Pseudomyxoma peritonei originating from appendix tumors / Pseudomixoma peritoneal com origem em tumores do apêndice

ABSTRACT Background: Appendix tumors represent about 1% of all gastrointestinal neoplasia, in other words they are quite rare. However, there is a specific type of appendiceal neoplasms (mucinous adenocarcinoma) that spreads to the peritoneum and in almost 20% of the cases, resulting in a disease called pseudomyxoma peritonei. Although, it is a very rare condition, it is nonetheless a very severe one and therefore it is crucial to know how to correctly diagnose and treat it. Objective: This study provides updated data on how to diagnose, classify and treat pseudomyxoma peritonei that originates from appendix tumors. Methods: A bibliographic research was performed on PubMed database, including articles published since 2000, as well as, cross-referencing with the initial research. Discussion: In the past, patients diagnosed with pseudomyxoma peritonei would only undergo palliative measures, so their overall survival rate was greatly reduced. Over the years pseudomyxoma peritonei treatment has evolved and patients are now undergoing treatment which is a combination of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. This new therapy has allowed an increase of survival chances of up to 5 years in those patients with values between 53% and 88%, depending on the type of tumor. Conclusion: Despite the great progress we have witnessed in recent years, which have led to a large increase in survival rates, more research needs to be done, on what to do when the disease is in an unresectable stage. Finding a less aggressive therapy than cytoreductive surgery + hyperthermic intraperitoneal chemotherapy will be an important step forward.

RESUMO Introdução: As neoplasias do apêndice são bastante raras, representando atualmente cerca de 1% de todas as neoplasias gastrointestinais. O adenocarcinoma mucinoso é um dos subtipos de neoplasia do apêndice e caracteriza-se por metastizar para o peritoneu, em 20% dos casos, facto que se manifesta sob a forma de uma doença designada por Pseudomixoma Peritoneal. Apesar de ser uma condição muito rara, a sua extrema gravidade justifica a importância de a saber diagnosticar e tratar corretamente. Métodos: Foi realizada uma pesquisa bibliográfica na base de dados PubMed, incluindo artigos publicados desde 2000 bem como artigos de pesquisa cruzada com os iniciais. Discussão: No passado, os doentes diagnosticados com Pseudomixoma Peritoneal eram apenas submetidos a medidas paliativas, pelo que a sua sobrevida era muito reduzida. Ao longo dos anos o tratamento do Pseudomixoma Peritoneal foi evoluindo sendo agora os doentes submetidos a uma combinação de cirurgia citoredutiva e quimioterapia hipertérmica intraperitoneal. Esta nova terapêutica tem permitido aumentar a sobrevida aos 5 anos destes pacientes para valores entre os 53% e os 88%, dependendo do tipo de tumor. Conclusões: Apesar dos grandes avanços que se têm verificado, e que culminaram com um grande aumento das taxas de sobrevivência, devem ser feitos mais estudos que encontrem novas abordagens para quando o tumor já é diagnosticado num estado irressecável, bem como terapêuticas menos invasivas.

Pseudomyxoma Peritonei in a Patient with History of Breast Cancer / 대한소화기학회지

Pseudomyxoma peritonei is a very rare condition, and even rarer in patients with history of cancer. A 70-year old woman with a history of breast cancer was admitted with abdominal pain and distention. Abdominal computed tomography revealed ascites collection, diffuse engorgement and infiltration of the mesenteric vessel, suggesting peritonitis or peritoneal carcinomatosis. Diagnostic paracentesis was attempted several times, but a sufficient specimen could not be collected due to the thick and gelatinous nature of the ascites. Therefore, the patient underwent diagnostic laparoscopy for tissue biopsy of the peritoneum, which indicated pseudomyxoma peritonei. However, the origin of the pseudomyxoma peritonei could not be identified intraoperatively due to adhesions and large amount of mucoceles. Systemic chemotherapy was performed using Fluorouracil, producing some symptomatic relief. After discharge, abdominal pain and distention gradually worsened, so at 18 months after initial diagnosis the patient received palliative surgery based on massive mucinous ascites and palpable mass at the omentum. The patient expired after surgery due to massive bleeding.

