Segmental filiform-polyposis in a treated case of ulcerative colitis, colon: An unusual presentation raising a doubt of primary diagnosis?

Inflammatory bowel disease is broadly classified into Crohn's disease and ulcerative colitis. The standard criteria to distinguish between the two is the manner of the involvement of the bowel, with the former showing classical skip lesions and the latter having continuous involvement of the colon, most commonly affecting the rectum. However, some cases exhibit overlapping features. Herein, we report a treated case of ulcerative colitis presenting with patchy involvement of the colon in the form of peculiar segmental filiform polyposis spanned abruptly by an intervening normal mucosa. The clinico-radiologically suspicion of carcinoma colon with Crohn's colitis was considered. The clinicians and pathologists must be aware of such atypical presentations and should not be misled to change the diagnosis from ulcerative colitis to Crohn's colitis on the post-treatment resection specimens or endoscopic biopsies solely in view of the patchy filiform polyposis (FP), which poses a drastic impact on the patient's management.

Pólipos adenomatosos en familiares de primer grado de pacientes con cáncer del colon

Introducción: El cáncer colorectal constituye en la actualidad la segunda neoplasia maligna más frecuente. La mayoría son esporádicos, otra pequeña proporción corresponde a formas hereditarias. Sin embargo, se estima que en un 15 % a 20 % de casos pueden existir un componente hereditario asociado. Los familiares de primer grado de pacientes con cáncer colorrectal, constituyen un universo de mayor riesgo que la población general de padecer esta enfermedad, por lo que se recomienda el cribado en estos individuos. Objetivo: Determinar la frecuencia de pólipos adenomatosos en individuos con familiares de primer grado diagnosticados con cáncer de colon. Métodos: Se realizó un estudio descriptivo, de corte transversal, que incluyó a 126 adultos con familiares de primer grado de consanguinidad con cáncer de colon, a los que se les realizó colonoscopia en el Instituto de Gastroenterología entre diciembre de 2019 y diciembre 2021. Se describen las características de los pólipos adenomatosos encontrados. Resultados: La media para la edad fue de 55,9 ± 10,6, predominaron las mujeres. Se encontraron pólipos adenomatosos; 27 eran sésiles y 12 pediculados; en 26 (66,7 %), el tamaño era menor de 10 mm. La mayoría de los pólipos, fueron del tipo tubular. Se observó diversidad en cuanto a la localización de las lesiones, sin que existiera un predominio en ningún segmento anatómico. Conclusiones: Lesiones precursoras del cáncer colorrectal, como los pólipos adenomatosos, son frecuentes en individuos asintomáticos con familiares de primer grado de consanguinidad que padecieron esta neoplasia maligna.

Introduction : Colorectal cancer is currently the second most frequent neoplasm. Most of them are sporadic, another small proportion corresponds to hereditary forms. However, it is estimated that in 15-20% of cases there may be an associated hereditary component. First-degree relatives of patients with colorectal cancer constitute a universe with a higher risk of developing this disease than that of the general population, so screening is recommended in these individuals. Objective : To determine the frequency of adenomatous polyps in individuals with first-degree relatives diagnosed with colon cancer. Methods : A descriptive, cross-sectional study was carried out, including 126 adult relatives with first-degree blood relatives with colon cancer, who underwent colonoscopy at the Institute of Gastroenterology between December 2019 and December 2021. The characteristics of the adenomatous polyps found are described. Results : The mean for age was 55.9 ± 10.6, women predominated: 94 (74.6 %). Thirty-nine (30.9 %) adenomatous polyps were found; 27 (69.2 %) were sessile and 12 (30.7 %) pedunculated; in 26 (66.7 %) the size was less than 10 mm. The majority, 37 (94.8 %), were of the tubular type. Diversity was observed as to the location of the lesions, with no predominance in any anatomical segment. Conclusions : Precursor lesions of colorectal cancer, such as adenomatous polyps, are common in asymptomatic individuals with first-degree relatives who have had this malignancy.

Intra-abdominal desmoid tumors in familial adenomatous polyposis: How much do clinical and surgical variables interfere with their development?

