ultra, anti-ghrelin antibody NOVUS, mouse monoclonal 2F4, diluted at 1:100. RESULTS: No significant difference in the expression of GPC number between group A and B was found (p = 0.87). No significant correlation between patients presenting a GPC number above (subgroup 1) or below (subgroup 2) the average, and EWL% changes, both at 1 and 6 years of follow-up, was recorded. CONCLUSIONS: Our study has shown that the expression of GPC is similar in the stomach of obese and non-obese controls, being mostly influenced by the inflammatory status of the gastric mucosa. A variation in the preoperative number of GPC has not influenced the weight loss in patients who underwent LSG.

Background and Aims: The aim of this study was to compare the accuracy and clinical impact of hybrid positron emission tomography [PET]/magnetic resonance-enterography [MR-E] and PET/computed tomography-enterography [CT-E] in patients with Crohn’s disease [CD]. Methods: A total of 35 patients with symptomatic small-bowel CD who were scheduled to undergo operation were evaluated before operation by same-day PET/CT-E and PET/MR-E. PET/MR-E was also compared with MR-E alone. Imaging accuracy for detecting pathological sites and discriminating between fibrotic and inflammatory strictures was assessed. Treatment was adjusted according to imaging findings and change in medical/surgical strategy was also evaluated. Results: PET/CT-E, PET/MR-E, and MR-E were equally accurate in detecting CD sites. PET/MR-E was more accurate in assessing extra-luminal disease [ p = 0.002], which was associated with higher need for stoma [ p = 0.022] and distant localisation [ p = 0.002]. When the latter was observed, laparoscopy was started with hand-assisted device, reducing operative time [ p = 0.022]. PET/MR-E was also more accurate in detecting a fibrotic component compared with PET/CT-E [ p = 0.043] and with MR-E [ p = 0.024]. Fibrosis was more frequently classified as inflammation with MR-E compared with PET/MR-E [ p = 0.019]. Out of 8 patients with predominantly inflammatory CD who received medical treatment, 6 [75%] remained surgery free. Overall, 29 patients received surgery. At median follow-up of 9 [6–22] months, no recurrences occurred in either the medical or the surgical group. Conclusions: Preoperative PET/MR-E imaging is highly accurate for assessing CD lesions before operation and contributed to clinical management of patients with small-bowel CD more often than PET/CT-E.

Hiatal hernia (HH) affects from 10% to 50% of adult population. The correlation between HH, gastroesophageal reflux disease, dyspeptic symptoms, and esophagitis has long been known in adults. The primary objective of our prospective observational study was to estimate the prevalence of HH in children undergoing esophagogastroduodenoscopy (EGD), irrespective of their symptoms. METHODS: We prospectively enrolled 111 consecutive children (48 boys and 63 girls; mean age 94.9 ± 52.3 months) referred for EGD. In all of the patients a asymptomatic score assessment based on the Rome III criteria was used to measure frequency, severity, and duration of gastrointestinal symptoms. HH presence was endoscopically defined; esophagitis presence was evaluated either endoscopically and histologically. Children were divided in 2 age-range groups: 48 months (group 2). RESULTS: Twenty-three patients of 111 (20.7%) had evidence of a sliding HH at EGD. In children from group 2, we found a statistically significant association of HH with heartburn (P = 0.03, 95% confidence interval 1-9.3, r = 0.1) and regurgitation (P = 0.003, 95% confidence interval 1.7-20.4, r = 0.3). Regarding esophagitis presence, no association was found at any age either with defined esophagitis or with dilated intercellular spaces. CONCLUSIONS: Prevalence of HH in our study population was 20.7%. According to our data, HH correlates with the presence of heartburn and regurgitation in children, but not in toddlers. No association was found with esophagitis at any age.

Rationale:There is an association between the presence of neuroendocrine neoplasms and incremented risk to develop second primary malignancies. This risk is estimated to be 17%. The most common secondary neoplasms were found in the Gastrointestinal and Genitourinary tracts.Patient concerns:A

