History of pseudomyxoma peritonei from its origin to the first decades of the twenty-first century.

Pseudomyxoma peritonei (PMP) is a disease surrounded by misunderstanding and controversies. Knowledge about the etymology of pseudomyxoma is useful to remove the ambiguity around that term. The word pseudomyxoma derives from pseudomucin, a type of mucin. PMP was first described in a case of a woman alleged to have a ruptured pseudomucinous cystadenoma of the ovary, a term that has disappeared from today's classifications of cystic ovarian neoplasms. It is known today that in the majority of cases, the origin for PMP is an appendiceal neoplasm, often of low histological grade. Currently, ovarian tumors are wrongly being considered a significant recognized etiology of PMP. PMP classification continues to be under discussion, and experts' panels strive for consensus. Malignancy is also under discussion, and it is shown in this review that there is a long-standing historical reason for that. Surgery is the main tool in the treatment armamentarium for PMP, and the only therapy with potential curative option.

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

Fundamento y objetivos: La neoplasia apendicular con extensión extraapendicular puede mostrarse con diferentes patrones clínicos, entre ellos el pseudomixoma peritoneal (PMP). Analizamos los resultados de una serie clínica tratada en nuestro centro. Material y métodos: Estudio retrospectivo de pacientes con carcinomatosis peritoneal apendicular (desde enero de 2012 hasta mayo de 2015). Resultados: Veintisiete pacientes consecutivos. Edad mediana 63 años (26-73), 14 varones. Índice de carcinomatosis peritoneal=16±8 (3-31). El origen tumoral supuesto preoperatoriamente fue el apéndice en 23, el ovario en 3 y urotelial en uno. Mortalidad postoperatoria 2 pacientes (7,4%). El 36% de los restantes presentó morbilidad. Morbilidad mayor (Clavien-Dindo grados 3 y 4) en 3 pacientes (12%). Conclusiones: Los adenocarcinomas mucinosos extraapendiculares pueden manifestarse como PMP con ascitis mucinosa, carcinomatosis nodular gelatinosa sin ascitis, carcinomatosis nodular o placas desmoplásicas sin masas/nódulos gelatinosos. La histología no se correlaciona con la forma de presentación. La sospecha preoperatoria de cáncer de ovario mucinoso en el contexto de carcinomatosis peritoneal obliga a descartar que se trate de metástasis ováricas apendiculares (AU)

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 center. 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 (AU)

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.

Evidence-based Guidelines for the Management of Exocrine Pancreatic Insufficiency After Pancreatic Surgery.

OBJECTIVE: To provide evidence-based recommendations for the management of exocrine pancreatic insufficiency (EPI) after pancreatic surgery. BACKGROUND: EPI is a common complication after pancreatic surgery but there is certain confusion about its frequency, optimal methods of diagnosis, and when and how to treat these patients. METHODS: Eighteen multidisciplinary reviewers performed a systematic review on 10 predefined questions following the GRADE methodology. Six external expert referees reviewed the retrieved information. Members from Spanish Association of Pancreatology were invited to suggest modifications and voted for the quantification of agreement. RESULTS: These guidelines analyze the definition of EPI after pancreatic surgery, (one question), its frequency after specific techniques and underlying disease (four questions), its clinical consequences (one question), diagnosis (one question), when and how to treat postsurgical EPI (two questions) and its impact on the quality of life (one question). Eleven statements answering those 10 questions were provided: one (9.1%) was rated as a strong recommendation according to GRADE, three (27.3%) as moderate and seven (63.6%) as weak. All statements had strong agreement. CONCLUSIONS: EPI is a frequent but under-recognized complication of pancreatic surgery. These guidelines provide evidence-based recommendations for the definition, diagnosis, and management of EPI after pancreatic surgery.

Síndrome del ducto pancreático desconectado: transección completa del páncreas secundaria a pancreatitis aguda / Disconnected pancreatic duct syndrome: complete pancreas transection secondary to acute pancreatitis

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Disconnected pancreatic duct syndrome: complete pancreas transection secondary to acute pancreatitis.