Abstract Objective: Familial Adenomatous Polyposis is a complex hereditary disease that exposes the carrier to a great risk of Colorectal Cancer (CRC). After prophylactic surgery, intra-abdominal desmoid tumors are known to be one the most important cause of death. Therefore, recognition of increased-risk patients and modification of operative strategy may be crucial. Aim: The objective of this study was to estimate the desmoid tumor risk in relation to various surgical and clinical variables. Methods: Patients who had undergone polyposis since 1958 were included in the study. After exclusion criteria were met, those who had developed desmoid tumors were selected to undergo further evaluation. Results: The study revealed that the risk of developing desmoid tumors was associated with various factors such as sex ratio, colectomy, and reoperations. On the other hand, the type of surgery, family history, and surgical approach did not affect the risk of developing desmoid tumors. The data collected from 146 polyposis patients revealed that 16% had desmoid polyps. The sex ratio was 7:1, and the median age at colectomy was 28.6 years. Family history, multiple abdominal operations, and reoperations were some of the characteristics that were common in desmoid patients. Conclusion: Recognition of clinical (female sex) and surgical (timing of surgery and previous reoperations) data as unfavorable variables associated with greater risk may be useful during the decision-making process.

Caracterización clínica y epidemiológica de los pacientes con cáncer de colon / Clinical and epidemiological characterization of patients with colon cancer

INTRODUCCIÓN. El cáncer de colon es una neoplasia del tubo digestivo considerada una de las más frecuentes en ambos sexos y que predomina en adultos mayores. OBJETIVO. Describir las características clínicas y epidemiológicas de los pacientes con cáncer de colon. MATERIALES Y MÉTODOS. Estudio observacional, descriptivo, retrospectivo. Población de 1 601 y muestra de 210 datos de Historias Clínicas Electrónicas de pacientes diagnosticados con cáncer de colon, atendidos por la Unidad de Oncología del Hospital de Especialidades Carlos Andrade Marín de la ciudad de Quito en el periodo enero de 2016 a diciembre de 2019. Criterios de inclusión: diagnóstico confirmado de Cáncer de Colon, edad igual o mayor a 18 años, y disponer de todos los datos clínicos requeridos en el estudio. Se utilizó el método de muestreo probabilístico con lo que se estimó una proporción para el estudio con un intervalo de confianza del 95%, un margen de error del 5% y una frecuencia esperada del 3%, de donde se obtuvo una muestra ajustada al 10% de pérdidas. El procesamiento de datos se realizó en los programas Microsoft Excel versión 16 y el Statistical Package for Social Sciences versión 24. RESULTADOS. La mayor presentación fue en adultos mayores de 50 años, con una relación 1:1 en cuanto a sexo, y en la procedencia, se ubicó mayoritariamente en la población de la región Sierra; las personas con una actividad económica de tipo profesional fueron las más afectadas; en lo que se refiere a los antecedentes se encontró mayor relación en los personales y dentro de estos los pólipos; no hubo relación con los antecedentes quirúrgicos ni familiares. El síntoma de debut más prevalente fue el dolor abdominal; la mayoría fueron sometidos a colonoscopia; predominó la lateralidad derecha y el tipo histológico principalmente identificado fue el adenocarcinoma. CONCLUSIÓN. No se observó relación estadísticamente significante entre estadíos, evolución y tratamientos instaurados, lo que pudo estar influenciado por el muestreo al azar; y que el 53,30% de los pacientes aún se encuentra en controles.

INTRODUCTION. Colon cancer is a neoplasm of the digestive tract considered one of the most frequent in both sexes and predominantly in older adults. OBJECTIVE. To describe the clinical and epidemiological characteristics of patients with colon cancer. MATERIALS AND METHODS. Observational, descriptive, retrospective study. Population of 1 601 and sample of 210 data from Electronic Medical Records of patients diagnosed with colon cancer, attended by the Oncology Unit of the Hospital de Especialidades Carlos Andrade Marín of the city of Quito in the period January 2016 to December 2019. Inclusion criteria: confirmed diagnosis of Colon Cancer, age equal to or older than 18 years, and having all the clinical data required in the study. The probability sampling method was used with which a proportion was estimated for the study with a confidence interval of 95%, a margin of error of 5% and an expected frequency of 3%, from which a 10% loss adjusted sample was obtained. Data processing was performed in Microsoft Excel version 16 and Statistical Package for Social Sciences version 24. The greatest presentation was in adults over 50 years of age, with a 1:1 ratio in terms of sex, and in terms of origin, it was mainly located in the population of the Sierra region; people with a professional economic activity were the most affected; in terms of history, a greater relationship was found in personal history and within these, polyps; there was no relationship with surgical or family history. The most prevalent debut symptom was abdominal pain; the majority underwent colonoscopy; right laterality predominated and the histological type mainly identified was adestatistically significant relationship was observed between stages, evolution and treatment, which could be influenced by random sampling; and that 53,30% of the patients are still in controls.