8566 Background: Thymic epithelial tumors (TETs) are rare thoracic malignancies. Widely recognized as different histopathological entities, thymoma (T) and thymic carcinoma (TC), show a different biological behavior with a higher tendency to hematogenous dissemination for TC and thoracic recurrence for T, sharing, however, a poor prognosis when characterized by high tumor burden. Up to date, there are no specific biomarkers for monitoring the biological course of these rare tumors. Analysis of circulating cell-free DNA (cfDNA) has potential applications throughout the natural course of cancer development, diagnosis and treatment, never the less several studies have suggested that cfDNA levels closely parallel overall tumor burden. For the first time the detection and the correlation of cfDNA levels with tumor burden and histological subtype of TET, has been carried on in this monocentric study. Methods: Starting from July 2018, serum samples from 19 patients with TET, 4 with completely resected TET (rTET) and 15 with advanced (aTET), were prospectively obtained before the initiation of therapy. Serum samples from 15 healthy donors were used as control. Five ml of blood was collected and processed within one hour or less, followed by centrifugation at 3000g for 10 minutes and storage at -80°C. The serum samples were processed for QiAamp MinElute cell-free DNA mini kit extraction (Qiagen). cfDNA quantification was assessed using Qubit Fluorometric Quantitation (Thermo Fisher Scientific). Clinical, and histo-pathological features of TET were assessed. Results: A median cfDNA amount in healthy donors of 0.108 ng/µl (0.083-0.868) was registered. A median cfDNA of 0.512 ng/µl (0.178-1.42) resulted for the rTET, including the value of 0.178 for the resected TC. A median cfDNA of 2.53 ng/µl (1.20-6.11) resulted for the aTET, with respectively a median of 2.845 ng/µl (1.3-5.24) and of 1.5 ng/µl (1.2-6.11) for TC and T. The highest registered level for both group of thymoma (6.11 ng/µl) and thymic carcinoma (5.24 ng/µl) correlates with the highest tumor burden. Conclusions: To the best of our knowledge, this is the first study that explore detection and quantification of cfDNA in TET. Higher baseline levels than the control group and the rTET group have been registered for both advanced T and TC. Highest levels of cfDNA may be associated with high tumor burden despite the histological subtype. We envision that further valuable information will be obtained with mutational analysis.

In the present case, we report the association of multiple ileal neuroendocrine tumors and idiopathic myointimal hyperplasia of the mesenteric veins, 2 rare conditions that could have an etiopathogenetic relationship. Idiopathic myointimal hyperplasia of mesenteric veins implies a near-total obliteration of venular vessels, which can lead to hypoxic disorders. Hypoxia in tumors is associated with increased metastatic potential and resistance to radiotherapy and chemotherapy. Herein we speculated on the existence of a relationship between hypoxia and multiple location of neuroendocrine tumors.

Patients suffering from Inflammatory Bowel Diseases (IBD) are at increased risk of developing cancers of the gastrointestinal tract (GI). Adenocarcinomas are the most commonly observed GI tumours in IBD, and occur through an in inflammation-driven pathway. A trend toward reduced risk of bowel cancers has been observed in IBD in recent years, presumably related to improved medical treatments. However, some cancers may be independent from active inflammation, probably originating from altered interactions between the extremely active immune system of IBD patients and environmental factors. Data concerning gastrointestinal stromal tumours (GIST) and carcinoids of the GI in IBD patients are scanty. We report our experience with these rare cancers, and provide the readers with an overview on the topic, focusing on distinguishing and peculiar features of GIST and carcinoids of the GI in IBD compared with other cancer types and with general population, and address the treatment of such challenging conditions. Available data do not support an increased risk of GIST in IBD patients, but GI carcinoids may be more commonly observed in Crohn's disease. However, the presentation of GIST and GI carcinoids is protean and does not seem to be associated with disease activity in the involved GI segment in IBD. Conversely, some evidences suggest a potential role of inflammation in sustaining GI carcinoids in IBD. Increased awareness, longer duration of disease, and improved diagnostic modalities should also be considered when evaluating the increasing trend of CaT in CD patients. Treatment of GIST and CaT is not dissimilar from that of non-IBD patients, but prompt suspicion and diagnosis are crucial to achieve optimal outcomes.

BACKGROUND The pediatric literature about the correlation between symptoms and histological lesions in patients investigated for gastroesophageal reflux disease is scarce and inconclusive. The primary aim of the present study was to assess the relation between the complained symptom severity and the esophageal histological grade, through the use of validated and reliable scores. METHODS All children ages between 2 and 17 years referred to perform upper gastrointestinal endoscopy because of gastroesophageal reflux disease symptoms were asked to complete the Pediatric Gastroesophageal Symptom and Quality of Life validated questionnaire, investigating the main symptoms complained and their impact on daily life and school activities. Esophageal mucosal samples taken during the procedure were analyzed and scored according to the Yerian-Fiocca classification. RESULTS A total of 164 children were included in the study. No significant association was found between symptomatic score and histological score (r(s): 0.05, P: 0.49). Even when focusing only on adolescents with heartburn or chest pain, no correlation between symptom severity and esophageal lesions was found (r(s): -0.18, P: 0.264). Intercellular space diameter values did not mirror symptom severity. CONCLUSIONS The main finding of this study on children with reflux symptoms is the lack of correlation between symptom severity and esophageal histological grade. The magnitude of intercellular spaces was found not to be related with the clinical score as well.

