Simultaneous idiopathic segmental infarction of the great omentum and acute appendicitis: a rare association

Idiopathic segmental infarction of the greater omentum is an uncommon cause of acute abdomen. The etiology is still unclear and the symptoms mimic acute appendicitis. Its presentation simultaneously with acute appendicitis is still more infrequent. We present a case of a 47-year old woman without significant previous medical history, admitted with an acute abdomen, in which the clinical diagnosis was acute appendicitis and in whom an infarcted segment of right side of the greater omentum was also found at laparotomy. As the etiology is unknown, we highlighted some of the possible theories, and emphasize the importance of omental infarction even in the presence of acute appendicitis as a coincident intraperitoneal pathological condition

A STUDY ON CORRELATION BETWEEN ELECTROMYOGRAPHICAL AND MORPHOLOGICAL FINDINGS OF BACK MUSCLES IN SCOLIOSIS, ESPECIALLY CHANGES IN THE INTRAMUSCULAR NERVE ENDINGS.

3. In the cases of neurofibromatosis with scoliosis, intramuscular nerve endings showed almost normal morphological appearances but, comparative histograms of the diameters of the end-plates in each 5μ. group showed 2 peak formations.. One of these cases recorded fibrillation voltage at the concave side showed abnormal staining of terminal filamints of the end-plates in deep back muscles at the same side. The sensory nerve endings showed perfectly preserved histological appearances. 4. In cases of rachitogenic scoliosis, motor end-plates of the back muscles were observed using the histochemical method of cholinesterase staining and vital staining with methylene blue. Histograms of the diameters of the end-plates showed a noted reduction in size and in number of units, but no apparent degenerative changes in sensory and motor endings or in terminal axons were seen. 5. In a case of discogenic scoliosis, silver impregnation was applied on the back muscles at both sides of the curvature. In the convex side, collateral branching and swelling of the nerve fibers was observed and also the motor end-plates showed an abnormality of staining in terminal arborization, but, fibrillation voltage was not recorded. 6. In congenital scoliosis, electromyographic findings failed to trace fibrillation voltage, but, terminal axons in deep muscles, showed collateral branchig and an increased terminal innervation ratio in a highly affected case. In slightly curved cases, atrophic changes, and intramuscular fibers and endings appeared almost normal. 7. In the so-called idiopathic scoliosis , about 50% had not traced fibrillation voltage in the paravertebral back muscles. Degenerative findings.of the intramuscular nerve fibers and endings were not observed, but comparative histograms of the diameters of the end -plates showed 2 peak formations in the convex side of the back muscles. Innervation ratio was not changed and sensory nerve endings showed normal appearances.8. In idiopathic scoliosis, characterised by fibrillation voltage which was traced in the paravertebral back muscles, intramuscular motor nerve endings were degenerated in deep muscles of the convex side, and disseminated muscle atrophy in the concave side. In deep muscles of the convex side, terminal axons showed collateral branching and motor endplates, club-like swelling, and 2 peak formations of the histograms of the diameters. However, sensory nerve endings were normal in appearance. These findings lead to a conclusion that changes in the motor endings observed in paralytic scoliosis differed from histological changes in the cases of idiopathic scoliosis, in which fibrillation voltage had been traced. In paralytic scoliosis, histological changes of the back muscles showed various kinds of degenerative findings, but, in cases of idiopathic scoliosis with fibrillation voltage they showed collateral branching and degenerative changes of the end-plates, respectively, in deep back muscles especially in the convex side. In other kinds of scoliosis, neuromuscular changes were largely influenced by their own basic disorders such as, degeneration of discs, abnormality of vertebral bodies, nutritional deficiencies and metablic disorders. In the cases of idiopathic scoliosis without fibrillation voltage intramuscular nerve endings were preserved in good condition, but almost all of the cases of non-paralytic scoliosis showed an abnormality of the histograms of the endplates, or atrophic changes in deep muscles. Prophylactic treatment must be employed on the back muscles for the prevention of further deformity and progression of scoliosis.An electromyographical and histological study on 20 cases of various kinds of scoliosis was carried out. In particular, a biopsy of the back muscles at the apex of the primary curve was performed using the methods of gold chloride staining, silver impregnation modified by Seto, vital staining with methylene blue, and the histochemical demonstration of cholinesterase on the subneural apparatuses of the endplates. Specimens were taken from superficial (M. longissimus dorsi) and deep (M. multifidus) back muscles of scoliosis patients and in all cases, an electromyographic fibrillation voltage was picked up under deep general anesthesia with ether. This was done from the paravertebral back muscles and a comparison of histologic and electromyographic findings was performed. The results obtained are summarized as follows : 1. Motor end-plates of normal back muscles, dissected at autopsy from adults were found to be concentrated in band-like narrow zones and situated at the mid-point of the muscle fibers. Through comparative histograms of the diameters of subneural apparatuses and those of the end-plates of gold chloride staining showed a close correlation. 2. Intramuscular endings in the cases of paralytic scoliosis showed remarkable collateral axonic sprouting and various kinds of pathological changes of the end-plates, multiple innervation, large and and small end-plates, thickened terminal filaments, failure to stain, irregular swelling, and abnormal terminal expansion. The motor endings of the musclespindle were also remarkably degenerated but sensory nerve fibers and endings remained undisturbed. In neuromuscular endings, pathological changes of the back muscles at the apex of the primary curve, displayed a distinguishing feature in a lack of uniformity in the degree of alignment and curvature, but, compared with superficial muscles, the deep muscles were significantly involved. In all cases a giant spike and fibrillation voltage was recorded from wide-spread various parts of the back muscles