Pseudomyxome peritoneal

Le pseudomyxome du peritoine est une affection dont la genese est peu connue. De plus sa rarete est confirmee par les 2 cas decouverts de maniere fortuite sur 10.000 laparotomies. Le tableau clinique est pauvre; fait de distension et douleurs abdominales. Il semble que le pseudomyxome soit consecutive a la rupture d'un muccocele appendiculaire. L'association soit a des tumeurs malignes bien differenciees soit a des tumeurs frontieres a souvent ete retrouvee. La production extracellulaire de mucine dans la cavite peritoneale constitue un critere majeur de diagnostic. Le traitement adequat associe la chirurgie complete des lesions macroscopiques et la chimiotherapie intraperitoneale en utilisant certaines molecules qui ont montre leur efficacite

Mucinous adenocarcinoma of the renal pelvis with pseudomyxoma peritonei.

Mucinous adenocarcinoma of the renal pelvis is an extremely rare tumor with very few case reports in literature. Pseudomyxoma peritonei is an uncommon condition characterized by the presence of mucinous gelatinous material in the peritoneal cavity. It occurs secondary to primary mucinous neoplasms of particularly the appendix and the ovary. We present a case of a 35-year-old female who had a history of dull aching pain in the right flank since one and a half years. Upon ultrasonography (USG) and computerized tomography (CT) scan, there was a large cystic mass measuring 15x15x12 cm, extending into the right lumbar region. Grossly, the entire kidney was converted into a cystic mass measuring 15x15x12 cm containing gelatinous mucinous material weighing 1 kg. Histologically, the tumor was composed of simple and complex glandular acini together with a superficial resemblance to colonic mucosa with abundant extracellular mucin. Thus, a diagnosis of mucinous adenocarcinoma of the renal pelvis leading to pseudomyxoma peritonei was made.

Peritoneal pseudomyxoma associated with synchronic malignant mucinous neoplasias of the cecum, appendix and rectum: case report and review of the literature

Peritoneal pseudomyxoma is a pathological condition that compromises the peritoneum, characterized by the production of large quantities of mucinous liquid, which progressively fills the peritoneal cavity, originating in general from mucinous appendicular or ovarian tumors. We report a peritonial pseudomyxoma associated with mucinous adenocarcinoma of the appendix synchronic with adenocarcinoma of the rectum in 44 years old patient, where the initial diagnosis was rectal adenocarcinoma. Tumour of the appendix and peritonial pseudomyxoma were incidental and found intraoperativelly. We focus the histological patterns of the lesions, diagnosis and the treatment, with revision of the literature.

O pseudomixoma peritoneal é uma condição patológica que acomete o peritoneo, caracterizada pela produção de grandes quantidades de líquido mucinoso, que progressivamente preenche a cavidade peritoneal, tendo em geral como origem tumores mucinosos apendiculares ou de ovário. Relatamos a ocorrência de um pseudomixoma peritoneal associado a adenocarcinoma mucinoso do apêndice sincrônico e adenocarcinoma do reto em paciente de 44 anos, cujo diagnóstico inicial foi de adenocarcinoma do reto. A neoplasia do apêndice e o pseudomixoma peritonial foram achados incidentais, intra-operatórios. Enfocamos as principais características anatomo-patológicas das lesões, o diagnóstico e tratamento, através de ampla revisão da literatura.

Pseudomixoma peritoneal: diagnóstico y tratamiento / Pseudomyxoma peritonei: diagnosis and treatment

