Recommendations in the management of epithelial appendiceal neoplasms and peritoneal dissemination from mucinous tumours (pseudomyxoma peritonei)

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 Intraperitoneal 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 (AU)

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Growing teratoma syndrome: an unusual evolution of treated ovarian immature teratoma - a case report.

Growing teratoma syndrome (GTS) is a rare condition among germ cell tumor (GCT) patients during treatment with systemic chemotherapy. It is characterized by the development of enlarging masses, the normalization of tumor markers, and the presence of only mature teratoma in the pathological specimen. The authors present the unusual case of a 15-year-old girl with an immature teratoma treated with conventional surgery and systemic chemotherapy. On her follow up, although tumor markers returned to normal, there was an enlargement of abdomino-pelvic masses confirmed by a PET/TC study. With the diagnosis of a GTS, the patient underwent a com- plete cytoreduction. Histologically, all the specimens contained mature teratoma tissue. The patient remains clear with no signs of recurrence with no further treatment. The knowledge and awareness of this syndrome are highlighted in order to prevent further unnecessary chemotherapy and allow an optimal cytoreduction, which seems to be the most effective therapy so far.

Recommendations in the management of epithelial appendiceal neoplasms and peritoneal dissemination from mucinous tumours (pseudomyxoma peritonei).

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.

Accuracy and clinical relevance of computed tomography scan interpretation of peritoneal cancer index in colorectal cancer peritoneal carcinomatosis: a multi-institutional study.

BACKGROUND: Evaluation of peritoneal metastases by computed tomography (CT) scans is challenging and has been reported to be inaccurate. METHODS: A multi-institutional prospective observational registry study of patients with peritoneal carcinomatosis from colorectal cancer was conducted and a subset analysis was performed to examine peritoneal cancer index (PCI) based on CT and intraoperative exploration. RESULTS: Fifty-two patients (mean age 52.6 ± 12.4 years) from 16 institutions were included in this study. Inaccuracies of CT-based assessment of lesion sizes were observed in the RUQ (P = 0.004), LLQ (P < 0.0005), RLQ (P = 0.003), distal jejunum (P = 0.004), and distal ileum (P < 0.0005). When CT-PCI was classified based on the extent of carcinomatosis, 17 cases (33%) were underestimations, of which, 11 cases (21%) were upstaged from low to moderate, 4 cases (8%) were upstaged from low to severe, and 2 cases (4%) were upstaged from moderate to severe. Relevant clinical discordance where an upstage occurred to severe carcinomatosis constituted a true inaccuracy and was observed in six cases (12%). CONCLUSIONS: The actual clinical impact of inaccuracies of CT-PCI was modest. CT-PCI will remain as a mandatory imaging tool and may be supplemented with other tools including positron emission tomography scan or diagnostic laparoscopy, in the patient selection for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

Giant perineal hernia: laparoscopic mesh repair complemented by a perineal cutaneous approach.

Symptomatic perineal herniation after abdominoperineal resection is a rare complication and its management remains challenging. Perineal laparoscopic mesh repair is safe and effective and can be performed with minimal complications. We report a giant perineal hernia treated by a combined laparoscopic mesh repair approach and plastic resection of the cutaneous perineal wound. To the best of our knowledge, this is the first report with this technical approach that we could find in the English literature.

The European contribution to "Sugarbaker's protocol" for the treatment of colorectal peritoneal carcinomatosis.

