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Resumen Caso clínico sobre un paciente masculino, 35 años, deportista, conocido sano, que presenta una historia clínica con síntomas gastrointestinales indeterminados, a quien se le realizan estudios complementarios, detectándose una lesión fusiforme, de apariencia quística, con contenido seroso, de aproximadamente 9cm, perteneciente al apéndice ileocecal. Inicialmente, debido a los hallazgos, en los estudios realizados a nuestro paciente, se sospecha de mucocele apendicular. Según la teoría el mucocele apendicular se origina de una obstrucción del apéndice ileocecal, lo cual provoca una acumulación retrograda de material mucinoso y eso ocasiona una dilatación de la luz apendicular, convirtiéndose en una masa quística. Con dicho diagnóstico presuntivo, se realiza apendicectomía con hemicolectomía derecha. El abordaje quirúrgico que se decide para este caso es la cirugía abierta versus laparoscópica, debido al riesgo de cistoadenocarcinoma mucinoso. Posteriormente se envía la lesión para realización de estudio patológico, donde se evidencia una neoplasia mucinosa de bajo grado del apéndice.

Abstract Clinical case of a 35-year-old male patient, an athlete, known to be healthy, who presented a clinical history with indeterminate gastrointestinal symptoms, who underwent complementary studies, detecting a fusiform lesion with a cystic appearance, with serous content, approximately 9cm, belonging to the ileocecal appendix. Initially, due to the findings in the studies carried out on our patient, an appendiceal mucocele was suspected. According to the theory, the appendicular mucocele originates from an obstruction of the ileocecal appendix, which causes a retrograde accumulation of mucinous material and this causes a dilation of the appendicular lumen, becoming a cystic mass. With this presumptive diagnosis, appendectomy with right hemicolectomy was performed. The surgical approach chosen for this case is open versus laparoscopic surgery, due to the risk of mucinous cystadenocarcinoma. Subsequently, the lesion is sent for a pathological study, where a low-grade mucinous neoplasm of the appendix is evidenced.

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Pseudomyxoma peritonei (PMP) refers to a growth disorder characterized by glycoprotein neoplasm in the peritoneum, where mucin oversecretion occurs. The tumors of the appendix region are well associated with PMP; however, ovarian, colon, stomach, pancreas, and urachus tumors have also been linked to PMP. Other mucinous tumors in the pelvis, paracolic gutters, greater omentum, retrohepatic space, and Treitz ligament can be the reason for PMP. Despite being rare and having a slow growth rate, PMP can be lethal without treatment. It is treated with neoadjuvant chemotherapy with the option of cytoreductive surgery and intraperitoneal chemotherapy. In the current study, we hypothesize that there may be novel gentle ways to inhibit or eliminate the mucin. Dr. David Morris has used mucolytics - such as bromelain and N-acetyl cysteine to solubilize mucin. In the present review, we aimed to study the regulation of mucin expression by promoter methylation, and drugs that can inhibit mucin, such as boldine, amiloride, naltrexone, dexamethasone, and retinoid acid receptors antagonist. This review also explored some possible pathways, such as inhibition of Na + , Ca2+ channels and induction of DNA methyltransferase along with inhibition of ten-eleven translocation enzymes, which can be good targets to control mucin. Mucins are strong adhesive molecules that play great roles in clinging to cells or cell to cell. Besides, they have been greatly involved in metastasis and also act as disease markers for cancers. Diagnostic markers may have exclusive roles in disease initiation and progression. Therefore, the present review explores various drugs to control and target mucin in various diseases, specifically cancers. (AU)

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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.

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.

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Pseudomyxoma peritonei (PMP) is a condition presenting with tumors of the abdominal cavity presenting which could lead abdominal distention and ascites secondary to mucus production. Tumors of this type are potentially fatal due to their obstructing of abdominal structures. The current management of the condition includes surgical debulking with intraoperative or postoperative chemotherapy with protocols such as hyperthermic intraperitoneal chemotherapy and early postoperative intraperitoneal chemotherapy (EPIC), respectively. We describe herein a case in which a tumor debulking surgery was performed. Afterwards, we placed intraperitoneal catheters so that a 4-day regimen of bedside intraperitoneal 5-fluorouracil chemotherapy could be administered on her bedside. Chemotherapy was infused and removed with the use of Hemovac and Jackson-Pratt drainage catheter systems attached to suction, for the intrahospital management of recurrent PMP in a young Hispanic female patient. Though it requires further study, we propose this method as a safe and effective alternative to current strategies at low income or resources centers.