Familial adenomatosis polyposis–related desmoid tumours treated with low-dose chemotherapy: results from an international, multi-institutional, retrospective analysis

[Introduction] Desmoid tumour (DT) is a locally aggressive fibroblastic proliferative disease representing the most common extraintestinal manifestation of familial adenomatosis polyposis (FAP). As data on the activity of chemotherapy in these patients are limited, we examined the outcomes of patients treated with low-dose methotrexate (MTX)+vinca alkaloids (vinorelbine or vinblastine).[Patients and methods] We retrospectively reviewed clinical and outcome data from all patients with confirmed FAP-associated DTs treated with weekly MTX+vinca alkaloids in seven European sarcoma reference centres between January 2000 and December 2018. Radiological responses were assessed using RECIST V.1.0 and V.1.1. The Kaplan-Meier method associated to the log-rank test was used to estimate and compare survival curves.[Results] We identified 37 patients (median age 29 years, range 7–44). According to RECIST, 20/37 (54.1%) patients achieved partial response (PR), 15/37 (40.5%) patients had stable disease and 2/37 (5.4%) had progressive disease as best response. Overall, the median progression-free survival (PFS) was 6.5 years (range, 0.3–12.1 years). In the subset of patients achieving PR as best response, the median PFS was not reached. In a subset of 11 patients with progressive disease offered MTX+vinca alkaloids rechallenge (after chemotherapy withdrawal following prolonged disease control), the disease control rate was 100%, resulting in a median PFS after rechallenge of 5.8 years.[Conclusions] This is the largest series on the activity of low-dose chemotherapy in patients with FAP-related DT. In this population, MTX+vinca alkaloids is an active combination, as already reported in patients with sporadic DT

Duodenal Adenomas and Cancer in MUTYH-associated Polyposis: An International Cohort Study

Although duodenal adenomas and cancer appear to occur significantly less frequently in autosomal recessive MUTYH-associated polyposis (MAP) than in autosomal dominant familial adenomatous polyposis (FAP),1 current guidelines recommend similar endoscopic surveillance for both disorders.2-4 This involves gastro-duodenoscopy starting at 25 to 35 years of age and repeated at intervals determined by Spigelman staging based on the number, size, histological type and degree of dysplasia of adenomas, and by ampullary staging. Case reports of duodenal cancers in MAP suggest that they may develop in the absence of advanced Spigelman stage benign disease and even without coexisting adenomas.1 Recent molecular analyses suggest thatMAPduodenal adenomashave a higher mutational burden than FAP adenomas and are more likely to harbor oncogenic drivermutations, such as those in KRAS.5 These apparent differences in the biology and natural history of duodenal polyposis in FAP and MAP challenge the assumption that the same surveillance should be applied in both conditions

Delphi Initiative for Early-Onset Colorectal Cancer (DIRECt) International Management Guidelines

Background & aims: Patients with early-onset colorectal cancer (eoCRC) are managed according to guidelines that are not age-specific. A multidisciplinary international group (DIRECt), composed of 69 experts, was convened to develop the first evidence-based consensus recommendations for eoCRC. Methods: After reviewing the published literature, a Delphi methodology was used to draft and respond to clinically relevant questions. Each statement underwent 3 rounds of voting and reached a consensus level of agreement of ≥80%. Results: The DIRECt group produced 31 statements in 7 areas of interest: diagnosis, risk factors, genetics, pathology-oncology, endoscopy, therapy, and supportive care. There was strong consensus that all individuals younger than 50 should undergo CRC risk stratification and prompt symptom assessment. All newly diagnosed eoCRC patients should receive germline genetic testing, ideally before surgery. On the basis of current evidence, endoscopic, surgical, and oncologic treatment of eoCRC should not differ from later-onset CRC, except for individuals with pathogenic or likely pathogenic germline variants. The evidence on chemotherapy is not sufficient to recommend changes to established therapeutic protocols. Fertility preservation and sexual health are important to address in eoCRC survivors. The DIRECt group highlighted areas with knowledge gaps that should be prioritized in future research efforts, including age at first screening for the general population, use of fecal immunochemical tests, chemotherapy, endoscopic therapy, and post-treatment surveillance for eoCRC patients. Conclusions: The DIRECt group produced the first consensus recommendations on eoCRC. All statements should be considered together with the accompanying comments and literature reviews. We highlighted areas where research should be prioritized. These guidelines represent a useful tool for clinicians caring for patients with eoCRC