Introduction There is a recent debate on the "transplantability" of ECD (Expanded Criteria Donors) kidneys and the selection criteria used to allocate them to single or double transplantation. Remuzzi et al. have defined a protocol incorporating pre-transplant donor biopsy to guide the use of older donor organs. They allocated organs as single or double transplants on the basis of histological findings. We aim to show the pros and cons of the only histological evaluation in the allocation of ECD kidneys, to compare the different experiences in United States and Europe and thus to discuss whether this tool should be used alone or included in a comprehensive clinical and histopathological evaluation. Discussion In the United States many Authors stated that the biopsy actually increases the percentage of kidney discarded and they raised questions about the importance of the biopsy in evaluating ECD kidneys for transplantation. On the other hand, the experiences of the majority of european transplant centers showed that allocating kidneys as single or dual transplant based on biopsy findings may achieve good graft and patient outcomes. Moreover, the experience of some centers as ours showed that kidneys allocated as DKT (Dual Kidney Transplant) on the basis of Remuzzi’s score could have been suitable for single transplantation suggesting the need of an adjustment of the Remuzzi Score System. Many Authors, who are in favor of histological evaluation, actually believe that a comprehensive clinical and histopathological assessment before transplantation remains necessary. Summary We lack precise national- or international-based selection criteria to guide clinicians. An adjustment of the Remuzzi Score System could be taken into consideration such as narrowing the indication for DKT from those ECD kidneys with higher scores and including the histological evaluation in a multifactor score.

Background An involvement of the appendiceal orifice as a distintive skip lesion in adults with left side ulcerative colitis (UC) has been reported. The aim of our prospective study was to evaluate, by endoscopy and histology, the prevalence of periappendiceal inflammation (PAI) in children affected by UC. Methods Fifty of 77 consecutive children undergoing total colonoscopy, who had a diagnosis of UC not extended beyond the hepatic flexure were enrolled. Results PAI was endoscopically present in 16 of 50 patients (32%) with UC. Patients were divided in 2 groups: group A included the 16 patients with PAI, whereas group B included 34 patients without PAI. We found that among the 2 groups, PAI was more frequent in patients with new diagnosis than in those with pre-existing UC (P = 0.016). At index colonoscopy, the patients of group A had a significant major extent of disease (P = 0.013). Moreover, the histologic grade of inflammation at the ascending colon was significantly higher in group A than in group B (P = 0.014). Clinical activity, measured by pediatric ulcerative colitis activity index, and use of medication did not show significant differences among groups (P = 0.464 and P = 0.723, respectively). The use of immunosuppressant was significantly higher in group A than in group B. Conclusions PAI is a frequent skip lesion in children with UC. It seems more frequent in patients with new diagnosis, and it is associated with a major extent of the disease and with a higher grade of histologic inflammation at the ascending colon.

Leiomyosarcoma is one of the most common types of soft tissue sarcoma, usually seen in deep soft tissues of the extremities and retroperitoneum. Leiomyosarcoma primarily involving the gastrointestinal tract is uncommon. This report presents a rare case of primary gastric giant cell-rich leiomyosarcoma associated with a gastrointestinal stromal tumor of the stomach in a 51-year-old man. The patient was alive with no evidence of recurrence 10 months later. To the authors’ knowledge, this association has not been previously reported.

Background Liposarcoma is the most common type of soft tissue sarcoma (STS). It is divided into five groups according to histological pattern: well-differentiated, myxoid, round cell, pleomorphic, and dedifferentiated. Dedifferentiated liposarcoma most commonly occurs in the retroperitoneum, while an intraperitoneal location is extremely rare. Only seven cases have been reported in literature. Many pathologists recognize that a large number of intra-abdominal poorly differentiated sarcomas are dedifferentiated liposarcomas. We report a case initially diagnosed as undifferentiated sarcoma that was reclassified as intraperitoneal dedifferentiated liposarcoma showing an amplification of the MDM2 gene. Case presentation A 59-year-old woman with abdominal pain and constipation was referred to the Department of Advanced Biomedical Sciences, University of Naples Federico II, Naples, Italy, in November 2012. On physical examination, a very large firm mass was palpable in the meso-hypogastrium. Computed tomography (CT) scan showed a heterogeneous density mass (measuring 10 × 19 cm) that was contiguous with the mesentery and compressed the third part of the duodenum and jejunum. At laparotomy, a large mass occupying the entire abdomen was found, adhering to the first jejunal loop and involving the mesentery. Surgical removal of the tumor along with a jejunal resection was performed because the first jejunal loop was firmly attached to the tumor. Macroscopic examination showed a solid, whitish, cerebroid, and myxoid mass, with variable hemorrhage and cystic degeneration, measuring 26 × 19 × 5 cm. Microscopic examination revealed two main different morphologic patterns: areas with spindle cells in a myxoid matrix and areas with pleomorphic cells. The case was initially diagnosed as undifferentiated pleomorphic sarcoma. Histological review showed areas of well-differentiated liposarcoma. Fluorescence in situ hybridization (FISH) analysis was performed and demonstrated an amplification of the MDM2 gene. Definitive diagnosis was intraperitoneal dedifferentiated liposarcoma. No adjuvant therapy was given, but 5 months after laparotomy, the patient presented with a locoregional recurrence and chemotherapy with high-dose ifosfamide was started. Conclusions No guidelines are available for the management of intraperitoneal dedifferentiated liposarcoma. We report this case to permit the collection of a larger number of cases to improve understanding and management of this tumor. Moreover, this study strongly suggests that poorly differentiated sarcomas should prompt extensive sampling to demonstrate a well-differentiated liposarcoma component and, if possible, FISH analysis.