Peutz-Jeghers syndrome in pediatric patients: experience in a tertiary care institution in Mexico / Síndrome de Peutz-Jeghers en pacientes pediátricos: experiencia en una institución de tercer nivel de atención en México

Abstract Background: Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant inherited disease characterized by the development of polyps in the gastrointestinal tract, mucocutaneous pigmentation, and the risk of developing malignant neoplasms. This study aimed to analyze the epidemiological, clinical, and histopathological data of patients with PJS treated in a tertiary pediatric hospital. Methods: We conducted a retrospective observational study to describe the epidemiological, clinical, endoscopic, and histological characterization of patients with PJS treated in a tertiary pediatric hospital in Mexico. Results: We included 13 cases with a male-female ratio of 1.16:1. Abdominal pain was the main reason for consultation, followed by rectorrhagia. Patients showed mucocutaneous pigmentation and polyps in the gastrointestinal tract, frequently of the hamartomatous type, although inflammatory polyps, follicular hyperplasia, and adenomatous polyps were also found. Among the complications, there was a high prevalence of emergency surgery secondary to abdominal obstructive processes, the main reason for first-time consultation in these patients. Conclusions: The main clinical manifestations were mucocutaneous pigmentation, abdominal pain, and rectorrhagia. PJS should be included in the differential diagnosis in the presence of intestinal obstruction. The diagnosis of PJS should not be excluded if hamartomatous polyps are not evident on the first endoscopy. Nutritional assessment should be included due to the risk of presenting some degree of malnutrition.

Resumen Introducción: El síndrome de Peutz-Jeghers es una enfermedad hereditaria autosómica dominante poco frecuente, caracterizada por el desarrollo de pólipos en el tubo digestivo, pigmentación mucocutánea y riesgo de desarrollar neoplasias malignas. El objetivo de este estudio fue analizar los datos epidemiológicos, clínicos e histopatológicos de los pacientes con SPJ atendidos en un hospital pediátrico de tercer nivel. Métodos: Se llevó a cabo un estudio observacional retrospectivo, para describir las características epidemiológicas, clínicas, endoscópicas e histopatológicas de los pacientes con SPJ atendidos en un hospital pediátrico de tercer nivel de atención en México. Resultados: Se recopilaron 13 casos con una relación masculino-femenino de 1.16:1. El dolor abdominal fue el principal motivo de consulta, seguido por rectorragia. Los pacientes presentaban pigmentación mucocutánea y pólipos en el tubo digestivo, la mayoría del tipo hamartomatoso, aunque también se hallaron pólipos inflamatorios, hiperplasia folicular y adenomatosos. Dentro de las complicaciones se encontró una alta prevalencia de cirugías de emergencia secundarias a procesos obstructivos abdominales, motivo principal de consulta de primera vez en estos pacientes. Conclusiones: Las principales manifestaciones clínicas fueron pigmentación mucocutánea, dolor abdominal y rectorragia. Ante un cuadro de obstrucción intestinal se debe considerar el SPJ en el diagnóstico diferencial. No se debe excluir el diagnóstico de SPJ si no se evidencian pólipos hamartomatosos en la primera endoscopia. Se debe incluir la valoración nutricional por el riesgo de presentar algún grado de desnutrición.

Prevalence of respiratory epithelial adenomatoid hamartomas (REAH) associated with nasal polyposis: an epidemiological study - how to diagnose

Abstract Objectives: To highlight the prevalence of respiratory epithelial adenomatoid hamartomas in the olfactory cleft of patients with nasal polyposis. To demonstrate characteristics indicative of hamartoma on the CT scans of paranasal sinuses during surgery and in histopathological exams. Methods: Cross-sectional study carried out in Hospital das Clínicas da UFMG and Núcleo de Otorrino BH. We performed 114 nasal endoscopic surgeries for polyposis, between February 2015 and November 2019. We assessed the olfactory cleft width in all preoperative CT scans. Upon seeing an indication of hamartoma on the CT scan, we took a tissue sample from the olfactory cleft during the surgery and sent for histopathological exam. We referred the samples to a pathologist experienced in the anatomopathological diagnosis of respiratory epithelial adenomatoid hamartomas. Results: Of the 114 patients with polyposis, 54 (47.4%) had olfactory cleft enlargement and, 100% of them had tissue with a dense and hardened polypoid aspect, with a slight cerebri-form appearance in this region during the surgery. Histology confirmed a respiratory epithelial adenomatoid hamartoma. Conclusion: This observation suggests that the presence of hamartomas in polyposis is common, but underdiagnosed. Level of evidence: Step 3 (Level 3).

