Diagnóstico de cuatro neoplasias primarias sincrónicas en un adulto / Diagnosis of four synchronous primary neoplasms in an adult

Paciente femenina de 72 años. Mediante estudios de imagen (ultrasonido y tomografía), se le identificó lesión en parénquima hepático, anexo derecho, peritoneo y apéndice cecal, y mediante estudio histopatológico se determinó la presencia concomitante de carcinoma hepatocelular de células claras, tumor mucinoso limítrofe de bajo potencial maligno o borderline, pseudomixoma peritoneal y neoplasia mucinosa de bajo grado del apéndice cecal, respectivamente. Debido a que las neoplasias reportadas no guardan relación con el mismo órgano ni con el sistema, se considera que son neoplasias aparecidas al azar y de tipo sincrónico por ser diagnosticadas en el mismo espacio temporal. Se practicó laparotomía exploradora con exéresis de lesión anexial y de apéndice cecal. La lesión hepática recibió quimioembilización transarte rial por radiología intervencionista. Posterior a la intervención quirúrgica, la paciente presenta buen estado general. En seguimiento con resonancia magnética se cataloga con persistencia de lesión hepática ya tratada, por lo tanto, con enfermedad estable; se refiere a oncología clínica para valoración de quimioterapia en el manejo del pseudomixoma peritoneal. Dieciocho meses después de los diagnósticos iniciales, se documenta carcinoma basocelular y se cataloga como neoplasia metacrónica por la diferencia de tiempo entre los diagnósticos

A 72-year-old female patient with a one-year history of abdominal pain in the right upper quadrant, colicky, radiating to the back, accompanied by adynamia and weight loss. Abdominal distension and a painful mass on palpation in the right hypochondrium were evidenced. Imaging studies identified different lesions in the hepatic parenchyma, right adnexa, peritoneum and cecal appendix. The histopathological study described the presence of clear cell hepatocellular carcinoma, borderline mucinous tumor of low malignant potential or borderline, peritoneal pseudomyxoma and low-grade mucinous neoplasm of the cecal appendix respectively, as synchronous neoplasms. An exploratory laparotomy was performed with excision of the adnexal lesion and the cecal appendix. The hepatic lesion received transarterial chemoembilization by interventional radiology. Follow-up with conservative management by clinical oncology was indicated. The patient evolved with good general condition, in the follow-up with magnetic resonance imaging was classified with persistence of stable hepatic lesion. Eighteen months after the diagnosis of synchronous neoplasms, basal cell carcinoma was identified, due to the difference in the time of diagnosis this is considered a metachronous neoplasm

Mucocele of the appendix: what to expect / Mucocele do apêndice: o que esperar

Abstract Mucoceles of the appendix are rare and can have quite variable imaging and clinical presentations, sometimes mimicking an adnexal mass. The underlying cause can be neoplastic or non-neoplastic. The typical imaging appearance of a mucocele of the appendix is that of a cystic structure with a tubular morphology. This structure is defined by having a blind-ending and being contiguous with the cecum. Radiologists should be familiar with key anatomical landmarks and with the various imaging features of mucoceles of the appendix, in order to provide a meaningful differential diagnosis of a lesion in the right lower abdominal quadrant. In addition, a neoplastic mucocele can rupture, resulting in pseudomyxoma peritonei, which will change the prognosis dramatically. Therefore, prompt diagnostic imaging is crucial.

Resumo Mucoceles do apêndice são raras e podem ter uma apresentação clínica e imagiológica bastante variável, por vezes mimetizando patologia anexial. As causas subjacentes podem ser neoplásicas ou não neoplásicas. O aspecto de imagem típico de mucoceles do apêndice é o de uma estrutura de natureza cística com morfologia tubular. Esta estrutura deverá terminar "em fundo cego" e ser contígua com o ceco. Os radiologistas devem estar familiarizados com os pontos anatômicos de referência e com as diferentes características imagiológicas de mucoceles do apêndice, de modo a fornecer um adequado diagnóstico diferencial de uma lesão localizada no quadrante abdominal inferior direito. Para além disso, uma mucocele neoplásica pode sofrer ruptura, resultando em pseudomixoma peritoneal, o que altera drasticamente o prognóstico. Assim, o diagnóstico por imagem em tempo útil é crucial.

Pseudomixoma peritoneal primario de origen ovárico / Primary pseudomyxoma peritonei originated from ovaries

Resumen ANTECEDENTES: El pseudomixoma peritoneal es muy raro; se caracterizada por ascitis mucinosa e implantes peritoneales relacionados con la rotura y diseminación del contenido de un tumor mucinoso. En 80 al 90% de los casos el tumor primario es apendicular y el ovario es una localización realmente excepcional. CASO CLÍNICO: Paciente de 49 años que acudió a consulta debido a un dolor abdominal. En las pruebas de imagen se visualizó una tumoración anexial izquierda y se advirtieron hallazgos sugerentes de pseudomixoma peritoneal. En la cirugía, la masa ovárica se objetivó parcialmente fragmentada, con el apéndice aumentado de tamaño y extensos implantes peritoneales; además de gran cantidad de mucina libre en la cavidad peritoneal. El análisis anatomopatológico determinó la existencia de un adenocarcinoma mucinoso en el ovario afectado, con inmunohistoquímica positiva para CK7+ y CK20+, múltiples implantes de mucina y el apéndice sin daño. Por lo anterior se diagnosticó: pseudomixoma peritoneal de origen ovárico. Luego de dos intervenciones quirúrgicas no se consiguió la citorreducción completa. La paciente permaneció estable durante siete años, momento en el que fue evidente el avance de los síntomas de la enfermedad, circunstancia que la condujo a la muerte. CONCLUSIÓN: Determinar el origen de un pseudomixoma peritoneal sigue siendo un reto pues, con frecuencia, tanto el apéndice como los ovarios se afectan simultáneamente. Por ello, la apendicectomía y la exploración bilateral de los ovarios deben ser prácticas de rutina. El análisis extenso de las muestras y la inmunohistoquímica pueden facilitar la catalogación de estos infrecuentes tumores.

