Totally laparoscopic, multi-stage, restorative proctocolectomy for inflammatory bowel diseases. A prospective study on safety, efficacy and long-term results

Background: Laparoscopic ileo-pouch-anal anastomosis (IPAA) has been reported as having low morbidity and several advantages. Aims: To evaluate safety, efficacy and long-term results of laparoscopic IPAA, performed in elective or emergency settings, in consecutive unselected IBD patients. Methods: All the patients received totally laparoscopic 2-stage (proctocolectomy and IPAA \u2013 stoma closure) or 3-stage (colectomy \u2013 proctectomy and IPAA \u2013 stoma closure) procedure according to their presentation. Results: From July 2007 to July 2016, 160 patients entered the study. 50.6% underwent a 3-stage procedure and 49.4% a 2-stage procedure. Mortality and morbidity were 0.6% and 24.6%. Conversion rate was 3.75%. 8.7% septic complications were associated with steroids and Infliximab treatment (p = 0.0001). 3-stage patients were younger (p = 0.0001), with shorter disease duration (p = 0.0001), minor ASA scores of 2 and 3 (p = 0.0007), lower inflammatory index and better nutritional status (p = 0.003 and 0.0001), fewer Clavien-Dindo's grade II complications (p = .0001), reduced rates of readmission and reoperation at 90 days (p = 0.03), and shorter hospitalization (p = .0001), but with similar pouch and IPAA leakage, compared to 2-stage patients. 8 years pouch failure and definitive ileostomy were 5.1% and 3.7%. Conclusion: A totally laparoscopic approach is safe and feasible, with very low mortality and morbidity rates and very low conversion rate, even in multi-stage procedures and high-risk patients

Increase in chromogranin A- and serotonin-positive cells in pouch mucosa of patients with ulcerative colitis undergoing proctocolectomy

Background: Inflammatory bowel disease (IBD) is associated with neuroendocrine cell hyperplasia. Aims: We investigated neuroendocrine cells in J-pouches of patients with ulcerative colitis undergoing restorative proctocolectomy and ileal pouch-anal anastomosis. Methods: Sections from pouch biopsies of 17 patients and ileal biopsies of 17 active IBD patients and 16 controls were processed by immunohistochemistry for chromogranin A (CgA) and serotonin. Mucosal tryptophan hydroxylase (TpH)-1 and serotonin-selective reuptake transporter (SERT) transcripts were measured by quantitative RT-PCR. TpH-1 and SERT transcripts were detected in pouch biopsies cultured with infliximab or its isotype control, while interleukin (IL)-6 and IL-8 were measured in biopsy supernatants. Results: A significant increase in CgA-positive cells and serotonin-positive cells was observed in both pouch and IBD ileum compared to control ileum. Significantly raised transcripts of TpH-1, but not SERT, were found in IBD ileum in comparison to control ileum, with no significant difference between pouch and IBD ileum. Infliximab had no influence on ex vivo pouch expression of TpH-1 and SERT, nor on the production of IL-6 and IL-8. Conclusion: We here demonstrated neuroendocrine cell hyperplasia in pouch mucosa. Further studies are needed to clarify the pathophysiological implication of this finding

MM2-thalamic Creutzfeldt-Jakob disease-Neuropathological, biochemical and transmission studies identify a distinctive prion strain

In Creutzfeldt-Jakob disease (CJD), molecular typing based on the size of the protease resistant core of the disease-associated prion protein (PrP(Sc) ) and the M/V polymorphism at codon 129 of the PRNP gene correlates with the clinico-pathologic subtypes. Approximately 95% of the sporadic 129MM CJD patients are characterized by cerebral deposition of type 1 PrP(Sc) and correspond to the classic clinical CJD phenotype. The rare 129MM CJD patients with type 2 PrP(Sc) are further subdivided in a cortical and a thalamic form also indicated as sporadic fatal insomnia. We observed two young patients with MM2-thalamic CJD. Main neuropathological features were diffuse, synaptic PrP immunoreactivity in the cerebral cortex and severe neuronal loss and gliosis in the thalamus and olivary nucleus. Western blot analysis showed the presence of type 2A PrP(Sc) . Challenge of transgenic mice expressing 129MM human PrP showed that MM2-thalamic sporadic CJD (sCJD) was able to transmit the disease, at variance with MM2-cortical sCJD. The affected mice showed deposition of type 2A PrP(Sc) , a scenario that is unprecedented in this mouse line. These data indicate that MM2-thalamic sCJD is caused by a prion strain distinct from the other sCJD subtypes including the MM2-cortical form

Small bowel carcinomas in celiac or Crohn's disease: Distinctive histophenotypic, molecular and histogenetic patterns