Symptoms Less Status of a Case of Bilateral Nasal Polyposis: A Case Report

Nasal Polyposis (NP) is not simply mucosa oedema, but rather grape shaped, smooth, soft, freely mobile mucosal swellings that are often visible on anterior rhinoscopy. Polyps arise from the lateral wall of nose. NP may be present without clinically significant sinus disease and vice versa. In Ayurveda, polyposis can be understood with Nasa Arsha. Modern management includes antibiotic, systemic and topical corticosteroids, decongestants and surgery. And lot of chances for recurrence after surgery especially in Ethmoidal polyps. In Ayurveda, various treatment modalities have been proposed for the treatment of Arsha as Shastra karma (operative procedure), Kshara karma (applying some alkaline drugs), Agni karma (cauterization) and Bhaishaja (conservative/medical treatment). The reported case revealed that B/L ethmoidal polyps are high recurrence rate after surgery and can be managed with Ayurvedic medicine to give sustained relief from symptoms.

Proctocolectomía total radical laparoscópica por poliposis familiar adenomatosa colorrectal asociada a cáncer de colon izquierdo / Laparoscopic radical total proctocolectomy by colorrectal adenomatous family polyposis associated with left colon cancer

Resumen Objetivo: Presentar el caso de una paciente mujer adulta, joven, con poliposis múltiple, asociado a cáncer colorrectal, evaluando su manejo quirúrgico oncológico. Materiales y Método: Datos e imágenes recopilados de la historia clínica del Hospital de Alta Complejidad Virgen de la Puerta (HACVP) EsSalud - La Libertad. Resultado: Mujer de 33 años que debuta con dolor abdominal en hemiabdomen izquierdo, más deposiciones con sangre. El hallazgo colonoscópico encuentra múltiples pólipos elevados, planos y sésiles en todo el colon, recto y ano compatible con displasias de alto y bajo grado; a nivel de colon izquierdo se halla lesión exofítica, estenosante cuyo resultado fue adenocarcinoma infiltrante moderadamente diferenciado. Por el gran riesgo de malignidad en todo el intestino grueso, incluyendo canal anal, se le realiza proctocolectomía total laparoscópica más ileostomía terminal. Discusión: La poliposis adenomatosa familiar (PAF) es un síndrome cuyo abordaje quirúrgico va desde una colectomía total con anastomosis ileorrectal, proctocolectomía con ileostomía terminal y proctocolectomía total con Pouch y anastomosis ileoanal. Conclusión: Individualizar el caso, sobre la mejor opción quirúrgica a adoptar para un adecuado manejo oncológico.

Aim: To present the case of a young adult female patient with multiple polyposis associated with colorectal cancer, evaluating her surgical oncological management. Materials and Method: Data and images collected from the clinical history of the Hospital de Alta Complejidad "Virgen de la Puerta" (HACVP) EsSalud - La Libertad. Result: 33-year-old woman presenting with abdominal pain in the left hemiabdomen plus bloody stools. At colonoscopy multiple raised, flat and sessile polyps throughout the colon, rectum, and anus compatible with high-and low-grade dysplasias; an exophytic, stenosing lesion was found in the left colon, the result of which was moderately differentiated infiltrating adenocarcinoma. Due to the great risk of malignancy in the entire large intestine including the anal canal, a total laparoscopic proctocolectomy plus terminal ileostomy was performed. Discussion: Familial adenomatous polyposis (FAP) is a syndrome whose surgical approach ranges from a total colectomy with ileorectal anastomosis, proctocolectomy with terminal ileostomy and total proctocolectomy with Pouch and ileoanal anastomosis. Conclusión: Individualize the case, regarding the best surgical option to adopt for an adequate oncological management.

Lymphomatous Polyposis: Case Report and Literature Review / Poliposis linfomatosa: reporte de caso y revisión de la literatura

Abstract Lymphomatous polyposis (LP) is the endoscopic feature of primary gastrointestinal mantle cell lymphoma (MCL), a rare type of B-cell non-Hodgkin's lymphoma (NHL) and a typical but rare endoscopic pattern of gastrointestinal tract involvement (GIT) by nodal MCL. We present the case of a 62-year-old man with nodal MCL, with LP as a manifestation of GIT, and review the literature.