Abstract BACKGROUND: Pseudomyxoma peritonei is very rare; it is characterized by mucinous ascites and peritoneal implants related to rupture and dissemination of the contents of a mucinous tumor. In 80 to 90% of cases the primary tumor is appendicular and the ovary is a truly exceptional location. CLINICALCASE: A 49 year old female patient presented for consultation due to abdominal pain. Imaging tests showed a left adnexal tumor and findings suggestive of pseudomyxoma peritonei. At surgery, the ovarian mass was partially fragmented, with an enlarged appendix and extensive peritoneal implants; in addition to a large amount of free mucin in the peritoneal cavity. The anatomopathological analysis determined the existence of a mucinous adenocarcinoma in the affected ovary, with positive immunohistochemistry for CK7+ and CK20+, multiple mucin implants and an undamaged appendix. Therefore, a diagnosis was made: pseudomyxoma peritoneum of ovarian origin. After two surgical interventions she did not achieve complete cytoreduction. The patient remained stable for seven years, at which time the symptoms of the disease became evident and led to her death. CONCLUSION: Determining the origin of a pseudomyxoma peritonei remains a challenge as often both the appendix and ovaries are affected simultaneously. Therefore, appendectomy and bilateral ovarian exploration should be routine practice. Extensive specimen analysis and immunohistochemistry can facilitate cataloging of these infrequent tumors.

Evolución atípica de un anciano con Pseudomixoma Peritoneal sometido a tratamiento quirúrgico / Atypical evolution of and elder man with peritoneal pseudomyxoma under surgical treatment

Se describe un paciente anciano de 76 años atendido en el Hospital General Docente Leopoldito Martínez de San José de las Lajas, Mayabeque, por presentar dolor abdominal y náuseas en el que se realizó el diagnóstico de Pseudomixoma con Carcinomatosis peritoneal. El estudio de ultrasonido y la tomografía abdominal demostró la existencia de una masa hiperecogénica trabeculada en región del hipocondrio izquierdo. En la primera intervención quirúrgica se hizo exéresis de una masa mucinosa del omento mayor y de la región subdiafragmática izquierda. Una segunda evaluación clínica e imaginológica evidencia una esplenomegalia por la cual se decide realizar esplenectomía. El estudio de biopsia muestra abundante infiltrado de células linfomonocitarias con aspecto inflamatorio crónico en el bazo.(AU)

A 76 year old male patient assisted at Leopoldito Martinez General Teaching Hospital in San Jose de las Lajas, Mayabeque, is described. He attended complaining of abdominal pain and nausea, the diagnosis of Pseudomyxoma with Peritoneal Carcinomatosis was performed. Ultrasonography study and abdominal tomography showed the existence of a hyperechoic and trabeculae mass in the site of the left hypochondria. In the first abdominal surgery, excision of a mucinous mass of the major omentum and the left sub diaphragmatic region was performed. A second clinical and images evaluation evidenced splenomegaly and splenectomy is decided to perform. The biopsy showed abundant infiltrates of lymphomonocytic cells with chronic inflammatory appearance in the spleen. Six years after the first intervention, the patient is still alive and with acceptable health status, althoug h he had not received cytoreductive surgery or intra-abdominal chemotherapy with hyperthermia as it is stated in the literature. (AU)

Imaging findings for mucinous tumors of the abdomen and pelvis. / Hallazgos en imagen en los tumores mucinosos de abdomen y pelvis.

This article aims to describe the imaging findings for mucinous tumors of the abdomen and pelvis, which have a similar appearance on imaging tests regardless of the organ in which they develop. Due to the high water content of mucus, the appearance of these tumors is generally similar to that of water on ultrasonography, computed tomography, and magnetic resonance imaging. Another common feature of mucin-producing tumors is that calcifications are often present. The rupture of these lesions and accumulation of mucinous material in the peritoneal cavity gives rise to pseudomyxoma peritonei. It is important to identify mucinous tumors because they have a different prognosis and clinical course than non-mucinous tumors and require different management. Depending on their anatomic location and their imaging characteristics, the treatment approach varies from follow-up to radical surgery together with chemotherapy or radiotherapy or both.

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.