Introducción: el pseudomixoma peritoneal (PMP) es una entidad clínica poco frecuente, caracterizada por múltiples implantes peritoneales, de un material mucinoso derivado generalmente de un tumor mucosecretor, que se localiza con mayor frecuencia en apéndice, ovario y páncreas. En un 10 de los casos no se puede determinar su origen.Caso Clínico: Varón, de 69 años, que consulta por distensión abdominal progresiva y pérdida de peso (16 Kg.) de un año de evolución exacerbada en los últimos 3 meses asociada a dolor abdominal difuso y estado nauseoso. Se realiza una laparotomía exploradora mediana supra-infraumbilical, la cual permitió drenar abundante cantidad de contenido mucinoso, constatándose el compromiso de varios órganos, especialmente el ciego. Ante la imposibilidad de llevar a cabo el procedimiento de Sugarbaker, por el grado de compromiso de las estructuras abdominales, se prcedió a instilar Mitomicina C (12,5/m2 de superficie corporal) intraoperatoria, hipertérmica (43º C) durante 90 minutos. El paciente evoluciona favorablemente, retirándose los drenajes, gracias al descenso gradual del débito.Discusión: Representa el primer caso en 95 años de labor asistencial en nuestra institución. Como factores que empeoran el pronóstico, se han descrito, la presencia de distensión abdominal, sexo masculino, historia de pérdida de peso, enfermedad difusa, afectación de órganos vecinos (todos presentes en nuestro paciente). La incidencia de esta patología es baja, según lo reportado por la literatura, no obstante esta es más frecuente en mujeres. Creemos que la cirugía es de suma utilidad, teniendo en cuenta que permite obtener un diagnóstico, la posibilidad de drenar el contenido mucinoso de la cavidad, y en los mejores casos realizar los procedimientos de Sugarbaker, Siendo paliativo el tratamiento realizado por nuestro equipo, el paciente experimentó una mejoría clínica importante, lo cual le permitió su reinserción familiar y laboral.

Pseudomyxoma peritonei presenting as sub-acute large bowel obstruction.

A case of pseudomyxoma peritonei, a rare mucin-secreting tumour in the peritoneum, in a 30-year-old male and presenting as sub-acute large bowel obstruction, is reported here with review of the literature.

Pseudomixoma peritoneal: aspectos tomográficos e na ressonância magnética - relato de três casos / Pseudomyxoma peritonei - report of three cases: computed tomography and magnetic resonance imaging findings

O pseudomixoma peritoneal é um tumor incomum, de curso indolente, que se caracteriza pela presença de ascite mucinosa ou implantes na cavidade peritoneal. Origina-se geralmente de lesões no apêndice ou no ovário. O diagnóstico pode ser feito por meio da citologia de aspiração por agulha fina, ultra-sonografia, tomografia computadorizada ou ressonância magnética. Os autores relatam três casos de pseudomixoma peritoneal cujo sítio primário era o ovário, e que foram submetidos a tomografia computadorizada e a ressonância magnética do abdome. Este trabalho enfatiza a importância destes métodos em função de sua capacidade de resolução espacial, imagens multiplanares e diferentes seqüências (na ressonância magnética), permitindo melhor avaliação das lesões. Os exames tomográficos demonstraram massas lobuladas, hipodensas, com limites bem definidos, determinando "lobulações" nas margens hepática e esplênica por compressão extrínseca secundária a implantes peritoneais, sem invasão dos órgãos. A ressonância magnética revelou lesões expansivas com baixo sinal nas imagens ponderadas em T1 e alto sinal em T2, de localização peritoneal, junto às margens do fígado e baço

Pseudomyxoma peritonei is rare tumor that presents insidious evolution and is characterized by mucinous ascites or implants in the peritoneal cavity. The origin of this tumor is frequently the appendix or the ovaries. Diagnosis can be established by fine needle aspiration cytology, ultrasonography, computed tomography or magnetic resonance imaging . The authors report three cases of patients with pseudomyxoma peritonei arising from the ovary that were submitted to computed tomography and magnetic resonance imaging examinations of the abdomen. This paper emphasizes the importance of these imaging methods that permit greater spatial resolution, multiplanar images and different types of sequences (magnetic resonance imaging), and allow better evaluation of these lesions. Computed tomography examinations showed lobulated low-density masses with well defined limits causing "scalloping" of the hepatic and splenic borders due to extrinsic compression by adjacent peritoneal implants. Invasion of the liver and spleen was not observed. Magnetic resonance imaging examinations revealed expansive peritoneal lesions with low-intensity signal on T1-weighted images and high-intensity signal on T2-weighted images, adjacent to the hepatic and splenic borders.

Pseudomyxoma peritonei of hernial sac--a case report.

A rare presentation of Pseudomyxoma Peritonei of hernial sac is described. The patient was admitted for repair of an inguinal hernia. During herniorraphy large amount of mucinous material was found in hernial sac. Microscopy revealed epithelial glandular cells with bland appearance within mucinous pools. A search for primary remained fruitless.

Occult primary adenocarcinoma causing pseudomyxoma peritonei with pleural and hepatic metastases.