Resumen La poliposis linfomatosa (PL) es la característica endoscópica del linfoma de células del manto (LCM) gastrointestinal primario, un tipo infrecuente de linfoma no Hodgkin (LNH) de células B, así como un patrón endoscópico típico, pero infrecuente, del compromiso del tracto gastrointestinal (TGI) por LCM nodal. Presentamos el caso de un hombre de 62 años con LCM nodal, con PL como manifestación del compromiso gastrointestinal, y realizamos una revisión de la literatura.

Effet de la prémédication par des corticoïdes systémiques sur la chirurgie endonasale de la polypose naso-sinusienne / Effect of premedication with systemic corticosteroids on endonasal surgery for naso-sinus polyposis

Déterminer si une cure préopératoire de corticoïdes systémiques réduirait le saignement au cours de la chirurgie endonasale de la polypose naso-sinusienne (PNS) et améliorerait la visualisation du champ opératoire. Méthodes: Étude prospective randomisée incluant des adultes (>18 ans) atteints de PNS primitive résistante au traitement médical et qui vont bénéficier d'une chirurgie endoscopique endonasale. Ces patients sont randomisés en deux groupes: un groupe préparé par des comprimés de prednisolone 20mg, à la posologie de 0.5mg/Kg/j pendant 7 jours avant la chirurgie et un groupe non préparé. Les critères d'évaluation sont: la perte sanguine avec la comparaison des hémoglobines (Hb) pré et postopératoires, le temps opératoire et la visibilité du champ opératoire évaluée par le chirurgien selon le score de Boezaart. Résultats: Nous avons inclus 32 patients. Dix-sept patients dans le groupe non préparé et 15 patients dans le groupe préparé. Les deux groupes étaient comparables sur le plan épidémiologique, clinique et paraclinique (p>0,05). La perte sanguine moyenne était plus importante dans le groupe non préparé (700±487 ml versus 500±480 ml) mais sans différence significative (p=0,38). La différence était statistiquement non significative (p=0,24) entre les 2 groupes pour l'Hb postopératoire et pour la différence d'Hb préopératoire - Hb post opératoire. Le temps opératoire était plus court dans le groupe préparé (80±24 min versus 90±64 min) sans différence statistiquement significative (p=0,11). Le score de Boezaart était comparable (médiane=3) (p=0,29). Conclusion: Cet essai clinique n'a pas démontré que l'administration d'une cure préopératoire de corticothérapie orale peut réduire considérablement la perte de sang au cours de la chirurgie de la PNS et améliorer la visualisation du champ opératoire

Smell disorders evolution after chronic rhinosinusitis with polyps surgery

: The effects of chronic rhino sinusitis with polyps (CRSWP) surgery on smell symptoms have not been sufficiently studied. The aim of this study was to evaluate the impact of CRSWP surgery on smell symptoms over short and long-term follow-up and to identify the factors that might influence their evolution. Patients and methods: This was a retrospective study about 184 patients operated endoscopically for CRSWP. In post operative period, long-term local steroids were prescribed systematically. The sense of smell was evaluated preand post-operatively according to a subjective score (1: good smell, 2: hyposmia, 3: anosmia). Some factors, related to the patient, the CRSWP and the treatment, were tested in order to identify predictors of smell outcome after surgery.. Results: Before surgery, the anosmia and the hyposmia were noted in 90.8% and in 8.7% of cases, respectively. At six months after surgery, the improvement of olfactory score was significant: 84% of patient had the score 1 compared with 0.5% preoperatively (p < 0.0001). This improvement was maintained during the 2 first years and decreased significantly at 3 years, although an average delay of polyps recurrence was 23.4 months. Among the factors studied in our series, only the observance of postoperative corticosteroids was retained as a predictor of smell recovery after surgery (p = 0,011). Conclusion: CRSWP surgery can significantly improve the smell sense, especially during the two first years. This effect can be sustainable if good post operative compliance for local corticosteroid are achieved

Clinical and endoscopic features and endoscopic treatment efficacy of cap polyposis / 中华消化内镜杂志