Peritoneal carcinomatosis treated with cytoreductive surgery and intraperitoneal chemotherapy / Carcinomatose peritoneal tratada com cirurgia citoreductiva e quimioterapia intraperitoneal

ABSTRACT Introduction: To evaluate the combined treatment with cytoreductive surgery and intraperitoneal chemotherapy for peritoneal carcinomatosis arising from colorectal cancer, pseudomyxoma peritonei and mesothelioma. Methods: Data were obtained from 73 patients with peritoneal carcinomatosis arising from colorectal cancer (52.1%), pseudomyxoma peritonei (41.1%) or mesothelioma (6.8%) between 2002 and 2011. We reported the morbidity grade (II, III and IV), mortality and survival rates of the candidates after cytoreductive surgery and intraperitoneal chemotherapy. Results: 41 (56.2%) women participated, and the median age was 50 years. Thirty-nine patients (53.4%) underwent complete cytoreductive surgery and intraperitoneal chemotherapy. Patients who underwent a complete cytoreduction received intraperitoneal chemotherapy with mitomycin C, from which only 16/39 (41%) had hyperthermic intraperitoneal chemotherapy (41-42 °C). The overall morbidity rate was 23.3% and the grade III/IV complication rate was 12.3%. The overall mortality rate was 5.5%. The univariate analysis showed that cytoreductive surgery and intraperitoneal chemotherapy (p = .029), a blood transfusion (p = .002) and the operative time (p = .001) were significant for the occurrence of postoperative complications. Patients with peritoneal carcinomatosis from colorectal cancer who underwent complete cytoreductive surgery and intraperitoneal chemotherapy had overall survival rates of 81.3%, 12.5% and 12.5% at 1, 3 and 5 years, respectively. Patients with peritoneal carcinomatosis from pseudomyxoma peritonei who underwent complete cytoreductive surgery and intraperitoneal chemotherapy had overall survival rates of 84.2%, 77.7% and 77.7% at 1, 3 and 5 years, respectively. Conclusion: The combined treatment for peritoneal carcinomatosis may be performed safely with acceptable morbidity and mortality in a specialized unit setting. Although over half of patients underwent normothermic intraperitoneal chemotherapy, our results were comparable to results from others centers.

RESUMO Introdução: O objetivo foi avaliar o tratamento combinado da cirurgia citorredutora e quimioterapia intraperitoneal em pacientes com carcinomatose peritoneal secundária ao câncer colorretal, pseudomixoma peritoneal e mesotelioma. Métodos: Foram obtidos dados de 73 pacientes com carcinomatose peritoneal secundária ao cirurgia citorredutora (52.1%), pseudomixoma peritoneal (41,1%) ou mesotelioma (6,8%). Foram avaliados o grau de morbidade, a taxa de mortalidade e as taxas de sobrevida após a cirurgia citorredutora e quimioterapia intraperitoneal. Resultados: 41 (56,2%) pacientes do sexo feminino participaram, com média de idade de 50 anos. 39 pacientes (53,4%) foram submetidos a cirurgia citorredutora completa e quimioterapia intraperitoneal. Todos esses receberam Mitomicina C, sendo 16/39 (41%) quimioterapia intraperitoneal hipertérmica (41-42°C). A morbidade global foi 23,3%, com taxa de mortalidade global de 5,5%. A análise univariada mostrou que câncer colorretal e quimioterapia intraperitoneal (p = .029), transfusão sanguínea (p = .002) e tempo operatório (p = .001) foram associados com complicações pós-operatórias. Pacientes com carcinomatose peritoneal secundária ao cirurgia citorredutora submetidos a cirurgia citorredutora completa e quimioterapia intraperitoneal tiveram sobrevida global de 81,3%; 12,5% e 12,5% em 1, 3 e 5 anos, respectivamente. Os pacientes com pseudomixoma peritoneal que foram submetidos a cirurgia citorredutora completa e quimioterapia intraperitoneal tiveram sobrevida global de 84,2%; 77,7% e 77.7% em 1, 3 e 5 anos, respectivamente. Conclusão: O tratamento combinado para carcinomatose peritoneal é seguro quando realizado em centros terciários com experiência no procedimento. Embora mais da metade dos pacientes tenham sido submetidos a quimioterapia intraperitoneal normotérmica após a cirurgia citorredutora completa, os resultados podem ser comparados a de outros centros que utilizam exclusivamente a quimioterapia hipertérmica.

Pseudomyxoma peritonei in a pediatric patient: A case report and literature review / Pseudomixoma peritoneal em paciente pediátrico: relato de caso e revisão de literatura

Summary Introduction: Pseudomyxoma peritonei (PMP) is a rare clinical condition, with an incidence of 1-2 cases per million, characterized by the dissemination of mucinous implants on the peritoneal surface and progressive gelatinous ascites. Although it usually presents an indolent behavior, its non-specific clinical presentation contributes to many cases remaining undiagnosed until a laparotomy is performed. With late diagnosis, performed after a long period of clinical deterioration and disease progression, it is common to find complications such as the formation of intestinal fistulas and obstruction. Method: Review of the medical record and search for references in the Medline, Lilacs, SciELO and MD Consult databases. Results: There are rare case reports found in the literature demonstrating atypical PMP presentations. Our report is that of a 17-year-old adolescent with a sporadic tumor diagnosed in a primary site in the transverse colon, contrary to data commonly found in the literature that mention a more frequent occurrence in women in the fifth decade of life and with a primary site in the ovary and appendix. The development of mucinous adenocarcinoma is rare in the pediatric population, and topography in the transverse colon and non-familial sporadic pattern are unusual. Conclusion: The case reported not only raises awareness about the atypical presentations of the disease, but also emphasizes the use of imaging examinations for diagnosis, which has an important impact on prognosis and survival if performed timely.