Non-familial small bowel carcinomas are relatively rare and have a poor prognosis. Two small bowel carcinoma subsets may arise in distinct immune-inflammatory diseases (celiac disease and Crohn's disease) and have been recently suggested to differ in prognosis, celiac disease-associated carcinoma cases showing a better outcome, possibly due to their higher DNA microsatellite instability and tumor-infiltrating T lymphocytes. In this study, we investigated the histological structure (glandular vs diffuse/poorly cohesive, mixed or solid), cell phenotype (intestinal vs gastric/pancreatobiliary duct type) and Wnt signaling activation (β-catenin and/or SOX-9 nuclear expression) in a series of 26 celiac disease-associated small bowel carcinoma, 25 Crohn's disease-associated small bowel carcinoma and 25 sporadic small bowel carcinoma cases, searching for new prognostic parameters. In addition, non-tumor mucosa of celiac and Crohn's disease patients was investigated for epithelial precursor changes (hyperplastic, metaplastic or dysplastic) to help clarify carcinoma histogenesis. When compared with non-glandular structure and non-intestinal phenotype, both glandular structure and intestinal phenotype were associated with a more favorable outcome at univariable or stage- and microsatellite instability/tumor-infiltrating lymphocyte-inclusive multivariable analysis. The prognostic power of histological structure was independent of the clinical groups while the non-intestinal phenotype, associated with poor outcome, was dominant among Crohn's disease-associated carcinoma. Both nuclear β-catenin and SOX-9 were preferably expressed among celiac disease-associated carcinomas; however, they were devoid, per se, of prognostic value. We obtained findings supporting an origin of celiac disease-associated carcinoma in SOX-9-positive immature hyperplastic crypts, partly through flat β-catenin-positive dysplasia, and of Crohn's disease-associated carcinoma in a metaplastic (gastric and/or pancreatobiliary-type) mucosa, often through dysplastic polypoid growths of metaplastic phenotype. In conclusion, despite their common origin in a chronically inflamed mucosa, celiac disease-associated and Crohn's disease-associated small bowel carcinomas differ substantially in histological structure, phenotype, microsatellite instability/tumor-infiltrating lymphocyte status, Wnt pathway activation, mucosal precursor lesions and prognosis

Synovial sarcoma of the esophagus simulating achalasia

Synovial sarcoma is a rare malignancy occurring mainly in the extremities. Only seven cases have been described arising in the esophagus. All of them presented as a polypoid mass involving the upper third of the esophagus. A case of infiltrating synovial esophageal sarcoma simulating achalasia in a 63-year-old woman is reported. According to the literature, the location and the clinical pattern of this tumor are exceptional. The clinical features, pathologic findings, differential diagnosis, and management of this condition are discussed

Flexible argon plasma coagulation treatment of obstructive tracheal metastatic melanoma

Metastases to the tracheobronchial tree may be considered rare, and melanoma metastases to the trachea are very uncommon. We here report the case of a 61-year-old woman with metastatic melanoma to the trachea occurring 2 years after the excision of a right shoulder skin nodule. The patient underwent argon plasma coagulation (APC) recanalization of the malignant airway under flexible bronchoscopy, which led to the stable resolution of the respiratory symptoms. We also discuss the possible palliative therapeutic options for such metastases, including the APC technique. Copyright 2002, Elsevier Science (USA). All rights reserved

Histopathological differential diagnosis in inflammatory bowel diseases

In front of the suspicious diagnosis of an inflammatory bowel disease (IBD), the pathologist must have adequate and complete clinical, anamnestic, instrumental informations and, if possible, the previous histopathologic examinations. This is necessary because: the diagnosis of IBD is made with exclusion criteria, different pathologic entities may have similar macroscopic and microscopic findings and the characteristic lesions are often present in little number. The authors consider in this paper the problem of the differential diagnosis of IBD

Lethal cardiac amyloidosis: Microscopic differential diagnosis with microfibrillar cardiomyopathy in a forensic case

In a previous study, we presented a case of an elderly woman\u2019s sudden death, in which microscopic examinations showed intramyocardial eosinophilic material suspected for amyloid, but not definable as such to the classic Congo Red staining. To overcome the arisen interpretative and diagnostic difficulties, we experimentally modified the classic Congo Red staining, using a specific one for corpse. The finding of a low-intensity positivity allowed us to formulate a very likely diagnosis of occult lethal cardiac amyloidosis. However, this low-intensity positivity obtained after having applied this experimental method for the first time and in only one case, as well as the existence of the rare pathology known as microfibrillar cardiomyopathy, which may be related to the observed microscopic findings, have forced us to investigate the correctness of the diagnosis. For this purpose, we performed in-depth investigations with sodium sulphate-Alcian Blue (SAB) staining and immunohistochemistry. Thanks to them, the amyloid nature of the intramyocardial material was confirmed and has been proved not only the reliability of our experimentally modified technique, but also the appropriateness of the diagnosis previously formulated. Therefore, the supposed involvement of the microfibrillar cardiomyopathy was excluded

A Pilot Study on the Diagnosis of Fatal Electrocution by the Detection of Myocardial Microhemorrhages

In electrocutions, death may be caused by alterations in the heart conduction system provoking ventricular fibrillation. This study aims to identify histological cardiac markers of high- and low-voltage electrocution. Two groups of decedents were evaluated: group A included 14 fatalities caused by high- or low-voltage electrocution and group B (control) included 14 fatalities due to other traumatic or disease causes. Myocardial sampling with microscopic examination was performed on all the hearts using the hematoxylin and eosin and Masson's trichrome stains to investigate morphological characteristics that could indicate the damage caused by high- and low-voltage electrocutions. Interstitial myocardial hemorrhagic infiltration was the only differentiating finding, which was shown only in high-voltage electrocution. This pathological finding has not been previously reported, and it may be specific to high-voltage electrocution deaths. Further studies are warranted