To investigate the clinical and endoscopic characteristics and endoscopic treatment efficacy of cap polyposis, data of 14 patients (56 polyps) who were histologically diagnosed as having cap polyposis after endoscopic submucosal dissection (ESD) or endoscopic mucosal resection (EMR) in Beijing Friendship Hospital from June 2017 to February 2021 was retrospectively analyzed. Of the 14 patients, 8 were males and 6 were females. The age ranged from 14 to 74 years, including 7 cases of <60 years old and 7 cases of ≥60 years old. 7 patients (50.0%) had clinical manifestations. Four cases had multiple polyps and 10 cases (71.4%) had single polyps. There were 42 polyps (75.0%) located in the rectum, 13 (23.2%) in the sigmoid colon and 1 in the transverse colon. According to the classification of Yamada, 44 polyps (78.6%) were type Ⅰ, 3 polyps were type Ⅱ, 5 polyps were type Ⅲ and 4 polyps were type Ⅳ. Under endoscopy, there were 41 polyps (73.2%) with obvious white cap-like coverings on the surface and 23 polyps with obvious hyperemia and redness on the mucosa, 8 of which were both visible. Two cases were treated with ESD and 12 cases were treated with EMR, all of which were completely excised. No bleeding, perforation, infection or other complications occurred during and after operation. The clinical symptoms of 7 patients were relieved. During the follow-up period, 11 cases (78.6%) completed colonoscopy, and no polyp recurrence was found. In conclusion, there is no gender or age difference in patients of cap polyposis. It is usually single and located in the rectum and sigmoid colon with Yamada type Ⅰ. The surface of lesions is mostly covered with white cap. Patients may have no obvious clinical symptoms. Treatment of ESD and EMR is safe and effective for cap polyposis.

Current management status of hereditary colorectal cancer / 中华胃肠外科杂志

Hereditary colorectal cancer accounts for approximately 5% of all colorectal cancer cases, mainly including familial adenomatous polyposis and Lynch syndrome. Total proctocolectomy plus ileal pouch-anal anastomosis and total colectomy plus ileorectal anastomosis are two major procedures for familial adenomatous polyposis, however, the exact impact of these two procedures on surgical efficacy, oncologic efficacy as well as functional results still remains uncertain. Segmental colectomy and total colectomy are two major procedures for Lynch syndrome, each of them both has advantages and disadvantages, and there still lacks a consensus about the optimal strategy because of the nature of retrospective study with a relatively insufficient evidence support. As a result, we would make a review about the current surgical treatment status and future perspectives of hereditary colorectal cancer.

Pancreatic solid-pseudopapillary neoplasm in patients with familial adenomatous polyposis / Neoplasia sólida pseudopapilífera pancreática em pacientes com polipose adenomatosa familiar

ABSTRACT BACKGROUND: Solid pseudopapillary neoplasm of the pancreas is an uncommon pancreatic tumor, which is more frequent in young adult women. Familial adenomatous polyposis is a genetic condition associated with colorectal cancer that also increases the risk of developing other tumors as well. AIM: The aim of this study was to discuss the association of familial adenomatous polyposis with solid pseudopapillary neoplasm of the pancreas, which is very rare. METHODS: We report two cases of patients with familial adenomatous polyposis who developed solid pseudopapillary neoplasm of the pancreas of the pancreas and were submitted to laparoscopic pancreatic resections with splenic preservation (one male and one female). RESULTS: ß-catenin and Wnt signaling pathways have been found to play an important role in the tumorigenesis of solid pseudopapillary neoplasm of the pancreas, and their constitutive activation due to adenomatous polyposis coli gene inactivation in familial adenomatous polyposis may explain the relationship between familial adenomatous polyposis and solid pseudopapillary neoplasm of the pancreas. CONCLUSION: Colonic resection must be prioritized, and a minimally invasive approach is preferred to minimize the risk of developing desmoid tumor. Pancreatic resection usually does not require extensive lymphadenectomy for solid pseudopapillary neoplasm of the pancreas, and splenic preservation is feasible.

RESUMO RACIONAL: A neoplasia sólida pseudopapilífera do pâncreas é um tumor pancreático incomum, mais frequente em mulheres jovens. A polipose adenomatosa familiar, por sua vez, é uma condição genética associada a câncer colorretal e que também aumenta o risco de desenvolvimento de outros tumores. OBJETIVOS: Discutir a associação entre polipose adenomatosa familiar e neoplasia sólida pseudopapilífera, que é bastante rara. MÉTODOS: Reportamos dois casos de pacientes com polipose adenomatosa familiar, um homem e uma mulher, que desenvolveram neoplasia sólida pseudopapilífera do pâncreas e foram submetidos a ressecção laparoscópica com preservação esplênica. RESULTADOS: As vias de sinalização da ß-catenina e Wnt tem um papel importante na tumorigênese da neoplasia sólida pseudopapilífera, e sua ativação constitutiva devido a inativação do gene adenomatous polyposis coli na polipose adenomatosa familiar pode explicar a relação entre polipose adenomatosa familiar e neoplasia sólida pseudopapilífera. CONCLUSÕES: A ressecção do cólon deve ser priorizada, com preferência pela abordagem minimamente invasiva para minimizar o risco de desenvolvimento de tumor desmoide. A ressecção pancreática geralmente não requer linfadenectomia extensa para neoplasia sólida pseudopapilífera, portanto, a preservação esplênica é factível.