Resumo Introdução: O pseudomixoma peritoneal (PMP) é uma condição clinica rara, com incidência de 1-2 casos por milhão, caracterizada pela disseminação de implantes de natureza mucinosa pela superfície peritoneal e acúmulo progressivo de ascite gelatinosa. Embora apresente geralmente um comportamento indolente, a apresentação clínica inespecífica contribui para que muitos casos permaneçam sem diagnóstico até a realização de laparotomia. Com o diagnóstico tardio, realizado após um longo período de deterioração clínica e progressão de doença, é comum encontrar complicações, como a formação de fístulas e obstruções intestinais. Método: Revisão do prontuário médico e pesquisa bibliográfica nas bases de dados Medline, Lilacs, SciELO e MD Consult. Resultados: São raros os relatos de caso encontrados na literatura que demonstram apresentações atípicas do PMP. O presente estudo apresenta o caso de um adolescente com 17 anos ao diagnóstico e sítio primário no colón transverso com tumor esporádico, contrário aos dados comumente encontrados na literatura, que referem acometimento mais comum em mulheres na quinta década de vida e com sítio primário em ovário e apêndice. O desenvolvimento de adenocarcinoma mucinoso é raro na população pediátrica e a topografia no cólon transverso e padrão esporádico não familial também são pouco usuais. Conclusão: O caso relatado alerta para as apresentações atípicas da doença e enfatiza o uso de exames de imagem para o diagnóstico, que, se realizado precocemente, impacta de maneira importante o prognóstico e a sobrevida.

Mucinous appendiceal neoplasms: Incidence, diagnosis and surgical treatment. / Tumores mucinosos del apéndice: incidencia, diagnóstico y tratamiento quirúrgico.

INTRODUCTION: Mucinous tumors of the appendix are a rare pathology, with a prevalence below 0.5%. Clinical presentation usually occurs during the sixth decade of life, and mucinous tumors can clinically mimic acute appendicitis. The aim of this study is to describe the clinical and demographic variables, therapeutic procedure and diagnosis of these tumors. We analyze the association between mucinous tumors and pseudomyxoma peritonei (PP), as well as the association with colorectal and ovarian tumors. METHODS: A retrospective study was performed including patients who underwent an appendectomy between December 2003 and December 2014. RESULTS: Seventy-two mucinous tumors of the appendix were identified among 7.717 patients reviewed, resulting in a prevalence of 0.9%. Mean age at presentation was 64 years, 62% patients were female and 38% males. An incidental diagnosis was made in 43% of patients. Mucinous tumors of low malignant potential were significantly related to the presence of pseudomyxoma peritonei, identified in 16 (22%) of the cases. We also observed an increased risk of ovarian mucinous tumors in patients with a diagnosis of appendiceal mucinous neoplasm. In our sample, 22 (30.5%) patients showed a synchronous or metachronous colorectal cancer. CONCLUSIONS: Appendiceal mucinous tumors are frequently an incidental finding. The diagnosis of mucinous tumors of low malignant potential is a factor associated with the development of pseudomyxoma peritonei. Histologic tumor grade and the presence of peritoneal dissemination will determine surgical treatment that can vary, from appendectomy to cytoreductive surgery.

Giant appendiceal mucinous cystadenoma treated by laparoscopy: a case report and review of the literature / Cistadenoma mucinoso apendicular gigante tratado por laparoscopia: relato de caso e revisão de literatura

ABSTRACT Appendiceal mucinous cystadenoma is a rare entity, which causes appendicular mucocele. It is more frequent in women over 50 years old. In half of the cases it is asymptomatic. Tomography of the abdomen is the gold standard in its preoperative diagnosis. The treatment is surgical, with good prognosis, the complete resection evolves without appendicular rupture and extravasation. We report a case of a 64-year-old man with appendiceal mucinous cystadenoma. A laparoscopic right hemicolectomy was performed. This therapy that can be safely used to treat appendiceal mucocele, as long as it is cautious.

RESUMO O cistadenoma mucinoso apendicular é entidade rara que causa mucocele apendicular, sendo mais frequente em mulheres acima dos 50 anos. Em metade dos casos, o cistadenoma mucinoso apendicular é assintomático. A tomografia do abdome é o padrão-ouro para um diagnóstico pré-operatório. O tratamento é cirúrgico e tem bom prognóstico; a ressecção completa evolui sem ruptura apendicular e sem extravasamento. Relatamos um caso de paciente homem de 64 anos com cistadenoma mucinoso apendicular. Foi realizada hemicolectomia laparoscópica direita. Esse é um procedimento que pode ser usado com segurança no tratamento de mucocele apendicular, desde que seja executado com cautela.

Adenocarcinoma of the appendix with extra-appendicular spread: Clinico-surgical and histologic analysis of 27 cases. / Adenocarcinoma de apéndice con extensión extraapendicular: Análisis clínico-quirúrgico e histológico de 27 casos.