Pseudomyxoma peritonei is characterized by diffuse collections of gelatinous flux resulting from implantation of malignant tumors or irritation from ruptured benign cysts. We report a patient with pseudomyxoma peritonei caused by an occult primary adenocarcinoma, who had both pleural and hepatic metastases.

Pseudomixoma peritoneal: valor diagnóstico de la tomografía computada y resonancia magnética / Pseudomyxoma peritonei: diagnostic value of CT and MRI

Los autores presentan un caso de pseudomixoma peritoneal desarrollado a partir de un adenocarcinoma mucosecretante de colon. Se analizan los signos aportados por TC y RM destacándose la mayor sensibilidad tomográfica para la demostración de comprensiones intestinales así como para la identificación de calcificaciones originadas postquimioterapia

Pseudomixoma peritoneal associado a lúpus eritematoso sistêmico: reporte de caso / Pseudomyxoma peritonei in association with systemic lupus erythematosus: case report

Descreve-se o caso de uma paciente jovem com ascite, dor abdominal, emagrecimento e poliartralgias. Foi feito diagnóstico de pseudomixoma peritoneal de ovário. Lúpus eritematoso sistêmico foi diagnosticado como patologia associada. Os autores apresentam breve revisao da literatura.

Pseudomixoma peritoneal secundario a cistoadenocarcinoma del apéndice cecal: caso clínico / Pseudomyxome peritonei secondary to a cystadenocarcinoma of the appendix: clinical case

We report a 40 years old female that presented with an ovarian tumor and ascitis. The exploratory laparotomy revealed a mucocele of the appendix that was informed as a cystadenoma of the appendix in the fast biopsy. The definitive pathological diagnosis, a cystadenocarcinoma of the appendix, motivated a second intervention, preforming a right hemicolectomy, left adnexectomy and omentumectomy. Three years later she presented with an abdominal mass and was subjected to a total hysterectomy and right adnexectomy. The pathological diagnosis was a mucinous cystadenocarcinoma with peritoneal involvement. The patient completed five years of follow up since the first intervention and is free of tumor. Pseudomixome peritoneai generally is a tumor of ovarian origin, followed by the appendix. Surgical treatment must include the excision of neighboring compromised organs. The follow up must be lengthy considering the possibility of late recurrences

Tratamento conservador de pseudomixoma peritonial

O pseudomixoma peritoneal e uma entidade clinica rara caracterizada por ascite gelatinosa relacionada com certa frequencia ao adenocarcinoma mucoprodutor do apendice ou ovario, de tratamento eminentimente cirurgico. A evolucao desfavoravel e geralmente devida a obstrucao intestinal causada pelo muco abdominal e complicacoes pos-operatorias. Alternativas terapeuticas nao operatorias poderiam contribuir para diminuir a mortalidade cirurgica. A revisao bibliografica, analisando diversas modalidades terapeuticas foi realizada, estimulando a verificacao do efeito mucolitico da glicose a 2,5 por cento, que no presente caso com pseudomixoma peritoneal e hernia umbilical operado tres comprovou-se de grande eficiencia.

Pseudomixoma peritoneal / Pseudomyxoma peritonei

Pseudomixoma de peritónio , uma patologia para que se manifesta por implantes de mucina na cavidade peritoneal decorrentes de tumor de ov rio ou apêndice. Os autores relatam um caso e fazem revisäo de literatura.

Pseudomyxoma peritonei: case report and review of the literature

Pseudomyxoma peritonei es una condición rara que se manifiesta por múltiples implantes de un material gelatinoso en la cavidad abdominal, omento y peritoneo. Usualmente surge de lesiones de la apéndice o del ovario. El diagnóstico se puede sospechar preoperatoriamente basándose en estudios radiográficos. El tratamiento básico de esta condición lo es cirugía donde debe extraerse la apéndice y los ovarios al igual que el omento. Debe sacarse todo aquel implante que sea posible. Se ha utilizado quimioterapia y radioterapia paa ayudar a mejorar la sobrevida a cinco (5) años, la cual se ha reportado es alrededor de 54%. Aquí presentamos un caso de un paciente con mucocele de la apéndice y pseudomyxoma peritonei que se presenta con una distensión masiva de su abdomen de dos años de evolución