Disconnected pancreatic duct syndrome is a serious complication of acute pancreatitis which is defined by a complete discontinuity of the pancreatic duct, such that a viable side of the pancreas remains isolated from the gastrointestinal tract. This pancreatic disruption is infrequently observed in the clinical practice and its diagnostic and therapeutic management are controversial. We present an extreme case of disconnected pancreatic duct syndrome with complete duct disruption and pancreatic transection following acute pancreatitis, as well as the diagnostic and therapeutic processes carried out.

Implicaciones pronósticas del estudio estandarizado de los márgenes de resección en el cáncer de páncreas / Prognostic implications of the standardized study of resection margins in pancreatic cancers

INTRODUCCIÓN: La afectación microscópica de los márgenes de resección es un factor pronóstico fundamental en la cirugía del cáncer de páncreas. Sin embargo, su definición anatomopatológica no está estandarizada. Este estudio pretende identificar el porcentaje real de pacientes con resecciones R1 al analizar las piezas quirúrgicas con un protocolo estandarizado y evaluar sus implicaciones sobre la supervivencia. PACIENTES Y MÉTODOS: Serie de 100 pacientes consecutivos intervenidos por adenocarcinoma ductal de páncreas y resecciones macroscópicamente completas, divididos en 2 grupos: pre- y posprotocolo, según se intervinieran antes o después de la aplicación de un protocolo estandarizado de las piezas de resección. RESULTADOS: En el grupo preprotocolo la tasa de resecciones R0 fue del 78%, mientras que tras la aplicación del mismo, se redujo al 47% (p = 0,003). El margen posterior retroperitoneal es el que se encuentra afectado con mayor frecuencia. En los casos con tumores localizados en cabeza de páncreas y analizados con el protocolo estandarizado, la detección del margen retroperitoneal afecto (R1) influye de forma negativa en la supervivencia. La mediana de supervivencia del grupo R0 fue de 22 meses frente a 16 meses en los que presentaban margen afecto (HR: 2,044; IC 95% 1,00-4,16; p = 0,043). CONCLUSIONES: La aplicación de un protocolo estandarizado para el estudio del margen retroperitoneal en el cáncer de páncreas incrementa la proporción de pacientes R1. En los pacientes con cáncer de cabeza de páncreas, la afectación del margen posterior retroperitoneal reduce significativamente la supervivencia

INTRODUCTION: Involvement of surgical resection margins is a fundamental prognostic factor in pancreatic oncological surgery. However, there is a lack of standardized histopathology definition. The aims of this study are to investigate the real rate of R1 resections when surgical specimens are evaluated according to a standardized protocol and to study its survival implications. Patients y methods: One hundred consecutive surgically resected patients with pancreatic ductal adenocarcinoma were included in the study. They were further divided in 2 groups: pre-protocol, evaluated before the introduction of the standardized protocol and post-protocol, analyzed with the standardized protocol. RESULTS: R0 resection rate in the pre-protocol group was 78%, falling to 47% after the introduction of the standardized protocol (p = 0,003). The posterior retroperitoneal margin was the most frequently involved margin. In cases with tumors located at the pancreatic head and analyzed according to the standardized protocol R1 involvement negatively affected survival. Median survival in the R0 group was 22 months versus 16 in those with the margin involved (HR: 2.044; IC 95% 1,00-4,16; P=.043). CONCLUSIONS: Standardized evaluation of the retroperitoneal margins in pancreatic cancer increases the rate of R1 patients. In cases with pancreatic cancer located at the pancreatic head involvement of posterior retroperitoneal margin significantly decreases survival

Considerations on pancreatic exocrine function after pancreaticoduodenectomy.

The pancreaticoduodenectomy (PD) procedure may lead to pancreatic exocrine and endocrine insufficiency. There are several types of reconstruction for this kind of operation. Pancreaticogastrostomy (PG) was introduced to reduce the rate of postoperative pancreatic fistula. Although some randomized control trials have shown no differences regarding pancreatic leakage between PG and pancreaticojejunostomy (PJ), recently some reports reveal benefits from the PG over the PJ. Some surgeons concern about the performing of the PG and inactivation of pancreatic enzymes being in contact with the gastric juice, and the detrimental results over the exocrine pancreatic function. The pancreatic exocrine function can be measured with direct and indirect tests. Direct tests have the highest sensitivity and specificity for detection of exocrine insufficiency but require tube placement. Among the tubeless indirect tests, the van de Kamer stool fat analysis remains the standard to diagnose fat malabsorption. The patient compliance and time consuming makes it not so suitable for its clinical use. Fecal immunoreactive elastase test is employed for screening of exocrine insufficiency, is not cumbersome, and has been used to study pancreatic function after resection. We analyze the FE1 levels in our patients after the PD with two types of reconstruction, PG and PJ, and we discuss some considerations about the pancreaticointestinal drainage method after pancreaticoduodenectomy.