BACKGROUND AND OBJECTIVES: Appendiceal neoplasms with extra-appendiceal spread may show different clinical patterns with pseudomyxoma peritonei (PMP) being one of them. We analyse the results in a series of patients treated in our centre. MATERIAL AND METHODS: Retrospective study of patients operated on for appendiceal peritoneal carcinomatosis from January 2012 to May 2015. RESULTS: Twenty-seven consecutive patients were included. Median age 63 years (26-73); 14 were men. Peritoneal carcinomatosis index=16±8 (3-31). The suspected preoperative origins were appendix in 23, ovary in 3 and urothelial in one. Postoperative mortality in 2 patients (7.4%). The remaining 36% presented morbidity. Major morbidity (Clavien-Dindo grades 3 and 4) occurred in 3 patients (12%). CONCLUSIONS: Mucinous adenocarcinomas with extra-appendiceal spread may present as PMP with mucinous ascites, jelly-nodular carcinomatosis without ascites, nodular or desmoplasic plates carcinomatosis without jelly mass/nodules. Histology is not correlated to clinical picture. Preoperative diagnosis of mucinous ovarian cancer in peritoneal carcinomatosis scenario may increase the doubt of their ovarian origin and force an appendiceal origin to be ruled out.

Pseudomixoma peritoneal / Pseudomyxoma peritonei

El pseudomixoma peritoneal es una condición relativamente rara y poco comprendida en la cual la mucina se acumula en la cavidad peritoneal. En forma clásica se caracteriza por una colección difusa gelatinosa abdominal (jelly belly) con implantes mucinosos en la superficie peritoneal. Se presenta un caso de pseudomixoma mucinoso que se originó de un cistadenoma mucinoso de ovario en una mujer de 56 años de edad, con antecedentes de dolor en hipogastrio, náuseas y vómitos. La ecografía pélvica reveló la presencia de una tumoración sólido-quística ecogénica que salía desde el anexo derecho y líquido libre con ecogenicidad mixta en el abdomen. Se realizó laparotomía ginecológica, aspiración del líquido mucinoso e histerectomía, encontrándose cistadenoma mucinoso de ovario y liquido peritoneal mucinoso compatible con pseudomixoma peritoneal.

Pseudomyxoma peritonei is a relatively rare and poorly understood condition in which mucin accumulates within the peritoneal cavity. Classically, it is characterized by diffuse intra-abdominal gelatinous collections ("jelly belly") with mucinous implants on peritoneal surfaces. A case of pseudomyxoma peritonei originated from a mucinous cystadenoma of the ovary is presented in a 56-year-old woman with history of hypogastric pain and abdominal distension, nausea and vomiting. Pelvic ultrasound scan revealed a solid-cystic echogenic mass arising from the right adnexa and free fluid of mixed echogenicity in the abdomen. Gynecological laparotomy, aspiration of mucinous fluid and hysterectomy were performed revealing mucinous cystadenoma of the ovary and mucoid peritoneal fluid compatible with pseudomyxoma peritonei.

Neoplasias mucinosas del apéndice / Mucinous Neoplasm of the Appendix

Se presenta el caso de una paciente femenina de 52 años de edad que consulta por dolor abdominal al servicio de urgencias, donde se le realizan estudios imaginológicos y se sospecha de mucocele. Se confirma el diagnóstico por patología de mucocele del apéndice por neoplasia mucinosa apendicular de bajo grado. En este artículo se describe la presentación clínica de la paciente y se analizan los hallazgos en los estudios por imágenes, en cirugía y en patología. Posteriormente se hace una revisión de la literatura de los tumores mucinosos del apéndice, con énfasis en los hallazgos imaginológicos. Estos tumores son raros, presentan diferente histología y no tienen predilección de género. Se presentan principalmente como abdomen agudo, aunque tienen otras formas de presentación que pueden confundir al clínico. Como se describe, los estudios por imágenes cumplen un papel importante en el diagnóstico, seguimiento y detección de complicaciones.

We present the case of a 52- year- old woman who complains of abdominal pain to the emergency room, ultrasound and CT scan suggested the diagnosis of mucocele and was confirmed by pathology as mucocele of the appendix due low grade appendix mucinous neoplasm. In our review we describe clinical presentation of the patient and discuss imaging findings as well as findings in surgery and pathology. Subsequently, there is a literature review and the mucinous tumors of the appendix, with emphasis in the imaging techniques. These tumors are rare, have different histology and no gender predilection. They occur mainly as acute abdomen, although they have other forms of presentation which may mislead the clinician. As described, imaging techniques play an important role in the diagnosis, follow-up, and detection of complications

[Pseudomyxoma peritonei. Two-case-report]. / Pseudomixoma peritoneal. Reporte de dos casos.