INTRODUCTION: In 1981, Dr. PH Sugarbaker, challenging oncological orthodoxy, considered carcinomatosis to be a locoregional stage of the disease that was still susceptible to treatment with curative intent. To this end he developed a new therapeutic alternative based on the combined treatment. The macroscopic disease treated by maximum radical oncological cytoreductive surgery (through the peritonectomies described by him), followed by treatment of the residual microscopic disease with the direct intra-abdominal application of intraoperative chemotherapy with locoregional intensification, modulated by hyperthermia and early normothermic postoperative intra-abdominal chemotherapy. Using this new therapeutic regimen, known as "Sugarbaker s Protocol", his group has reported 45% survival rates in carcinomatosis of colorectal origin at 5 years, and, in selected groups of patients, 50% survival rates at 5 years. The scientific community, however, has criticized these results considering that: it is a personal experience, with a not homogenous treatment protocol with developmental modifications over time, that it is a retrospective non-randomized study, and finally that the cytostatics used in his protocol are obsolete. Various European groups have replied to these main criticisms confirming the good results that this new therapeutic alternative offers for patients with carcinomatosis of colorectal origin. The purpose of this article is to present these contributions. MATERIAL AND METHODS: All the articles published in the English language by European groups in the world s medical literature have been reviewed using the Pubmed-MEDLINE database to identify the relevant articles related to the treatment of carcinomatosis of colorectal origin using cytoreduction and intraperitoneal chemotherapy from January 1980 to January 2008. RESULTS: The European contribution during these 25 years in favour of the "Sugarbaker s Protocol" has consisted fundamentally in: a) one multicenter retrospective study; b) two randomized prospective phase III studies; and c) the use of oxaliplatin and irinotecan as new cytostatic agents in the protocols for intraperitoneal chemotherapy. At the same time, two new transcendental European contributions have been made in which the possibility has been considered of combined simultaneous treatment for patients with hepatic metastases and carcinomatosis, and the introduction, as a selection factor, of patients responsive to intravenous induction chemotherapy within the regimen of sandwich treatment (with systemic neoadjuvant and adjuvant chemotherapy) complementary to intraperitoneal chemotherapy. CONCLUSIONS: The results obtained by European groups using "Sugarbaker s protocol" and "Elias protocol" with oxaliplatin compel us to request that these treatments be considered by all professionals involved in the treatment of patients with colorectal carcinomatosis as the best treatment currently available for this condition. Furthermore a randomized, prospective, multicenter study should be carried out to clarify its value and the degree of scientific evidence. A validation of this treatment will change, in the future, the dogmatic consideration of carcinomatosis as an incurable disease stage.

Aportación europea al “protocolo de Sugarbaker” en el tratamiento de la carcinomatosis peritoneal colorrectal / The European contribution to "Sugarbaker's protocol" for the treatment of colorectal peritoneal carcinomatosis

Introducción: el Dr. P. H. Sugarbaker en 1981, desafiando la ortodoxia oncológica, consideró la carcinomatosis como un estadio locorregional de la enfermedad susceptible todavía de tratamiento con intención curativa. Para ello desarrolló una nueva alternativa terapéutica basada en el tratamiento combinado. La enfermedad macroscópica mediante la máxima cirugía citorreductora radical oncológica (merced a las peritonectomías por él descritas), seguido del tratamiento de la enfermedad microscópica residual con la aplicación directa intraabdominal, de quimioterapia de intensificación locorregional, intraoperatoria modulada por hipertermia y de quimioterapia intraabdominal normotérmica postoperatoria precoz. Con este nuevo esquema terapéutico, conocido como "Protocolo de Sugarbaker", su grupo ha publicado supervivencias en carcinomatosis de origen colorrectal de 45% a 5 años y en grupos selectos de pacientes supervivencia de 50% a 5 años. La comunidad científica, sin embargo, ha criticado estos resultados al considerar que: se trata de una experiencia personal, con un protocolo de tratamiento no homogéneo con modificaciones evolutivas en el tiempo, tratarse de un estudio retrospectivo no randomizado, y finalmente considerar que los citostáticos empleados en su protocolo son obsoletos. Diversos grupos europeos han dado respuesta a las principales objeciones, confirmando los buenos resultados que esta nueva alternativa terapéutica ofrece en pacientes con carcinomatosis de origen colorrectal. El objetivo de este trabajo es presentar estas aportaciones. Material y métodos: se han revisado todos los artículos publicados en lengua inglesa por grupos europeos en la literatura médica mundial usando la base de datos Pubmed-MEDLINE para identificar los artículos relevantes relacionados con el tratamiento de la carcinomatosis de origen colorrectal mediante citorreducción y quimioterapia intraperitoneal desde enero de 1980 a enero de 2008...(AU)

