Surgical Principles of Primary Retroperitoneal Sarcoma in the Era of Personalized Treatment: A Review of the Frontline Extended Surgery.

Surgery is the key treatment in retroperitoneal sarcoma (RPS), as completeness of resection is the most important prognostic factor related to treatment. Compartmental surgery/frontline extended approach is based on soft-tissue sarcoma surgical principles, and involves resecting adjacent viscera to achieve a wide negative margin. This extended approach is associated with improved local control and survival. This surgery must be tailored to tumor histology, tumor localization, and patient performance status. We herein present a review of compartmental surgery principles, covering the oncological and technical basis, and describing the tailored approach to each tumor subtype and localization in the retroperitoneum.

National survey on the treatment of sarcomas in Spain.

Surgical units attending sarcomas in Spain are poor studied. The aim is to know the management of this pathology to identify areas of improvement through multicenter study based on a voluntary survey. The survey was completed by 74 surgeons of different hospitals, which 32,4% is exclusively dedicated to sarcomas. Only 24.3% declared to receive specific training in sarcomas. The most frequent type of hospital was the third level (56.8%), where 38,1% of the surgeons belong to societies or working-groups in sarcoma fields vs. 9,4% in first-second levels. The number of surgeons with specific theoretical training and papers published in this field are higher in third level hospitals. 55,4% belonged to a multidisciplinary unit. A multidisciplinary team was available in 57% of third level hospital vs 28% in others. Most services in charge of this patients are characterized by deficient specialization, low workload and the absence of a multidisciplinary team.

Pancreatic endocrine tumors or apudomas.

INTRODUCTION AND OBJECTIVE: pancreatic endocrine tumors (PET) are difficult to diagnose. Their accurate localization using imaging techniques is intended to provide a definite cure. The goal of this retrospective study was to review a PET series from a private institution. PATIENTS AND METHODS: the medical records of 19 patients with PETs were reviewed, including 4 cases of MEN-1, for a period of 17 years (1994-2010). A database was set up with ten parameters: age, sex, symptoms, imaging techniques, size and location in the pancreas, metastasis, surgery, complications, adjuvant therapies, definite diagnosis, and survival or death. RESULTS: a total of 19 cases were analyzed. Mean age at presentation was 51 years (range: 26-67 y) (14 males, 5 females), and tumor size was 5 to 80 mm (X: 20 mm). Metastatic disease was present in 37% (7/19). Most underwent the following imaging techniques: ultrasounds, computed tomography (CT) an magnetic resonance imaging (MRI). Fine needle aspiration punction (FNA) was performed for the primary tumor in 4 cases. Non-functioning: 7 cases (37%), insulinoma: 2 cases [1 with possible multiple endocrine neoplasia (MEN)], Zollinger-Ellison syndrome (ZES) from gastrinoma: 5 (3 with MEN-1), glucagonoma: 2 cases, 2 somatostatinomas; carcinoid: 1 case with carcinoide-like syndrome. Most patients were operated upon: 14/19 (73%). Four (4/14:28%) has postoperative complications following pancreatectomy: pancreatitis, pseudocyst, and abdominal collections. Some patients received chemotherapy (4), somatostatin (3) and interferon (2) before or after surgery. Median follow-up was 48 months. Actuarial survival during the study was 73.6% (14/19). CONCLUSIONS: age was similar to that described in the literature. Males were predominant. Most cases were non-functioning (37%). Most patients underwent surgery (73%) with little morbidity (28%) and an actuarial survival of 73.6% at the time of the study.

National survey on the treatment of sarcomas in Spain. / Encuesta nacional sobre el tratamiento de los sarcomas en España.

Surgical units attending sarcomas in Spain are poor studied. The aim is to know the management of this pathology to identify areas of improvement through multicenter study based on a voluntary survey. The survey was completed by 74 surgeons of different hospitals, which 32.4% is exclusively dedicated to sarcomas. Only 24.3% declared to receive specific training in sarcomas. The most frequent type of hospital was the third level (56.8%), where 38.1% of the surgeons belong to societies or working-groups in sarcoma fields vs. 9.4% in first-second levels. The number of surgeons with specific theoretical training and papers published in this field are higher in third level hospitals. 55.4% belonged to a multidisciplinary unit. A multidisciplinary team was available in 57% of third level hospital vs. 28% in others. Most services in charge of these patients are characterized by deficient specialization, low workload and the absence of a multidisciplinary team.