BACKGROUND: Pseudomyxoma peritonei is a disease characterized by an intraperitoneal adenomucinous tumor cell disemination, being cecal appendix the most common etiology. OBJECTIVE: To report a surgeon's group experience and a detailed up to date literature review. CLINICAL CASES: 1. A 74 year old woman with a history of four days with mesogastrium and right lower quadrant abdominal pain associated with diarrhea and fever was admitted to our institution. Upon admission she presented with signs of peritoneal irritation and muscular rigidity, leukocytosis of 14,500 cels/mm(3), 89% neutrophils and 1% bands. An acute appendicitis diagnosis was established, being scheduled for diagnostic laparoscopy, during procedure, significant bowel distention was found, so conversion to laparotomy was required. Case 2. A 73 year old male with a history of one year with intermittent abdominal pain at right iliac fossa and hypogastrium was admitted to our institution. Dyspnea, constipation alternating with periods of diarrhea, gastric fullness and heartburn occurred. On physical examination only a palpable tumor occupying mesogastrium and both iliac fossae was noticed, therefore, a laparotomy was performed. In both cases diagnoses were made during procedure, finding multiple scattered multilobulated mucinous tumor implants within the peritoneal cavity. Histopathological studies confirmed a pseudomyxoma peritonei diagnoses. CONCLUSIONS: Peritoneal pseudomyxoma is a rare malignant tumor with a difficult diagnosis characterized by copious production of mucinous ascites and multiple tumors implants on serosa of intracavitary viscera. The gold standard treatment consists of cytoreductive surgery and perioperative intraperitoneal chemotherapy and/or adjuvant chemotherapy.

Antecedentes: el pseudomixoma peritoneal se caracteriza por la diseminación celular intraperitoneal de un tumor adenomucinoso; el apéndice cecal es la etiología más frecuente. Objetivo: reportar la experiencia de un grupo de Cirugía General y revisar la bibliografía. Casos clínicos: 1: paciente femenina de 74 años de edad, que cuatro días previos inició con dolor abdominal tipo cólico en el mesogastrio y fosa iliaca derecha, evacuaciones diarreicas y fiebre. A su ingreso al hospital se la encontró con datos de irritacion peritoneal y resistencia muscular, leucocitosis de 14,500 células por mm3, neutrófilos 89%, bandas 1%. Se diagnosticó: apendicitis aguda y se programó para laparoscopia diagnóstica, con distensión importante de asas, por lo que se decidió convertir a laparotomía. Caso 2: paciente masculino de 73 años de edad; el padecimiento actual se inició con dolor intermitente en la fosa iliaca derecha e hipogastrio, de un año de evolución. Al cuadro se agregaron: disnea, estreñimiento alternado con periodos de diarrea, plenitud gástrica, y pirosis. A la exploración física se encontró un tumor palpable en el mesogastrio y ambas fosas iliacas. Por eso se le realizó la laparotomía exploradora. En ambos casos el diagnóstico se estableció en el transoperatorio con el hallazgo de múltiples implantes tumorales mucinosos multilobulados diseminados en la cavidad peritoneal, el diagnóstico se confirmó con el reporte histopatológico de pseudomixoma peritoneal. Conclusiones: el pseudomixoma peritoneal es una neoplasia poco frecuente y de difícil diagnóstico, caracterizada por producción copiosa de ascitis mucinosa y de múltiples implantes tumorales en la serosa de las vísceras intracavitarias. El tratamiento ideal es la cirugía citorreductiva y la quimioterapia intraperitoneal perioperatoria o la quimioterapia postoperatoria, o ambas.

Pseudomixoma peritoneal por tumor del apéndice cecal / Peritoneal pseudomyxoma due to cecal appendix tumor

Objetivo: presentar un caso de una patología de muy baja incidencia y realizar una revisión bibliográfica de la misma. Paciente y método: paciente femenina de 87 años de edad que ingresa por guardia por cuadro compatible con hernia inguinal atascada, por lo que se decide cirugía de urgencia. En el acto operatorio se identifica un saco herniario de paredes engrosadas, con contenido intestinal con buena vitalidad, sin signos de necrosis, identificando además abundante secreción mucinosa en el saco herniario y proveniente de la cavidad abdominal. Se decide terminar con la hernioplastia y realizar estudios complementarios diferidos. Luego de los mismos, el comité de tumores decide la realización de nueva cirugía (hemicolectomía derecha con ileotransverso anastomosis por tumor mucinoso de apéndice), durante la cual se constata ascitis gelatinosa. La paciente evoluciona favorablemente y es dada de alta a las 72 horas del post operatorio. La anatomía patológica informa adenocarcinoma mucinoso de apéndice bien diferenciado. Discusión: el Pseudomixoma Peritoneal es una entidad muy poco frecuente, que hace referencia a la diseminación peritoneal de un tumor cuyas células producen gran cantidad de mucina. La supervivencia global de los pacientes es de aproximadamente 75 y 68% durante 5 y 10 años. La clasificación más aceptada es la de Ronnet. Tradicionalmente, el tratamiento ha sido la cirugía de citoreducción, aunque hay autores que proponen procedimientos más agresivos como la peritonectomía más quimioterapia hipertérmica intraoperatoria.

Objective: to present a case of a very low incidence disease and a bibliographic review of it. Patient and method: 87 years old female patient with a compatible history and physical exam for a stuck inguinal hernia, for which we decided to perform emergency surgery. During the surgical procedure, we find a thickened hernia sac, with mucus in it and in the abdominal cavity. The small intestine was vital and not complicated. We finished the hernioplasty, and decided to perform complementary ambulatory studies to the patient. After having them done, we decided, in an interdisciplinary committee, to perform an exploratory surgery, (hemicolectomy with ileum transverse anastomosis for an appendix tumor), during which gelatinous ascites was noticed. The patient evolve favorably and was discharged 72 hours post operation. The surgical biopsy informed an appendix mucinous adenocarcinoma well differentiated. Discussion: the peritoneal pseudomyxoma is a very infrequent entity. The overall survival is 75 and 68% for 5 and 10 years, respectively. Ronnet had published the most accepted histological classification. The accepted treatment is the cytoreductive surgery, although some authors proposed a more aggressive treatment such peritonectomy with hyerthermic intraperitoneal chemotherapy.