Introduction: in 1981, Dr. PH Sugarbaker, challenging oncological orthodoxy, considered carcinomatosis to be a locoregional stage of the disease that was still susceptible to treatment with curative intent. To this end he developed a new therapeutic alternative based on the combined treatment. The macroscopic disease treated by maximum radical oncological cytoreductive surgery (through the peritonectomies described by him), followed by treatment of the residual microscopic disease with the direct intra-abdominal application of intraoperative chemotherapy with locoregional intensification, modulated by hyperthermia and early normothermic postoperative intra-abdominal chemotherapy. Using this new therapeutic regimen, known as "Sugarbaker's Protocol", his group has reported 45% survival rates in carcinomatosis of colorectal origin at 5 years, and, in selected groups of patients, 50% survival rates at 5 years. The scientific community, however, has criticized these results considering that: it is a personal experience, with a not homogenous treatment protocol with developmental modifications over time, that it is a retrospective non-randomized study, and finally that the cytostatics used in his protocol are obsolete. Various European groups have replied to these main criticisms confirming the good results that this new therapeutic alternative offers for patients with carcinomatosis of colorectal origin. The purpose of this article is to present these contributions. Material and methods: all the articles published in the English language by European groups in the world's medical literature have been reviewed using the Pubmed-MEDLINE database to identify the relevant articles related to the treatment of carcinomatosis of colorectal origin using cytoreduction and intraperitoneal chemotherapy from January 1980 to January 2008...(AU)

Pseudomixoma peritoneal. Supervivencia superior a 10 años tras citorreducción, quimioterapia intraperitoneal e hipertermia / No disponible

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Prevalencia y tratamiento de la patología oncológica en el anciano. El reto que se avecina / No disponible

La esperanza media de vida en España se ha más que duplicadoa lo largo del siglo XX, llegando en la actualidad a 75 años enel varón y 83 años en la mujer. Las predicciones de la evolución ycrecimiento poblacional a nivel mundial y nacional auguran unshock demográfico cuando en España el número de mayores de65 años alcance el 33,5% de la población en el 2050.Es conocido que el cáncer está intrínseca y directamente relacionadocon la edad, siendo una patología de personas mayores,ya que al menos el 60% de ellos aparecen en mayores de 65años. Los ancianos constituyen el grupo más importante de lapráctica oncológica médica. Las predicciones de envejecimientode la población española, permiten intuir que el cáncer en el ancianoy su tratamiento deba ser considerado un problema sanitariode primer orden.El cáncer ya no es sinónimo de muerte para la mayoría de lospacientes. Globalmente el 60% se curan o cronifican. Esta mejorapronóstico tiene un precio tanto en las expectativas de tratamientocomo de posterior seguimiento y mantenimiento de las secuelasque pueden generarse.El mayor y mejor conocimiento y comprensión del proceso deenvejecimiento, permitirá identificar y seleccionar aquellos pacientesmayores que pueden beneficiarse de medidas de prevención ytratamiento, y lo más importante permitirá identificar aquellos pacientesque no son candidatos de tratamientos con intención curativapor tratarse de población frágil.Los adelantos en el campo de la cirugía, especialmente en lacirugía mínimamente invasiva y su aplicación al campo de la cirugíaoncológica, permiten prever que un mayor número de pacientesancianos podrán beneficiarse de un tratamiento con intencióncurativa. La edad no prevendrá de tratamientos apropiados en individuosancianos con cáncer, especialmente aquellos en los quetengan una adecuada expectativa de vida y reserva funcional.Combatir esta discriminación sanitaria constituye una de las principalesprioridades en la estrategia para el mantenimiento de lasalud del anciano. Presentamos y analizamos en este trabajo los cambios poblacionalesque se avecinan de forma particular en España, la vinculacióndel cáncer con la edad con sus particularidades específicas,los criterios generales de fragilidad del anciano, las limitacionesque la edad suponen para la aplicación de distintos tratamientosadyuvantes complementarios, y las nuevas alternativas quirúrgicasaplicables en pacientes oncológicos ancianos en los cánceres másfrecuentes de la práctica clínica

