Mesotelioma peritoneal multiquístico benigno: reporte de un caso y revisión de la literatura / Benign multicystic peritoneal mesothelioma: case report and literature review

INTRODUCCIÓN: El mesotelioma peritoneal multiquístico benigno (MPMB) es una entidad con muy baja incidencia que se presentan con mayor frecuencia en mujeres jóvenes. CASO CLÍNICO: Paciente de 17 años que consulta por amenorrea secundaria. Además, refería pérdida de peso, estreñimiento y molestias abdominales. La exploración fue normal y en las pruebas de imagen se objetivó una pelvis ocupada por una masa multiquística, de unos 20 cm de diámetro máximo, de paredes finas y contenido anecoico. Se solicitaron marcadores tumorales de los cuales sólo se encontró elevado el CA 19.9 (35,2 U/mL). Se decidió realizar una laparoscopia exploradora para establecer un diagnóstico y ante los hallazgos y al considerarse la masa fácilmente resecable, en el mismo acto quirúrgico se extirpó por completo para su estudio anatomopatológico, que confirmó un MPMB. Se desestimaron tratamientos adicionales y actualmente se encuentra en seguimiento y asintomática. CONCLUSIÓN: La patogénesis del MPMB no está clara. Algunos autores defienden que se trata de una reacción peritoneal a un daño inflamatorio, otros apoyan que se trata de una neoplasia benigna. Esta naturaleza incierta, junto con la escasa experiencia, hacen que el manejo resulte complejo. Es necesario individualizar cada caso e intentar ser conservadores especialmente en pacientes jóvenes. Si se opta por un manejo activo, lo más acertado es la citorreducción completa seguida de quimioterapia hipertérmica peritoneal. El seguimiento, en cualquier caso, debe ser exhaustivo y multidisciplinar principalmente con pruebas de imagen.

BACKGROUND: Benign Multicystic Peritoneal Mesothelioma (BMPM) is an entity with a very low incidence that occurs more frequently in young women. CASE REPORT: A 17-year-old woman attended for secondary amenorrhea. She also referred to weight loss, constipation and abdominal discomfort. The examination was normal and echography revealed a pelvis occupied by a 20 centimeters multicystic mass, with thin walls and anechoic content. A preoperative study with tumor markers was requested in which only a CA 19.9 elevation was observed (35.2 U/ml 0-27). It was decided to perform an exploratory laparoscopy to establish a diagnosis. Due to the findings and because of the fact that the mass was considered easy to remove, it was completely removed in the same surgical act. The pathological result confirmed a BMPM. Additional treatments have been dismissed and are the patient is currently being followed up without symptoms. CONCLUSION: The pathogenesis of BMPM is unclear. Some authors argue that it is a peritoneal reaction to inflammatory damage, others support that it is a benign neoplasm. This uncertain nature coupled with limited experience make management complex. It is necessary to evaluate each case and try to be conservative especially in young patients. If surgery management is chosen, complete cytoreduction followed by hyperthermic peritoneal chemotherapy is the most successful option. The follow-up in any case must be exhaustive and multidisciplinary, mainly with imaging tests.

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.

Mujer joven con hepatocarcinoma fibrolamelar metastásico; citorreducción y HIPEC: a propósito de un caso / Young woman with metastatic fibrolamellar hepatocarcinoma; cytoreductive surgery and HIPEC: a case report

Objetivo: Reportar un caso clínico de hepatocarcinoma fibrolamelar metastásico y su manejo multidisciplinario. Caso clínico: Paciente de 24 años de edad con dolor abdominal, distensión abdominal y fiebre. Se le realizó tomografía computarizada de abdomen donde se encontró tumoración hepática irregular. Se realizó laparotomía con evidencia de múltiples implantes en cavidad abdominal y se diagnosticó mediante estudio histopatológico hepatocarcinoma fibrolamelar metastásico. Se decidió realizar citorreducción más quimioterapia hipertérmica intraperitoneal (HIPEC). La sobrevida de la paciente fue de 11 meses. Discusión: El hepatocarcinoma fibrolamelar es un tumor raro. Aún no hay consenso sobre el mejor tratamiento en pacientes con metástasis que tengan buena funcionalidad. El manejo actual se basa en la quimioterapia sistémica y la resección quirúrgica en casos localizados. En el caso de nuestra paciente, la cirugía citorreductora más HIPEC se realizó con la intención de mejorar la supervivencia. Se necesita más evidencia para definir esta estrategia como tratamiento estándar.

Aim: To report a clinical case of metastatic fibrolamellar hepatocarcinoma and its multidisciplinary management. Case report: 24 year-old patient with abdominal pain, bloating and fever. A computed tomography of the abdomen was performed; an irregular hepatic tumor was found. A laparotomy was performed with evidence of multiple implants in the abdominal cavity and the histopathology report was metastatic fibrolamellar hepatocarcinoma. It was decided to perform cytoreductive surgery plus HIPEC. The patient's survival was 11 months. Discussion: Fibrolamellar hepatocarcinoma is a rare tumor. There is still no consensus on the treatment of choice in patients with metastases with good functionality status. Current management is based on systemic chemotherapy and surgical resection in localized cases. In the case of our patient, cytoreductive surgery plus HIPEC was performed with the intention of improving survival. More evidence is needed to define this strategy as standard treatment.