Prognostic implications of the standardized study of resection margins in pancreatic cancers.

INTRODUCTION: Involvement of surgical resection margins is a fundamental prognostic factor in pancreatic oncological surgery. However, there is a lack of standardized histopathology definition. The aims of this study are to investigate the real rate of R1 resections when surgical specimens are evaluated according to a standardized protocol and to study its survival implications. PATIENTS Y METHODS: One hundred consecutive surgically resected patients with pancreatic ductal adenocarcinoma were included in the study. They were further divided in 2 groups: pre-protocol, evaluated before the introduction of the standardized protocol and post-protocol, analyzed with the standardized protocol. RESULTS: R0 resection rate in the pre-protocol group was 78%, falling to 47% after the introduction of the standardized protocol (p=0,003). The posterior retroperitoneal margin was the most frequently involved margin. In cases with tumors located at the pancreatic head and analyzed according to the standardized protocol R1 involvement negatively affected survival. Median survival in the R0 group was 22 months versus 16 in those with the margin involved (HR: 2.044; IC 95% 1,00-4,16; P=.043). CONCLUSIONS: Standardized evaluation of the retroperitoneal margins in pancreatic cancer increases the rate of R1 patients. In cases with pancreatic cancer located at the pancreatic head involvement of posterior retroperitoneal margin significantly decreases survival.

Indicaciones quirúrgicas en los pólipos de vesícula biliar / Surgical indications in gallbladder polyps

Introducción La cirugía por pólipo de vesícula biliar está poco definida debido a la carencia de guías clínicas basadas en la evidencia. Objetivo Analizar el manejo de los pólipos en nuestro medio, y revisión de la literatura y estándares de tratamiento. Material y métodos De la base de datos de Patología se extrajeron los informes de colecistectomía con pólipos vesiculares (PV). De la base de datos de Cirugía se identificó a pacientes intervenidos con diagnóstico de PV. Se confeccionó un listado único y se llevó a cabo la revisión de las historias clínicas incluyendo edad, sexo, clínica, informe ecográfico e informe anatomopatológico. Resultados Se incluyó a 30 pacientes, mediana de edad 51 años (rango 22-83), 21 mujeres. En 19 pacientes el diagnóstico ecográfico fue PV, 7 de PV y litiasis, y 4 de litiasis sin pólipo. Otros diagnósticos concurrentes con PV fueron hemangiomas múltiples (3), gran quiste simple único (1), quistes simples múltiples (1). Once pacientes tuvieron dolor típico (origen biliar), 5 de los cuales sin litiasis ecográfica. Ocho presentaron dolor inespecífico, persistiendo en 3 tras la colecistectomía. Se encontraron pseudopólipos en 20 vesículas, y pólipos verdaderos en 4 casos. En 3 casos no se hallaron pólipos en el examen patológico. Conclusiones El informe ecográfico debe especificar el tamaño, forma y número de pólipos. Los pacientes con dolor biliar típico se beneficiarán de una colecistectomía. Ante un PV menor de 10mm y edad menor de 50 años la probabilidad de malignidad es mínima y no requiere colecistectomía. Los PV mayores de 10mm deben ser indicación de colecistectomía (AU)