Life expectancy in Spain has more than duplicated during thelast 20th Century, and is currently 75 years for men and 83 yearsfor women. Predictions on the evolution of the National and globalpopulation anticipate a demographic shock in Spain when individualsolder than 65 years eventually make up more than 33.5%of the Spanish population by year 2050.It is known that cancer is directly related with age, and that itis a disease of older people –at least 60% of all cancers are diagnosedin patients older than 65 years. The older people group isthe most important group of patients in oncologic practice today.Predictions on the aging of the Spanish population showthat cancer in the aging patient and its treatment must be considereda first-line health problem. The diagnosis of cancer isnot associated with death in the majority of patients. Sixty percentof cancers are globally cured or chronified. This advancedprognosis has its toll not only in the expectancy of treatment butalso in subsequent follow-up and post-treatment adverse effectsthat can be generated.A greater and better knowledge and understanding of the agingprocess will allow to identify and select those old patientsthat can benefit from prevention and treatment options, andmore importantly will identify those other patients that are notcandidates to treatments with curative intention because of theirfrail status.Progress in surgery, mainly in minimally invasive surgery, andits application to the field of oncologic surgery allows to forecastthat a greater number of aging patients will benefit from treatmentwith curative intent. Age will not be a barrier for adequate treatmentin healthy elderly patients, especially in those with long lifeexpectancy and functional reserves. Fighting this healthcare discriminationis one of the main priorities in the strategy of improvedhealth in the elderlyWe present in this study and analyze the foreseen changes in theworld’s population, particularly in Spain; the association between cancer and age with its peculiar specificities; the general criteria forfrailty in older patients, the limitations that aging generate for adjuvanttreatments, and the new alternatives of treatment to be used inelderly oncologic patients for the most frequent tumors

Respuesta de autores: Réplica: Lesión iatrogénica de un conducto aberrante del sectorial anterior derecho hepático independiente. Dificultad en su diagnóstico y tratamiento / No disponible

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Lesión iatrogénica de un conducto aberrante del sectorial anterior derecho hepático (segmentos V-VIII) independiente. Dificultades en su diagnóstico y tratamiento / No disponible

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[Prevalence and treatment of oncologic disease in the elderly --an impeding challenge]. / Prevalencia y tratamiento de la patología oncológica en el anciano. El reto que se avecina.

Life expectancy in Spain has more than duplicated during the last 20th Century, and is currently 75 years for men and 83 years for women. Predictions on the evolution of the National and global population anticipate a demographic shock in Spain when individuals older than 65 years eventually make up more than 33.5% of the Spanish population by year 2050. It is known that cancer is directly related with age, and that it is a disease of older people -at least 60% of all cancers are diagnosed in patients older than 65 years. The older people group is the most important group of patients in oncologic practice today. Predictions on the aging of the Spanish population show that cancer in the aging patient and its treatment must be considered a first-line health problem. The diagnosis of cancer is not associated with death in the majority of patients. Sixty percent of cancers are globally cured or chronified. This advanced prognosis has its toll not only in the expectancy of treatment but also in subsequent follow-up and post-treatment adverse effects that can be generated. A greater and better knowledge and understanding of the aging process will allow to identify and select those old patients that can benefit from prevention and treatment options, and more importantly will identify those other patients that are not candidates to treatments with curative intention because of their frail status. Progress in surgery, mainly in minimally invasive surgery, and its application to the field of oncologic surgery allows to forecast that a greater number of aging patients will benefit from treatment with curative intent. Age will not be a barrier for adequate treatment in healthy elderly patients, especially in those with long life expectancy and functional reserves. Fighting this healthcare discrimination is one of the main priorities in the strategy of improved health in the elderly. We present in this study and analyze the foreseen changes in the world s population, particularly in Spain; the association between cancer and age with its peculiar specificities; the general criteria for frailty in older patients, the limitations that aging generate for adjuvant treatments, and the new alternatives of treatment to be used in elderly oncologic patients for the most frequent tumors.