A quimioterapia intraperitoneal hipertérmica e o cuidado do enfermeiro no transoperatório: uma revisão sistematizada

A cirurgia citorredutora associada à quimioterapia Intraperiotoneal Hipertermica no tratamento da disseminação Peritoneal das neoplasias é um procedimento, de fato, consolidado no nosso meio, capaz de oferecer benefícios no tratamento de pacientes que apresentam disseminação de doença restrita ao peritônio. O objetivo do estudo é identificar na literatura científica a técnica da quimioterapia intraperitoneal hipertérmica relacionada aos cuidados do enfermeiro no transoperatório. O método utilizado para a pesquisa é uma revisão bibliográfica sistematizada nas bases de dados sobre a quimioterapia intraperitoneal hipertérmica e o cuidado do enfermeiro no transoperatório. Os estudos indicam que em relação à cirurgia citorredutora associada à quimioterapia Intraperiotoneal Hipertermica no tratamento da disseminação Peritoneal das neoplasias são obtidos resultados satisfatórios nos casos em que há menos volume de carcinomatose e que a cirurgia proporciona uma citorredução satisfatória. Conclui-se que a evolução do paciente será avaliada ao longo do processo, quando este apresentará respostas em relação ao seu tratamento, demonstrada através da eficácia das ações de enfermagem

Cytoreductive surgery combined with chemotherapy in the treatment of hyperthermia Intraperiotoneal Periontoneal spread of cancer is a procedure in fact consolidated in our midst, that provides benefits in the treatment of patients with limited disease spread to the peritoneum. The objective is to identify the scientific literature the technique of hyperthermic intraperitoneal chemotherapy nursing care during surgery. The method used for the research is a systematic review of the literature review in databases and hyperthermic intraperitoneal chemotherapy in perioperative nursing care. The results indicate that in relation to cytoreductive surgery combined with chemotherapy in the treatment of hyperthermia Intraperiotoneal Periontoneal spread of tumors are obtained in cases where there is less volume of carcinomatosis and that surgery has gotten a good cytoreduction. It is concluded that the patient's course will be evaluated throughout the process, when it will deliver responses to the treatment, demonstrated by the effectiveness of nursing actions

Carcinomatosis peritoneal de origen colorrectal. Cirugía citorreductora y quimioterapia hipertérmica intraperitoneal / Colorectal peritoneal carcinomatosis: citoreductive surgery and hyperthermic intraperitoneal chemotherapy

Antecedentes: El pronóstico de los pacientes con carcinomatosis peritoneal de origen colorrectal es pobre. Tradicionalmente, para su tratamiento se utilizó la quimioterapia sistémica mientras que la cirugía estuvo reservada al tratamiento de las complicaciones. Objetivo: Establecer el estado actual de la cirugía citorreductora y la quimioterapia hipertérmica y valorar sus alcances en parámetros objetivos de acuerdo con la bibliografía. Material y Métodos: Se utilizaron para la búsqueda todos los artículos publicados acerca del tema en inglés y francés entre los años 1990 y 2008 en las bases de datos de PubMed de la Biblioteca Nacional de Medicina de los EEUU. Resultados: La cirugía citorreductora actúa sobre las masas neoplásicas macroscópicas, mientras que la quimioterapia hipertérmica intraperitoneal (QTHIP) es útil para tratar la enfermedad residual microscópica. La asociación entre ambas se debe a que la primera intenta resecar todo el tumor visible o, en su defecto, hasta 2 mm de espesor, a fin de permitir la acción de las drogas quimioterápicas, que pueden penetrar esa distancia como máximo en los tejidos tumorales. Los pacientes con un aceptable estado clínico (“performance status”), sin diseminación extraabdominal, con metástasis ganglionares y/o hepáticas resecables, baja carga de enfermedad peritoneal y potencialmente pasibles de una citorreducción completa, se considerarían para este tratamiento combinado. Los factores predictivos más utilizados en la actualidad lo constituyen el Índice de Cáncer Peritoneal (ICP) y la Citorreducción Completa. La mayoría de los autores coincide en admitir que los beneficios de la cirugía citorreductora (CC) más la QTHIP dependen principalmente de la capacidad de la cirugía de alcanzar una resección completa. La quimioterapia intraperitoneal no ha podido, por sí sola, tratar grandes volúmenes de carcinomatosis: solamente se han reportado tratamientos exitosos con el tratamiento combinado...

Background: Colorectal peritoneal carcinomatosis leads to a poor prognosis. Traditionally, its treatment has consisted in systemic chemotherapy, whereas surgery has been reserved to the management of complications. Objective: To establish the current state of cytoreductive surgery (CRS) and hyperthermic chemotherapy (HIPEC), and to assess their implications with objective parameters according to literature. Material and Methods: All papers published in english and french about the subject between 1990 and 2008 in the PubMed database were retrieved. Results: Cytoreductive surgery works over macroscopic neoplastic masses, as on microscopic residual disease HIPEC does. Their association is realted with their goals: the first one tries to resect all visible tumor or at least less than 2 mm, to allow chemoterapic drugs a deep penetration in tumoral tissues. Patients with an adequate performance status, with no extraabdominal spread, with resectable lymph nodes and/or hepatic metastasis, low burden of peritoneal disease and potentially fit for a complete cytoreduction, would be considered for this treatment. Nowadays, the most utilized predictive factors are constituted by the Peritoneal Cancer Index and Completeness of Cytoreduction. Most authors agree in the consideration that CRS plus HIPEC benefits depends mainly on the possibility of a complete surgical resection. Intraperitoneal chemotherapy could not, for its own, treat large tumoral masses: there are only reported successful treatments with the combined modality. At the same time, most papers prove that HIPEC combined with an optimum cytoreductive surgery cures about 25 per cent of the patients considered as unresectable. Benefits in survival related to this treatment are reached in spite of a high morbidity and mortality. The most severe complications are represented by anastomotic fistulas...