Smell disorders evolution after chronic rhinosinusitis with polyps surgery

The effects of chronic rhino sinusitis with polyps (CRSWP) surgery on smell symptoms have not been sufficiently studied. The aim of this study was to evaluate the impact of CRSWP surgery on smell symptoms over short and long-term follow-up and to identify the factors that might influence their evolution. Patients and methods: This was a retrospective study about 184 patients operated endoscopically for CRSWP. In post operative period, long-term local steroids were prescribed systematically. The sense of smell was evaluated preand post-operatively according to a subjective score (1: good smell, 2: hyposmia, 3: anosmia). Some factors, related to the patient, the CRSWP and the treatment, were tested in order to identify predictors of smell outcome after surgery.. Results: Before surgery, the anosmia and the hyposmia were noted in 90.8% and in 8.7% of cases, respectively. At six months after surgery, the improvement of olfactory score was significant: 84% of patient had the score 1 compared with 0.5% preoperatively (p < 0.0001). This improvement was maintained during the 2 first years and decreased significantly at 3 years, although an average delay of polyps recurrence was 23.4 months. Among the factors studied in our series, only the observance of postoperative corticosteroids was retained as a predictor of smell recovery after surgery (p = 0,011). Conclusion: CRSWP surgery can significantly improve the smell sense, especially during the two first years. This effect can be sustainable if good post operative compliance for local corticosteroid are achieved.

AXIN2-associated adenomatous colorectal polyposis

Abstract: Introduction Most cases of colorectal cancer (CRC) occur sporadically; however, ~3% to 6% of all CRCs are related to inherited syndromes, such as Lynch syndrome and familial adenomatous polyposis (FAP). The adenomatous polyposis coli (APC) andmutY DNA glycosylase (MUTYH) germline mutations are the main genetic causes related to colorectal polyposis. Nevertheless, in many cases mutations in these genes have not been identified. The aim of the present case report is to describe a rare case of genetic colorectal polyposis associated with the axis inhibition protein 2 (AXIN2) gene. Case Report: The first colonoscopy screening of a 61-year-old male patient with no known family history of CRC revealed ~ 50 colorectal polyps. A histological evaluation of the resected polyps showed low-grade tubular adenomas. Germline genetic testing through a multigene panel for cancer predisposition syndromes revealed a pathogenic variant in the AXIN2 gene. In addition to colorectal polyposis, the patient had mild features of ectodermal dysplasia: hypodontia, scant body hair, and onychodystrophy. Discussion: The AXIN2 gene acts as a negative regulator of the Wnt/β -catenin signaling pathway, which participates in development processes and cellular homeostasis. Further studies are needed to support the surveillance recommendations for carriers of the AXIN2 pathogenic variant. (AU)

Interferon-α action in cytokine profile in eosinophilic nasal polyp cultures / Ação do interferon-α sobre o perfil de citocinas em culturas de pólipos nasais eosinofílicos

Abstract Introduction Chronic rhinosinusitis is currently classified into two types: chronic rhinosinusitis without nasal polyps and chronic rhinosinusitis with nasal polyps. In the West, approximately 80% of chronic rhinosinusitis with nasal polyps cases are characterized by a predominantly eosinophilic cell infiltrate and a Th2 cytokine pattern. Objective To evaluate the effect of Interferon-α on cytokine levels of the eosinophilic nasal polyp cell culture supernatant. Methods Cell cultures were performed based on nasal polypoid tissue samples collected from 13 patients with eosinophilic chronic rhinosinusitis with nasal polyps. Polyps were considered eosinophilic according to the histopathological examination. Cell cultures were stimulated with 3000 IU of interferon-α. Before and after the stimulus, concentrations of Interferon-γ, tumor necrosis factor αand IL 2, 4, 6 and 10, using cytometric bead array, were assessed. Results Cell samples from eosinophilic nasal polyps from 13 patients were included in the study. Twenty-four hours after interferon-α stimulation, eosinophilic nasal polyp culture supernatants showed significantly decreased IL-4 concentrations and increase in interferon-γ, IL-10 and IL-6 concentrations compared to controls. There were no significant differences in tumor necrosis factor -α and IL-2 concentrations. Conclusion We demonstrated that interferon-α in vitro alters the pattern of cytokines in cell cultures of eosinophilic nasal polyps. Analysis of these alterations suggests that interferon-α promotes a rebalancing of inflammatory profiles in cell cultures, favoring the expression of Th1 and regulatory cytokines over Th2 cytokines.