[Retroperitoneal mucinous cystadenocarcinoma presenting seven years after appendectomy and right hemicolectomy]. / Cistoadenocarcinoma mucinoso retroperitoneal ocurrido a los 7 años de apendicectomía y hemicolectomía derecha.

Retroperitoneal mucinous cystadenocarcinoma (RMC) with no involvement of neighboring structures is very rare, regardless of whether the lesion is primary or secondary. We report the case of a patient who presented RMC seven years after appendectomy and right hemicolectomy. We discuss both diagnostic possibilities.

Cistoadenocarcinoma de ovario y apéndice / Mucinous cystic adenocarcinoma of the ovary and appendix

We report a 73 years old woman presenting with a right ovarian tumor. Preoperative carcinoembryonic antigen and CA-125 levels were above normal levels. The patient was operated, performing a bilateral oopho-rectomy an appendectomy. The pathological study of the surgical piece reported a mucinous ovarian cystic adenocarcinoma that infiltrated the wall but did not reach the capsule. A mucinous cystadenoma was found in the contralateral ovary. A mucinous adenocarcinoma was detected in the appendix, involving the wall but sparing the serosa. The patient was subjected to chemotherapy and six months after the first operation a tumor relapse was detected. A new excision was carried out and the patient continues with adjuvant chemotherapy. The ovarian an appendiceal tumors could be synchronic or a primary and secondary localization.

Introducción: El cistoadenocarcinoma de apéndice es una neoplasia poco frecuente. La asociación de tumores apendiculares y ováricos es conocida cuando existe pseudomixoma peritoneal. Sin embargo, la relación de la patogénesis entre los tumores es todavía desconocida. Caso clínico: Mujer de 73 años con clínica de plenitud postprandial y dispepsia de un año de evolución. La RMN abdómino-pélvica demuestra una gran tumoración de 15x13x9 cm, politabicada, con origen en ovario derecho. Presenta un CEA preoperatorio de 50 y un CA-125 de 30. Se realizó laparotomía infraumbilical con anexectomía bilateral y apendicectomía. El resultado anátomo-patológico informa de cistoadenocarcinoma mucinoso de ovario bien diferenciado que infiltra casi toda la pared, abierta, sin alcanzar la cápsula ovárica; el ovario contralateral presenta un cistoade-noma mucinoso; el apéndice cecal presenta adenocarcinoma mucinoso bien diferenciado, con infiltración de casi toda la pared, respetando la serosa. Discusión: Presentamos el caso de una tumoración pélvica con diagnóstico anátomo-patológico de cistoadenocarcinoma mucinoso de ovario. Asimismo se demuestra afectación de apéndice cecal por adenocarcinoma mucinoso. Nos planteamos, por tanto, la posibilidad de que se trate de una infiltración metastásica o dos tumores sincrónicos. Revisamos el concepto de cistoadenocarcinoma apendicular y ovárico, y analizamos los posibles mecanismos de asociación entre ellos. Conclusión: Destacamos la necesidad de una exhaustiva revisión de la cavidad abdominal ante este tipo de patología, dada su asociación con otros procesos neoplásicos intraabdominales.

Multimodal treatment of peritoneal malignancies: results of the implantation in a tertiary hospital

INTRODUCTION: Peritoneal carcinomatosis is a condition that may be present in the natural history of colorectal cancer and some other tumors, such as pseudomyxoma peritonei. It has been associated with poor prognosis. The treatment for patients with this condition, up until recently, was systemic chemotherapy or palliative care to relieve the pain and suffering caused by peritoneal dissemination of certain cancers. Promising results, however, have been reported after the implementation of radical cytoreductive surgery followed by peroperative hyperthermic intraperitoneal chemotherapy. OBJECTIVE: To evaluate the results of cytoreductive surgery and peroperative hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with peritoneal carcinomatosis secondary to colorectal cancer and pseudomyxoma peritonei. RESULTS: We retrospectively evaluated 24 patients from 2004 to 2011. Mean age was 51.31 years, and 54% were female. The primary diagnosis in 50.01% of the cases was pseudomyxoma peritonei, 41.66%, of colon cancer, and 8.33%, of mesothelioma. The overall complication rate was around 83%, two patients (8%) subsequently died between the 8th and 30th postoperative day. There was an association between the peritoneal carcinomatosis index (PCI) and operative time. The one-year survival rate in the group examined was 60% for colon cancer, and 78.5% for pseudomyxoma. Overall survival was 50% in three years. CONCLUSION: cytoreductive surgery combined with HIPEC is a treatment option for patients with peritoneal carcinomatosis of colorectal origin and pseudomyxoma. Despite the high rate of peroperative complications (83%), it was possible to achieve a superior survival rate in relation to conventional treatments reported in the literature. (AU)