Introduction The surgery of gallbladder polyps is not well defined due to the lack of evidence-based clinical guidelines. Objective To analyse the management of polyps in Spain, and a review of the literature and treatment standards. Material and methods The reports on cholecystectomy with gallbladder polyps (GBP) were extracted from the Pathology data base. Patients subjected to surgery with a diagnosis of GBP were identified in the Surgery data base. A single list was prepared and a review was made of the clinical histories, including, age, gender, clinical data, ultrasound report, and histopathology report. Results A total of 30 patients, with a median age of 51 years (range 22-83), 21 of whom were female, were included. The ultrasound diagnosis was GBP in 19 patients, GBP and calculi in 7 cases, and calculi with no polyps in 4 cases. Other diagnoses concurrent with GBP were multiple haemangiomas (3), large single simple cyst (1), and multiple simple cysts (1). Eleven patients had typical pain (biliary origin), 5 of which showed no calculi on ultrasound. Eight had non-specific pain, which persisted in 3 cases after the cholecystectomy. Pseudopolyps were found in 20 gallbladders, and true polyps in 4 cases. In 3 cases, polyps were not found in the pathology study. Conclusions The ultrasound report must specify the size, shape, and number of polyps. Patients with biliary type pain would benefit from a cholecystectomy. The probability of malignancy is minimum if the GBP is less than 10mm and aged under 50 years, and a cholecystectomy is not required. A GBP greater than 10mm should be an indication of cholecystectomy (AU)

[Surgical indications in gallbladder polyps]. / Indicaciones quirúrgicas en los pólipos de vesícula biliar.

INTRODUCTION: The surgery of gallbladder polyps is not well defined due to the lack of evidence-based clinical guidelines. OBJECTIVE: To analyse the management of polyps in Spain, and a review of the literature and treatment standards. MATERIAL AND METHODS: The reports on cholecystectomy with gallbladder polyps (GBP) were extracted from the Pathology data base. Patients subjected to surgery with a diagnosis of GBP were identified in the Surgery data base. A single list was prepared and a review was made of the clinical histories, including, age, gender, clinical data, ultrasound report, and histopathology report. RESULTS: A total of 30 patients, with a median age of 51 years (range 22-83), 21 of whom were female, were included. The ultrasound diagnosis was GBP in 19 patients, GBP and calculi in 7 cases, and calculi with no polyps in 4 cases. Other diagnoses concurrent with GBP were multiple haemangiomas (3), large single simple cyst (1), and multiple simple cysts (1). Eleven patients had typical pain (biliary origin), 5 of which showed no calculi on ultrasound. Eight had non-specific pain, which persisted in 3 cases after the cholecystectomy. Pseudopolyps were found in 20 gallbladders, and true polyps in 4 cases. In 3 cases, polyps were not found in the pathology study. CONCLUSIONS: The ultrasound report must specify the size, shape, and number of polyps. Patients with biliary type pain would benefit from a cholecystectomy. The probability of malignancy is minimum if the GBP is less than 10mm and aged under 50 years, and a cholecystectomy is not required. A GBP greater than 10mm should be an indication of cholecystectomy.

Leiomioma perineal grande en gestante de 9 semanas / Giant perineal leiomyoma in the 9th week of pregnancy

Los leiomiomas perineales son tumores infrecuentes de origen mesenquimal clasificados en somáticos y retroperitoneales. Los retroperitoneales son los que afectan sobre todo a las mujeres durante el período perimenopáusico. El diagnóstico va dirigido a diferenciarlos de los leiomiosarcomas y de los tumores estromales gastrointestinales. Presentamos el caso de una mujer de 30 años de edad, gestante de 9 semanas. Consulta por una tumoración de crecimiento progresivo en espacio isquiorrectal derecho que coincide con el embarazo, pero paucisintomático. Se abordó por vía perineal y se practicó una resección en bloque de un tumor de 9 cm (AU)

Perineal leiomyoma are rare mesenchymal tumours classified as somatic or retroperitoneal. The retroperitoneal variety are mainly related to women during the peri-menopausal phase. Diagnosis is directed at differentiating them from leiomyosarcomas and gastrointestinal stromal tumours. We report on a case of a 30-year-old and 9-week pregnant woman. She consulted due to a progressive growing mass in the right ischiorrectal fossa coinciding with pregnancy, but with sparse symptoms. Surgery was performed through a perineal access with a complete resection of a 9 cm tumour (AU)

Respuesta del autor: Tratamiento del carcinoma de vesícula incidental / Author’s reply: Management of incidental gallbladder carcinoma

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