Resumo Introdução A rinossinusite crônica, atualmente, é classificada em dois tipos: Rinossinusite Crônica sem Pólipos Nasais (RSCsPN) e Rinossinusite Crônica com Pólipos Nasais (RSCcPN). No Ocidente, cerca de 80% dos casos de RSCcPN caracterizam-se por um infiltrado celular predominantemente eosinofílico e um padrão de citocinas Th2. Objetivo Avaliar o efeito do Interferon-alpha nos níveis de citocinas do sobrenadante de culturas celulares de pólipos nasais eosinofílicos. Método Foram feitas culturas celulares a partir de amostras de tecido polipoide nasal coletadas de 13 pacientes com RSCcPN eosinofílica. Os pólipos eram considerados eosinofílicos segundo exame histopatológico. As culturas celulares foram estimuladas com 3000 UI de IFN-α. Antes e após tal estímulo, foram avaliadas, no sobrenadante das culturas celulares, as concentrações do Interferon-γ (IFN-γ), do Fator de Necrose Tumoral alfa (TNF-α) e das Interleucinas (IL) 2, 4, 6 e 10, usou-se o Cytometric Bead Array. Resultados Foram incluídas no estudo amostras celulares dos pólipos nasais eosinofílicos de 13 pacientes. Vinte e quatro horas após o estímulo com IFN-α, os sobrenadantes das culturas dos pólipos nasais eosinofílicos apresentaram, de forma significante, diminuição da concentração de IL-4 e aumento das concentrações de IFN-γ, IL-10 e IL-6, em relação ao controle. Não houve diferença significante nas concentrações de TNF-α e IL-2. Conclusão Demonstramos que o IFN-α, in vitro, altera o padrão de citocinas nas culturas celulares de pólipos nasais eosinofílicos. A análise do conjunto dessas alterações sugere que o IFN-α promove, nas culturas celulares, um rebalanceamento dos perfis inflamatórios, favorece a expressão de citocinas Th1 e regulatórias, em detrimento de citocinas do padrão Th2.

Montelukast Has no Impact on the Systemic Production of TGFβ-1 in Patients with Nasal Polyposis Associated with Aspirin Intolerance

Abstract Introduction Nasal polyposis is a disease characterized by a mechanical dysfunction of the nasal mucosa, closely related to the unique makeup of its extracellular matrix, which develops as the result of an anomalous tissue remodeling process. Transforming growth factor beta 1 (TGF-β1) is reduced not only in the nasal polypoid tissue, but also in the plasma of aspirin-intolerant patients. These patients exhibit an imbalance in the production of eicosanoids characterized by an increase in leukotrienes. Thus, it is important that the relationship between the production of leukotrienes and TGF-β1 be assessed. Objective To evaluate the effects of the cysteinyl leukotriene (CysLT) receptor antagonist montelukast on the systemic production of TGF-β1 in patients with nasal polyposis, with or without concomitant aspirin intolerance. Methods The sample comprised 48 individuals with diagnosis of nasal polyposis and 15 healthy controls for comparison of the baseline TGF-β1 levels in the peripheral blood and after treatment with CysLT receptor antagonist montelukast in the nasal-polyposis group. Results There was no difference in the change in TGF-β1 levels after the treatment with montelukast in the subgroup of patients with polyposis and asthma (p = 0.82) and in the subgroup with polyposis, asthma, and aspirin intolerance (p = 0.51). Conclusion we found no impact of the therapy with a leukotriene receptor blocker on the production of TGF-β1, making the antileukotriene therapy a highly questionable choice for the treatment of nasal polyposis, particularly from the standpoint of seeking to modify the remodeling process in this disease.

Research advances in the association between nonalcoholic fatty liver disease and colorectal adenomatous polyps / 临床肝胆病杂志

Nonalcoholic fatty liver disease (NAFLD) and colorectal adenomatous polyps are closely associated with the various components of metabolic syndrome. This article summarizes the recent studies on the association between NAFLD and colorectal adenomatous polyps, and the results show that NAFLD is associated with an increased risk of colorectal adenomatous polyps, while related mechanism remains unclear, which may be associated with insulin resistance, chronic inflammatory response, adipokines, and intestinal flora disturbance.