Treatment of peritoneal carcinomatosis from ovarian cancer. Present, future directions and proposals.

Peritoneal carcinomatosis, considered years ago as a final stage of unresectable cancer, can now be managed with curative intention by means of a radical cytoreductive surgical procedure with associated peritonectomy and intraperitoneal chemotherapy, as described by Sugarbaker. Malignant neoplasms such as mesothelioma and pseudomyxoma peritonei, ovarian and colon cancer nowadays are experiencing some new therapeutical approaches. Higher survival rates can be reached in ovarian cancer, which is commonly diagnosed in the presence of peritoneal carcinomatosis, using an optimal cytoreductive radical surgery with intraperitoneal chemotherapy. An actualised review of the treatment of advanced ovarian cancer and a proposal of a national multicentre protocol for the treatment of peritoneal carcinomatosis from ovarian cancer has been performed by a group of Spanish surgeons and oncologists dedicated to a therapeutical approach to this pathology.

Treatment of peritoneal carcinomatosis from ovarian cancer. Present, future directions and proposals

Peritoneal carcinomatosis, considered years ago as a final stage of unresectable cancer, can now be managed with curative intention by means of a radical cytoreductive surgical procedure with associated peritonectomy and intraperitoneal chemotherapy, as described by Sugarbaker. Malignant neoplasms such as mesothelioma and pseudomyxoma peritonei, ovarian and colon cancer nowadays are experiencing some new therapeutical approaches. Higher survival rates can be reached in ovarian cancer, which is commonly diagnosed in the presence of peritoneal carcinomatosis, using an optimal cytoreductive radical surgery with intraperitoneal chemotherapy. An actualised review of the treatment of advanced ovarian cancer and a proposal of a national multicentre protocol for the treatment of peritoneal carcinomatosis from ovarian cancer has been performed by a group of Spanish surgeons and oncologists dedicated to a therapeutical approach to this pathology (AU)

Management of peritoneal surface malignancy with cytoreductive surgery and perioperative intraperitoneal chemotherapy.

AIMS: A new treatment strategy combining maximal cytoreductive surgery for treatment of macroscopic disease and maximal perioperative intraperitoneal chemotherapy for residual microscopic disease, suggests that in a selected group of patients benefit is possible. The purpose of this study was to report our experience with this combined treatment and to identify the principal prognostic factors. METHODS: The study included 266 patients from 9 institutions operated on between July 1990 and July 2004. The median age was 55 years. RESULTS: The mortality rate was 7.8% and the morbidity rate 37.5%. The overall median survival was 13.7 months. Positive independent prognostic factors by multivariate analysis were gender, perioperative intraperitoneal chemotherapy and treatment by the second-look procedure. CONCLUSIONS: The therapeutic approach combining cytoreductive surgery with perioperative intraperitoneal chemotherapy achieved long-term survival in a selected group of patients with an acceptable morbidity and mortality.

Peritoneal carcinomatosis of colorectal origin. Current treatment. Review and update.

Colorectal cancer is the most frequent tumor of the digestive tract. The high incidence of abdominal dissemination; the poor prognosis of these patients, with median survival consistently ranging from 5 to 9 months in all studies of peritoneal carcinomatosis from colorectal cancer; the failure of adjuvant systemic chemotherapy treatment with a maximal survival of 18 months despite the development of new cytostatic drugs, and new combinations of use, make it crucial to search for and develop new treatment strategies. We review the principles of Sugarbaker s treatment protocol, which involves the combination of maximum cytoreductive radical oncological surgery for the treatment of all macroscopically disseminated disease with maximum perioperative intraperitoneal intensification chemotherapy to treat residual microscopic disease. We present the results of several scientific papers, all of them phase II studies with more than 10 patients treated, published in the medical literature by the main groups working in this line of treatment, together with the only phase III study reported and published so far, and finally the results of a recently reported retrospective international multicenter study. With this new alternative therapeutic approach, overall mean survival is 40% at 36 months, and 20% at 5 years. Based on these results, this new therapeutic approach is proposed as the treatment of choice for these unfortunate patients.