Systematic review on abdominal aortic aneurysm screening cost-efficiency and methodological quality assessment.

INTRODUCTION: Abdominal aortic aneurysm (AAA) is a silent, progressive disease that can lead to death. It is easily diagnosed with noninvasive methods and its routine treatment has excellent results. This creates an optimal situation for population screening programs. The aim of this paper was to assess results and methodological quality of cost-utility studies on screening versus no screening scenarios for AAA to assess future establishment of new AAA screening programs. EVIDENCE ACQUISITON: A systematic review of efficiency (cost-effectiveness and cost-utility) studies was performed, finally selecting cost-utility studies on AAA screening versus no screening. Papers were selected that dealt with efficiency of screening for AAA according to PICOTS framework and the methodological quality assessed according to the economic evaluation analyses described by Drummond and Caro. Two independent reviewers were involved in the procedure. EVIDENCE SYNTHESIS: Research retrieved 88 studies. From those, 26 showed cost-effectiveness and cost-utility results. Finally, 10 studies had cost-utility results and suited criteria (published in the last 10 years; time-horizon: 10 years or more) for exhaustive analysis. All publications, except one, showed adequate incremental cost-utility ratios according to different national perspectives. Methodological assessment showed some quality limitations, but the majority of items analyzed were favorably answered after applying the questionnaires. CONCLUSIONS: Confirmation of the cost-utility results in this revision at a national/regional level should be the basis for the implantation of new national screening programs worldwide. The methodological evaluation applied in this revision is crucial for the corresponding future piggy-back trials to assess routine application of national AAA screening programs.

Encuesta nacional sobre el tratamiento de los sarcomas en España / National survey on the treatment of sarcomas in Spain

Las unidades encargadas de los sarcomas en España están poco estudiadas. El objetivo es conocer el manejo de esta patología para identificar áreas de mejora mediante un estudio multicéntrico basado en una encuesta voluntaria.La encuesta fue completada por 74 cirujanos de centros diferentes. El 32,4% se dedican exclusivamente a los sarcomas. Solo el 24,3% han recibido formación específica. El hospital más frecuente fue el tercer nivel (56,8%), donde el 38,1% de los cirujanos pertenecen a sociedades/grupos de trabajo específicos vs. 9,4% en segundo-primer nivel. El número de cirujanos con formación teórica específica y artículos publicados en este campo es mayor en los de tercer nivel. El 55,4% pertenecen a una unidad multidisciplinar. Los equipos multidisciplinares están disponibles en el 57% de los hospitales terciarios vs. el 28% en los demás.La mayoría de los servicios que atienden esta patología presentan escasa especialización, baja carga de trabajo y carecen de equipos multidisciplinares (AU)

Surgical units attending sarcomas in Spain are poor studied. The aim is to know the management of this pathology to identify areas of improvement through multicenter study based on a voluntary survey.The survey was completed by 74 surgeons of different hospitals, which 32.4% is exclusively dedicated to sarcomas. Only 24.3% declared to receive specific training in sarcomas. The most frequent type of hospital was the third level (56.8%), where 38.1% of the surgeons belong to societies or working-groups in sarcoma fields vs. 9.4% in first-second levels. The number of surgeons with specific theoretical training and papers published in this field are higher in third level hospitals. 55.4% belonged to a multidisciplinary unit. A multidisciplinary team was available in 57% of third level hospital vs. 28% in others.Most services in charge of these patients are characterized by deficient specialization, low workload and the absence of a multidisciplinary team (AU)

Laparoscopic liver surgery: 8 years of multicenter Spanish register.