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The objective of this systematic review is to provide efficacy and safety data in the application of Intra-Abdominal Hyperthermia Chemotherapy (HIPEC) and Cytoreductive Surgery (CRS) in patients with Peritoneal Pseudomyxoma (PMP) of origin in the cecal appendix. The databases Medline and Central Cochrane were consulted. Patients with PMP of origin in the cecal appendix, classified as low grade, high or indeterminate, submitted to HIPEC and CRS. The results were meta-analyzed using the Comprehensive Metanalysis software. Twenty-six studies were selected to support this review. For low-grade PMP outcome, 60-month risk of mortality, Disease-Free Survival (DFS), and adverse events was 28.8% (95% CI 25.9 to 32), 43% (95% CI 36.4 and 49.8), and 46.7% (95% CI 40.7 to 52.8); for high-grade PMP, 60-month risk of mortality, Disease-Free Survival (DFS) and adverse events was 55.9% (95% CI 51.9 to 59.6), 20.1% (95% CI 15.5 to 25.7) and 30% (95% CI 25.2 to 35.3); PMP indeterminate degree, 60-month risk of mortality, Disease-Free Survival (DFS) and adverse events was 32.6% (95% CI 30.5 to 34.7), 61.8% (95% CI 58.8 to 64.7) and 32.9% (95% CI 30.5 to 35.4). The authors conclude that the HIPEC technique and cytoreductive surgery can be applied to selected cases of patients with PMP of peritoneal origin with satisfactory results.

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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.

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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.

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Abstract The objective of this systematic review is to provide efficacy and safety data in the application of Intra-Abdominal Hyperthermia Chemotherapy (HIPEC) and Cytoreductive Surgery (CRS) in patients with Peritoneal Pseudomyxoma (PMP) of origin in the cecal appendix. The databases Medline and Central Cochrane were consulted. Patients with PMP of origin in the cecal appendix, classified as low grade, high or indeterminate, submitted to HIPEC and CRS. The results were meta-analyzed using the Comprehensive Metanalysis software. Twenty-six studies were selected to support this review. For low-grade PMP outcome, 60-month risk of mortality, Disease-Free Survival (DFS), and adverse events was 28.8% (95% CI 25.9 to 32), 43% (95% CI 36.4 and 49.8), and 46.7% (95% CI 40.7 to 52.8); for high-grade PMP, 60-month risk of mortality, Disease-Free Survival (DFS) and adverse events was 55.9% (95% CI 51.9 to 59.6), 20.1% (95% CI 15.5 to 25.7) and 30% (95% CI 25.2 to 35.3); PMP indeterminate degree, 60-month risk of mortality, Disease-Free Survival (DFS) and adverse events was 32.6% (95% CI 30.5 to 34.7), 61.8% (95% CI 58.8 to 64.7) and 32.9% (95% CI 30.5 to 35.4). The authors conclude that the HIPEC technique and cytoreductive surgery can be applied to selected cases of patients with PMP of peritoneal origin with satisfactory results.

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INTRODUCTION AND IMPORTANCE: Low-Grade Appendiceal Mucinous Neoplasms (LAMN) are noninvasive epithelial tumors of the appendix. Symptomatology is unspecific and can simulate appendicitis. The multidisciplinary approach for the correct diagnosis and management of this kind of tumor is important, as the prognosis depends on it. PRESENTATION OF THE CASE: A 70-years-old man with an appendiceal picture is presented. McBurney and Blumberg's signs were found and the Echography has reported an acute appendicitis. He underwent laparoscopic surgery, founding a Tumor in the Cecum of 10 × 5 cm. We decided to perform an appendectomy with a total cecectomy. The pathological diagnosis was a LAMN. Ten months later, a Computed Tomography (CT) Scan control was performed reporting no evidence of Tumoral activity or Distance Metastasis, neither pseudomyxoma peritonei (PMP) evidence. DISCUSSION: LAMN has been a very controversial tumor. The definitive management is appendectomy. There are controversies about the laparoscopic and open approach, and when there are positive margins. The stage was a pTis (LAMN) pNx according to the 8th edition of the American Joint Committee on Cancer (AJCC). The prognosis depends on the tumoral stage and the presence of PMP. In our case, we have performed a CT scan follow up and there was no Tumoral activity or Distance Metastasis, neither PMP evidence. CONCLUSION: LAMN is a very interesting and rare tumor. The diagnosis of this case was a real challenge. Our management was simple due to the tumoral stage. Pathology played a very important role in treatment and prognosis.