INTRODUÇÃO: A carcinomatose peritoneal é uma condição que pode estar presente na história natural do câncer colorretal e de algumas outras neoplasias, como o pseudomixoma peritoneal, sendo associada com um prognóstico desfavorável. O tratamento oferecido aos pacientes portadores dessa condição, até recentemente, era a quimioterapia sistêmica ou apenas os cuidados paliativos para aliviar a dor e o sofrimento causados pela disseminação peritoneal de determinadas neoplasias. Resultados promissores, no entanto, têm sido relatados após a implementação da cirurgia citorredutora radical, seguida da quimioterapia hipertérmica intraperitoneal per-operatória. OBJETIVO: Avaliar os resultados da cirurgia citorredutora e da quimioterapia intraperitoneal hipertérmica per-operatória (HIPEC) em pacientes portadores da carcinomatose peritoneal, secundária ao câncer colorretal e ao pseudomixoma peritoneal. RESULTADOS: Foram avaliados, retrospectivamente, 24 pacientes de 2004 a 2011. A idade média foi de 51,31 anos, 54% eram do sexo feminino. O diagnóstico primário em 50,01% dos casos foi de pseudomixoma peritoneal, 41,66% de câncer de cólon e 8,33% de mesotelioma. A taxa de complicação global girou em torno de 83%, sendo que dois pacientes (8%) evoluíram para o óbito no pós-operatório, entre 8 e 30 dias. Houve associação entre o índice de carcinomatose peritoneal (PCI) e o tempo operatório. A sobrevida em um ano, no grupo analisado, foi de 60% para o câncer de cólon e de 78,5% para o pseudomixoma. A sobrevida global foi de 50% em 3 anos CONCLUSÃO: A cirurgia citorredutora combinada com a HIPEC é uma opção de tratamento para pacientes com carcinomatose peritoneal originária de câncer colorretal e de pseudomixoma. A despeito de uma alta taxa de complicação per-operatória (83%) foi possível alcançar uma sobrevida superior a do tratamento convencional relatado na literatura. (AU)

Mucocele of the appendix: appendectomy or colectomy?

Mucocele of the appendix is a rare disease. It can be triggered by benign or malignant diseases, which cause the obstruction of the appendix and the consequent accumulation of mucus secretion. The preoperative diagnosis is difficult due to non-specific clinical manifestations of the disease. Imaging tests can suggest the diagnosis. The treatment is always surgical and depends on the integrity and size of the appendix base and on the histological type of the original lesion. The prognosis is good in cases of integrity of the appendix. The perforation of the appendix and subsequent extravasation of its contents into the abdominal cavity may lead to pseudomyxoma peritonei, which has very poor prognosis if not treated properly. (AU)

A mucocele do apêndice cecal é uma doença rara. Pode ser causada por doenças benignas ou malignas que provocam a obstrução da luz do apêndice e, consequente, acúmulo de secreção mucoide. O diagnóstico pré-operatório é difícil devido ao quadro clínico inespecífico da doença. Exames de imagem sugerem o diagnóstico. O tratamento é sempre cirúrgico e é determinado pela integridade do órgão, dimensões da base e tipo histológico da lesão. O prognóstico é bom nos casos com integridade do apêndice cecal. A perfuração do apêndice e o consequente extravasamento do seu conteúdo para a cavidade abdominal pode ocasionar o pseudomixoma peritoneal, cujo prognóstico é bastante desfavorável se não tratado adequadamente. (AU)

Pseudomixoma peritoneal: relato de caso e revisão da literatura / Pseudomyxoma peritonei: case report and literature review

Pseudomixoma peritoneal (PMP) é uma doença incomum, caracterizada pela presença de coleções líquidas gelatinosas em abdome e pelve, com implantes mucinosos na superfície peritoneal. A maioria dos casos é associada a neoplasias apendiculares. Os sintomas mais importantes são aumento de volume abdominal, emagrecimento, dor abdominal e sintomas mimetizando apendicite aguda. Esta condição clínica progride com disseminação peritoneal, obstrução intestinal e comprometimento nutricional. O caso relatado é de uma paciente feminina, 68 anos, com emagrecimento, aumento de volume abdominal e massa anexial, causando hidronefrose bilateral. Laparotomia exploradora evidenciou massa ocupando cavidades intraperitonial e retroperitonial, originária de tumor apendicular. Após análise histopatológica, o diagnóstico final foi de pseudomixoma peritoneal secundário a neoplasia de apêndice

Pseudomyxoma peritonei (PMP) is an uncommon disease, characterized by the presence of gelatinous collections in abdominal and pelvic cavities, with mucinous implants on peritoneal surface. The majority of PMP cases are associated with appendiceal carcinomas. The most important symptoms are increasing abdominal girth, weight loss, abdominal pain, and symptoms mimicking an acute appendicitis. This entity has a borderline behavior with progression to peritoneal seeding, intestinal obstruction, and nutritional compromise. The case reported is of a 68-year-old woman with weight loss, increasing abdominal girth, and an adnexal mass. Exploratory laparotomy demonstrated a mass occupying intraperitonial and retroperitonial spaces, originating in an appendiceal tumor. After histopathology examination, the final diagnosis was pseudomyxoma peritonei, due to appendiceal tumor