BACKGROUND: To present the data of laparoscopy in liver surgery and to assess the real indications and outcomes of this kind of approach. METHODS: From February 2000 to March 2008, a prospective study was performed on 182 patients from 15 Spanish surgical centres. RESULTS: A total of 308 lesions was collected. The mean age was 57 years old, and 61.5% were female. Among patients with cystic lesions we found: 45 simple cysts, 19 policystic diseases, 12 hydatidic cysts and 2 cystoadenomas. Among solid lesions (n = 104), we found 34 patients with benign pathology (12 focal nodular hyperplasia, 10 adenomas, 7 haemangyomas and 5 other lesions) and 70 with malignant pathology (38 metastases, 29 hepatocellular carcinomas, 2 cholangiocarcinomas and 1 lymphoma). The global rate of conversion was 8.8%. The global morbidity rate was 14.8%, and 5 of them required re-intervention. CONCLUSIONS: Nowadays there are strong criteria for patients being submitted to laparoscopy procedures based both on type and location features. The postoperative morbidity rate is low, also for hepatocellular carcinoma in cirrhotic liver. In case of malignant pathology, we think the use of ultrasonography is mandatory to obtain a free margin, which implies a long-term survival rate.

[Curative partial hepatectomy in adult Caroli's disease]. / Hepatectomía parcial curativa en la enfermedad de Caroli del adulto.

Caroli's disease consists of a congenital malformation of the intrahepatic bile ducts characterized by saccular, segmental, cystic dilatations giving rise to recurrent cholangitis. The inheritance pattern is unclear and the disease may be associated with other hereditary diseases such as adult polycystic kidney disease. The most effective therapeutic option in symptomatic unilobar Caroli's disease is resection of the affected lobe. In bilobar involvement, liver transplantation should be considered. Due to their rarity, we report three cases of Caroli's disease successfully resolved through lobectomy of the affected lobe: two patients with recurrent cholangitis and one patient with associated adult polycystic kidney disease.

[Inversion of the sequence of surgery after neoadjuvant chemotherapy for synchronous liver metastases from colorectal cancer]. / Cirugía secuencial inversa después de quimioterapia neoadyuvante por metástasis hepáticas sincrónicas de cáncer colorrectal.

In some patients with colorectal cancer and synchronous liver metastases, chemotherapy and current combinations of chemotherapy allow the size of these metastases to be reduced so that they can be surgically resected. However, in many patients, the initial systematic treatment of the primary tumor is associated with growth of the metastases (which predict the patient's life expectancy). This metastatic growth contraindicates surgical treatment that might otherwise be curative. We report the case of a patient with advanced recto-colonic cancer, which responded well to chemotherapy given as neoadjuvant treatment prior to surgery, in which the hepatic metastases were resected before excision of the primary tumor.

[Surgical resection of liver metastases from colorectal carcinoma. Experience in Sant Pau Hospital]. / Resección quirúrgica de metástasis hepáticas de cáncer colorrectal. Experiencia del Hospital de Sant Pau.

INTRODUCTION: Surgical resection is the only available treatment that improves survival in patients with liver metastases from colorectal cancer, particularly when carried out by a multidisciplinary team. MATERIAL AND METHOD: We retrospectively analyzed a consecutive series of 116 patients who underwent 138 liver resections (65.4% minor and 35.5% major) for hepatic metastases from colorectal cancer between 1998 and 2004. In 34.5% of the patients, the lesions were synchronous. All patients were individually assessed by a multidisciplinary team. The mean number of metastases removed per patient was 2.43 (range: 1-10). The mean size of the largest tumor per patient was 40 mm (range: 12-90). In 67.3% of the patients, the primary tumor was at an advanced stage (III-IV). In 98% of the patients, the diagnosis was confirmed by helical computed tomography scans/magnetic resonance imaging and intraoperative ultrasonography. RESULTS: Postoperative morbidity was 31.2% and mortality was 2.2%. A mean of 2.7 units of blood was transfused per patient. Overall 5-year survival was 43.2% (median 50 months). Survival rates varied according to whether the patients had < 4 or > or = 4 colorectal liver metastases (50 and 43 months respectively), tumor size (more or less than 5 cm) (60 and 50.6 months respectively) and whether the site was monolobar or bilobar (60 and 43.11 months respectively). In 16 patients, recurrence of liver metastases led to 22 rehepatectomies. Overall 5-year survival was 36.7% (median 60 months) after the first rehepatectomy but was 36 and 12 months respectively after a second or third rehepatectomy. CONCLUSIONS: These results confirm that multidisciplinary decisions and interventions by specialist liver surgeons, as in our hospital, reduce postoperative morbidity and mortality and increase survival in patients requiring surgical removal of liver metastases from colorectal cancer.