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Abstract Objective: To determine the most common imaging features of pseudomyxoma peritonei (PMP), as well as the histologic subtypes of the primary tumors. Materials and Methods: We reviewed 30 cases of women with pathologically confirmed PMP. Only computed tomography scans were available. All cases were retrospectively studied by four radiologists, working independently. We identified the most common imaging findings, the predominant primary site of the disease, and the growth pattern. The most common sites of recurrence were also analyzed. Results: The most common computed tomography finding was peritoneal/omental nodules (including "omental caking"), followed by visceral scalloping and non-mucinous ascites. The most common site of the primary tumor was the appendix (in 63.3%), followed by the ovaries (in 16.6%), and 16.6% of the tumors were of undetermined origin. There was one case of synchronous appendiceal and ovarian tumors. Low-grade mucinous neoplasm was the most common histologic subtype, accounting for 84.2% of the appendiceal tumors and 40% of the ovarian tumors. Conclusion: Although PMP is a relatively rare entity, radiologists must be aware of its possible imaging findings, common locations, and possible patterns of recurrence. The origin of the primary tumor should also be investigated. Future studies are needed in order to determine which preoperative imaging findings predict surgical outcomes and to characterize the main findings of radiological recurrence.

Resumo Objetivo: Determinar as características de imagem mais frequentes do pseudomixoma peritonial (PMP), bem como os subtipos histológicos dos tumores primários. Materiais e Métodos: Foram revisados 30 casos confirmados patologicamente de PMP em mulheres. Somente a tomografia computadorizada estava disponível. Todos os casos foram estudados de forma independente e retrospectiva por quatro radiologistas. Os autores relataram os achados de imagem mais frequentes, a localização predominante da doença primária e o padrão de crescimento. Os locais de recorrência mais comuns também foram analisados. Resultados: Os achados tomográficos mais frequentes foram nódulos peritoniais/omentais (incluindo "bolo omental"), seguidos por scalloping visceral e ascite não mucinosa. Os padrões de localização mais comuns do PMP também foram documentados. A grande maioria dos tumores primários foi de origem apendicular (63,3%), seguida de origem ovárica (16,6%) e indeterminada (16,6%). Houve um caso síncrono de tumor apendicular e ovário. A neoplasia mucinosa de baixo grau foi o subtipo histológico mais frequente, representando 84,2% dos tumores do apêndice e 40% dos tumores primários ovarianos. Conclusão: Embora o PMP continue sendo uma entidade relativamente rara, o radiologista deve estar ciente de seus possíveis achados de imagem, locais comuns e possíveis padrões de recorrência. A investigação do tumor primário também deve ser encorajada. Futuros estudos são necessários para prever o resultado cirúrgico da imagem pré-operatória e caracterizar os principais achados de recorrência radiológica.

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OBJECTIVE: To determine the most common imaging features of pseudomyxoma peritonei (PMP), as well as the histologic subtypes of the primary tumors. MATERIALS AND METHODS: We reviewed 30 cases of women with pathologically confirmed PMP. Only computed tomography scans were available. All cases were retrospectively studied by four radiologists, working independently. We identified the most common imaging findings, the predominant primary site of the disease, and the growth pattern. The most common sites of recurrence were also analyzed. RESULTS: The most common computed tomography finding was peritoneal/omental nodules (including "omental caking"), followed by visceral scalloping and non-mucinous ascites. The most common site of the primary tumor was the appendix (in 63.3%), followed by the ovaries (in 16.6%), and 16.6% of the tumors were of undetermined origin. There was one case of synchronous appendiceal and ovarian tumors. Low-grade mucinous neoplasm was the most common histologic subtype, accounting for 84.2% of the appendiceal tumors and 40% of the ovarian tumors. CONCLUSION: Although PMP is a relatively rare entity, radiologists must be aware of its possible imaging findings, common locations, and possible patterns of recurrence. The origin of the primary tumor should also be investigated. Future studies are needed in order to determine which preoperative imaging findings predict surgical outcomes and to characterize the main findings of radiological recurrence.