Efficacy of laparoscopic Toupet fundoplication compared to endoscopic and surgical procedures for GERD treatment: a randomized trials network meta-analysis

Purpose: We aim to review and quantitatively compare laparoscopic Toupet fundoplication (LTF), Nissen fundoplication (LNF), anterior partial fundoplication (APF), magnetic augmentation sphincter (MSA), radiofrequency ablation (RFA), transoral incisionless fundoplication (TIF), proton pump inhibitor (PPI), and placebo for the treatment of GERD. A number of meta-analyses compared the efficacy of surgical and endoscopic procedures for recalcitrant GERD, but considerable debate on the effectiveness of operative strategies remains. Methods: A systematic review of MEDLINE databases, EMBASE, and Web of Science for randomized controlled trials (RCTs) comparing the aforementioned surgical and endoscopic GERD treatments was performed. Risk ratio and weighted mean difference were used as pooled effect size measures, whereas 95% credible intervals (CrI) were used to assess relative inference. Results: Thirty-three RCTs were included. Surgical and endoscopic treatments have similar RR for heartburn, regurgitation, bloating. LTF has a lower RR of post-operative dysphagia when compared to APF (RR 3.3; Crl 1.4-7.1) and LNF (RR 2.5; Crl 1.3-4.4). The pooled network meta-analysis did not observe any significant improvement regarding LES pressure and pH < from baseline. LTF, APF, LNF, MSA, RFA, and TIF had have a similar post-operative PPI discontinuation rate. Conclusion: LTF has a lower rate of post-operative dysphagia when compared to APF and LNF. The pre-post effects, such as GERD-HQRL, LES pressure, and pH <4, should be avoided in meta-analyses because results may be biased. Last, a consensus about the evaluation of GERD treatments' efficacy and their outcomes is needed

Lynch syndrome-related non-endometrioid endometrial cancer: Analysis of outcomes

Objective Women with Lynch syndrome have a risk up to 40-60% of developing endometrial cancer, which is higher than their risk of developing colorectal or ovarian cancer. To date, no data on the outcomes of patients with Lynch syndrome diagnosed with non-endometrioid endometrial cancer are available. The goal of this study was to evaluate the outcome of patients with Lynch syndrome diagnosed with non-endometrioid endometrial cancer. Methods Data from consecutive patients diagnosed with Lynch syndrome and with a histological diagnosis of non-endometrioid endometrial cancer were retrospectively collected in two referral institutes in Italy. A case-control comparison (applying a propensity matching algorithm) was performed in order to compare patients with proven Lynch syndrome and controls. Inclusion criteria were: (a) histologically-proven endometrial cancer; (b) detection of a germline pathogenic variant in one of the MMR genes; (c) adequate follow-up. Only carriers of pathogenic or likely pathogenic variants (ie, class 5 and 4 according to the InSiGHT classification) were included in the study. Survival outcomes were assessed using KaplanMeier and Cox models. Results Overall, 137 patients with Lynch syndrome were collected. Mean patient age was 49.2 (10.9) years. Genes involved in the Lynch syndrome included MLH1, MSH2, and MSH6 in 43%, 39%, and 18% of cases, respectively. The study population included 27 patients with non-endometrioid endometrial cancer, who were matched 1:2 with patients with sporadic cancers using a propensity matching algorithm. After a median follow-up of 134 months (range 1-295), 2 (7.4%) of the 27 patients developed recurrent disease (3 and 36 months) and subsequently died of disease (7 and 91 months). Patients diagnosed with Lynch syndrome experienced better disease-free survival (HR 7.86 (95% CI 1.79 to 34.5); p=0.006) and overall survival (HR 5.33 (95% CI 1.18 to 23.9); p=0.029) than controls. Conclusions Non-endometrioid endometrial cancer occurring in patients with Lynch syndrome might be associated with improved oncologic outcomes compared with controls. Genetic/molecular profiling should be investigated in order to better understand the mechanism underlying the prognosis