Tumores endocrinos o apudomas pancreáticos / Pancreatic endocrine tumors or apudomas

Introducción y objetivo: los tumores endocrinos pancreáticos (TEP) son difíciles de diagnosticar. Su localización exacta mediante métodos de imagen tiene el propósito de lograr una curación definitiva. El objetivo de este trabajo retrospectivo fue revisar una serie institucional privada de TEP. Pacientes y métodos: se revisaron las historias clínicas de 19 pacientes con TEP, 4 casos con NEM-1, observados durante 17 años (1994-2010). Se creó una base de datos con diez parámetros: edad y sexo, síntomas, métodos diagnósticos de imagen, tamaño y situación en el páncreas, metástasis, cirugía, complicaciones, tratamientos complementarios, diagnóstico definitivo, supervivencia o éxitus. Resultados: en total se analizaron 19 casos. La edad media de presentación fue 51 años (intervalo: 26-67 años) (14 varones y 5 mujeres), con un tamaño del tumor de 5 a 80 mm (X: 20 mm). El 37% (7/19) tenían metástasis. En la mayoría se practicaron los siguientes métodos de imagen: ecografía, TAC y RM. La PAAF del tumor primitivo se practicó en 4 casos. No funcionantes: 7 casos (37%), insulinomas: 2 casos (1 con posible NEM), SZE por gastrinomas: 5 (3 con NEM-1), glucagonoma: 2 casos, 2 somatostatinomas, carcinoide: 1 caso con síndrome carcinoide-like. La mayoría de los enfermos fueron intervenidos quirúrgicamente 14/19 (73%). En cuatro (4/14: 28%) pacientes hubo complicaciones postoperatorias después de pancreatectomías: páncreas, seudoquiste y colecciones abdominales. Algunos casos fueron tratados con quimioterapia (4), somatostatina (3) e interferón (2) antes o después de la cirugía. La mediana de seguimiento fue de 48 meses. La supervivencia actuarial en el momento del estudio fue del 73,6% (14/19). Conclusiones: la edad fue similar a lo descrito en la literatura. El sexo predominante fue el masculino. La mayoría fueron no funcionantes (37%). La mayoría fueron intervenidos quirúrgicamente (73%), con escasa morbilidad (28%) y con una supervivencia actuarial en el momento de cerrar el estudio del 73,6%(AU)

Introduction and objective: pancreatic endocrine tumors (PET) are difficult to diagnose. Their accurate localization using imaging techniques is intended to provide a definite cure. The goal of this retrospective study was to review a PET series from a private institution. Patients and methods: the medical records of 19 patients with PETs were reviewed, including 4 cases of MEN-1, for a period of 17 years (1994-2010). A database was set up with ten parameters: age, sex, symptoms, imaging techniques, size and location in the pancreas, metastasis, surgery, complications, adjuvant therapies, definite diagnosis, and survival or death. Results: a total of 19 cases were analyzed. Mean age at presentation was 51 years (range: 26-67 y) (14 males, 5 females), and tumor size was 5 to 80 mm (X: 20 mm). Metastatic disease was present in 37% (7/19). Most underwent the following imaging techniques: ultrasounds, computed tomography (CT) and magnetic resonance imaging (MRI). Fine needle aspiration punction (FNA) was performed for the primary tumor in 4 cases. Nonfunctioning: 7 cases (37%), insulinoma: 2 cases [1 with possible multiple endocrine neoplasia (MEN)], Zollinger-Ellison syndrome (ZES) from gastrinoma: 5 (3 with MEN-1), glucagonoma: 2 cases, 2 somatostatinomas; carcinoid: 1 case with carcinoide-like syndrome. Most patients were operated upon: 14/19 (73%). Four (4/14: 28%) has postoperative complications following pancreatectomy: pancreatitis, pseudocyst, and abdominal collections. Some patients received chemotherapy (4), somatostatin (3) and interferon (2) before or after surgery. Median follow-up was 48 months. Actuarial survival during the study was 73.6% (14/19). Conclusions: age was similar to that described in the literature. Males were predominant. Most cases were non-functioning (37%). Most patients underwent surgery (73%) with little morbidity (28%) and an actuarial survival of 73.6% at the time of the study(AU)

[Analysis of the Spanish National Registry of Laparoscopic Pancreatic Surgery]. / Análisis del Registro Nacional Español de la Cirugía Laparoscópica del Páncreas.