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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.

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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.

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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.

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BACKGROUND: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) for pseudomyxoma peritonei and appendix tumours are widespread in the world. It is unclear what should be the attitude in elderly patients. METHODS: This retrospective multicenter study collected the database from ten Spanish centers from Spanish Group of Peritoneal Cancer Surgery. The study period was between November 2002 and March 2014. Seventeen patients with age greater than or equal to 75 years with peritoneal carcinomatosis from pseudomyxoma peritonei and appendix tumours met the selection criteria for the study. Outcomes in terms of morbidity and mortality such as disease-free and overall survival were analyzed. RESULTS: Median PCI was 16 (range 6-39). Ten postoperative adverse events were detected in nine patients (44.4%). 28% were grade I-II and 17% were grade III-IV. Disease-free survival at 1 and 3 years was 67 and 44%, respectively. Overall survival at 1 and 3 years was 100 and 88%, respectively. Only cytoreduction was related to worst disease free survival after univariate (p = 0.007) and multivariate (OR 11.639, 95% CI 1.24-109.74, p = 0.03) analyses. Cytoreduction was related to the worst overall survival after univariate analysis (p = 0.046). CONCLUSION: Cytoreductive surgery and HIPEC for pseudomyxoma peritonei and appendix tumours in elderly patients it is a procedure with feasible postoperative morbi-mortality and survival outcomes. TRIAL REGISTRATION: researchregistry1587 (retrospectively registered).

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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.

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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.

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Los tumores mucosos apendiculares tienen baja incidencia y comúnmente se diagnostican en el estudio anatomo-patólogico después de la apendicectomía. Se reporta el caso de una mujer de 41 años de edad, con un cuadro clínico de ocho meses de evolución, caracterizado por dolor abdominal de tipo opresivo, difuso y de gran intensidad en el hemiabdomen inferior, acompañado de náuseas. Después de cinco meses de iniciado este cuadro clínico, se evidenció una masa en la fosa iliaca derecha; el dolor se agudizó e intensificó, y las náuseas continuaron, por lo cual fue remitida al hospital. En los exámenes practicados se observó una masa quística compleja abdomino-pélvica de origen indeterminado, y la tomografía computadorizada de abdomen fue sugestiva de mucocele apendicular. Con estos hallazgos, se optó por el tratamiento quirúrgico por laparotomía, consistente en hemicolectomía derecha, con resección parcial de íleon, epiplectomía, histerectomía y salpigooforectomía bilateral.

The clinical manifestations of an appendicular mucous tumors are non specific. The vast majority are associated with complications of intrabdominal rupture causing acute abdomen, while the spectrum associated while the associated with extrinsic compression of adjacent organic structures is exceptional. We report a case of partial intestinal obstruction caused by an appendiceal mococele fistulized to the proximal ileum.

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The epithelial appendiceal neoplasms are uncommon and are usually detected as an unexpected surgical finding. The general surgeon should be aware of the diversity of its clinical manifestations and biological behaviors along with the significance of the surgical treatment on the progression of the illness and the prognosis of the patients. The operative findings and, especially, tumor histology, determine the type of surgery. Intestinal histologic subtype behaves and should be treated similarly to the right colon neoplasms; while mucinous tumors, often discordant between histology and its aggressiveness, can be treated with a simple appendectomy or require complex oncological surgeries. Mucinous tumors are often associated with the presence of mucin or tumor implants in the abdominal cavity, being the clinical syndrome known as pseudomyxoma peritonei (PMP). PMP tends to present an indolent but deadly evolution and requires a multimodal approach as a single treatment with curative potential: complete cytoreductive surgery plus hyperthermic Intra-peritoneal chemotherapy (CCRS + HIPEC) now considered the standard of care in this pathology. The general surgeon should be aware of the governing principles of the treatment of appendiceal neoplasms with or without peritoneal dissemination, know the therapeutic frontiers in every situation (avoiding unnecessary or counterproductive surgeries) and sending early these patients to specialised centres in the radical management of malignant diseases of the peritoneum in the conditions and with the necessary information to facilitate a possible radical treatment.

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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.

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