INTRODUCTION: The reported experience with laparoscopic pancreatic surgery remains limited to case reports or small series of patients. A recent European multicenter study has allowed the limits and results of this technique to be known. This article presents an analysis of the results of the Spanish National Registry of Laparoscopic Pancreatic Surgery. MATERIAL AND METHODS: A total of 132 patients with lesions in the left pancreas were included in this series. The final diagnosis included 42 neuroendocrine tumors, 40 cystic neoplasms, 24 cysts and pseudocysts, 8 inflammatory tumors, 8 ductal carcinomas, 7 intraductal papillary mucinous tumors, 1 acinar carcinoma and 2 solid pseudopapillary tumors. RESULTS: The conversion rate was 9.7%. Tumor enucleation was performed only in patients with insulinomas. The most frequent technique was spleen-preserving distal pancreatectomy. There were no postoperative deaths. The overall rate of postoperative pancreatic-related complications was 16%. CONCLUSIONS: Although only a few Spanish hospitals participated in the registry, a greater number of hospitals are expected to enroll patients in the very near future.

[Solid-pseudopapillary tumor of pancreas]. / Tumor sólido seudopapilar del páncreas.

Solid-pseudopapillary tumor of pancreas is a very rare neoplasm of low malignant potential and unknown origin. It generally occurs in young women and the prognosis is usually good after complete surgical removal. Two cases of solid pseudopapillary tumor with distinct disease duration are reported.

[Intraductal papillary mucinous neoplasms of the pancreas]. / Neoplasia papilar intraductal mucinosa del páncreas.

INTRODUCTION: Mucin-producing pancreatic tumors account for 1% of all malignant tumors of the pancreas. Notable among these are intraductal papillary mucinous neoplasms of the pancreas, due to their variable clinical behavior, increased frequency, and the controversy surrounding the most suitable treatment and diagnostic methods. The aim of this study was to analyze the clinical presentation, epidemiological and pathological features, diagnostic methods, treatment and survival rates of these tumors. PATIENTS AND METHOD: We performed a prospective review of patients with a diagnosis of intraductal papillary mucinous neoplasm of the pancreas between 1996 and 2001. Demographic and clinical characteristics, diagnostic tests, surgical treatment, tumor characteristics and survival rates were studied. RESULTS: There were 8 patients. The mean age was 65 years. The most common clinical presentation was abdominal pain. The most frequent localization was the pancreatic head. The most common diagnostic methods used were abdominal computed tomography, endoscopic retrograde pancreatography, and serum CA 19.9 levels. Seven patients underwent pancreatic resection and only one patient was not surgically treated. The mean follow-up period was 38 months with a survival rate of 75% and a recurrence rate of 25%. CONCLUSION: Intraductal papillary mucinous neoplasms of the pancreas generally appear in the sixth decade of life. The most common presenting symptom is abdominal pain. These neoplasms typically occur in the head of the pancreas. The most commonly used diagnostic test is computed tomography and the main treatment is early surgical resection. The prognosis is good after complete resection, with a 5-year survival rate of 60-70%.

Pancreatic duct after pancreatoduodenectomy: morphologic and functional evaluation with secretin-stimulated MR pancreatography.

OBJECTIVE: The purpose of this study was to evaluate the usefulness of secretin-enhanced MR pancreatography in evaluating morphologic changes and pancreatic function after pancreatoduodenectomy. SUBJECTS AND METHODS: Twenty patients who underwent pancreatoduodenectomy were evaluated with secretin MR pancreatography. Single-shot fast spin-echo T2-weighted dynamic MR pancreatograms were obtained before and every minute for 10 min after secretin injection. Image analysis included image quality for the visualization of the pancreatic duct and morphologic features of the pancreatic duct (side branches, ductal narrowing, irregular ductal contour, and patency of the anastomotic site). Pancreatic function was assessed using the Van de Kamer method and the fasting blood glucose and oral glucose tolerance tests. Jejunal filling was graded from the lowest amount of filling (grade 1) to normal filling (grade 3) on the last MR pancreatogram. RESULTS: The visualization of the main pancreatic duct was significantly improved with dynamic MR pancreatography (p < 0.05). The anastomotic site was visualized in 14 patients (70%) on MR pancreatography after secretin administration (p < 0.05). No statistically significant improvement in other morphologic data was seen after secretin administration. The sensitivity, specificity, positive predictive value, and negative predictive value of reduced jejunal filling (grade 1) for assessment of reduced pancreatic exocrine function were 92%, 71%, 85%, and 83%, respectively. The relation between reduced jejunal filling (grade 1) and diabetes was statistically significant (p < 0.05). CONCLUSION: The administration of secretin improves visualization of the pancreatic ducts and helps in the evaluation of remnant pancreatic function after pancreatoduodenectomy.

Hepatectomía parcial curativa en la enfermedad de Caroli del adulto / Curative partial hepatectomy in adult Caroli's disease

La enfermedad de Caroli consiste en una malformación congénita de la vía biliar intrahepática caracterizada por dilataciones saculares, segmentarias múltiples y quísticas, cuyas manifestaciones clínicas se centran en crisis repetitivas de colangitis. Esta enfermedad tiene una base hereditaria incierta y puede asociarse a otras enfermedades de carácter hereditario como la enfermedad poliquística renal del adulto (EPRAD). La mejor opción terapéutica en los casos sintomáticos unilobares hepáticos es la exéresis hepática del lóbulo afecto correspondiente; en caso de afección bilobar, debe plantearse el trasplante hepático. Presentamos por su rareza 3 casos de enfermedad de Caroli resueltos con éxito mediante una lobectomia hepática del lado afecto: 2 pacientes manifestaron la enfermedad en forma de episodios de colangitis de repetición y 1 paciente, con asociación a EPRAD (AU)

Caroli's disease consists of a congenital malformation of the intrahepatic bile ducts characterized by saccular, segmental, cystic dilatations giving rise to recurrent cholangitis. The inheritance pattern is unclear and the disease may be associated with other hereditary diseases such as adult polycystic kidney disease. The most effective therapeutic option in symptomatic unilobar Caroli's disease is resection of the affected lobe. In bilobar involvement, liver transplantation should be considered. Due to their rarity, we report three cases of Caroli's disease successfully resolved through lobectomy of the affected lobe: two patients with recurrent cholangitis and one patient with associated adult polycystic kidney disease (AU)

Cirugía secuencial inversa después de quimioterapia neoadyuvante por metástasis hepáticas sincrónicas de cáncer colorrectal / Inversion of the sequence of surgery after neoadjuvant chemotherapy for synchronous liver metastases from colorectal cancer

En algunos pacientes afectos de cáncer colorrectal con metástasis hepáticas sincrónicas, la quimioterapia y sus nuevas combinaciones permiten reducir el tamaño de las metástasis y rescatarlas para un tratamiento quirúgico. Sin embargo, en muchos pacientes, el tratamiento inicial sistemático del tumor primario se acompaña de un crecimiento de las metástasis (que marcan el pronóstico vital del paciente). Esta progresión de las metástasis impide cualquier opción de tratamiento con intención curativa. Presentamos el caso de un paciente con enfermedad colorrectal avanzada, con muy buena respuesta a la quimioterapia y candidato a una novedosa estrategia, que combina neoadyuvancia primero, con cirugía de las metástasis hepáticas que se adelanta a la cirugía colorrectal (AU)

In some patients with colorectal cancer and synchronous liver metastases, chemotherapy and current combinations of chemotherapy allow the size of these metastases to be reduced so that they can be surgically resected. However, in many patients, the initial systematic treatment of the primary tumor is associated with growth of the metastases (which predict the patient's life expectancy). This metastatic growth contraindicates surgical treatment that might otherwise be curative. We report the case of a patient with advanced recto-colonic cancer, which responded well to chemotherapy given as neoadjuvant treatment prior to surgery, in which the hepatic metastases were resected before excision of the primary tumor (AU)