Updated Review and Clinical Recommendations for the Diagnosis and Treatment of Patients with Retroperitoneal Sarcoma by the Spanish Sarcoma Research Group (GEIS).

Soft tissue sarcomas (STS) are an uncommon and biologically heterogeneous group of tumors arising from mesenchymal cells. The incidence is estimated at five cases per 100,000 people per year. Retroperitoneal sarcomas (RPS) account for 10-15% of all STS, and their management depends on their anatomical characteristics and histotype. Due to their very low incidence, it is recommended that RPS be treated in reference centers and evaluated by an experienced multidisciplinary team (MDT). In Spain, the Spanish Group for Research in Sarcomas (GEIS) brings together experts from various specialties to promote research on sarcomas and improve treatment results. This paper summarizes the GEIS recommendations for the diagnosis, treatment, and follow-up of patients with RPS.

Avances en tumores del estroma gastrointestinal: ¿hacia dónde vamos?

Gastrointestinal Stromal Sarcomas (GIST) are mesenchymal neoplasms whose incidence accounts for 1-2% of digestive tumors, being located in the stomach (55-60%) and small intestine (30%). The advances in its knowledge and management succeeded in the last years have being spectacular. This review aims to summarize the most important of them for surgeons. We identified four areas of interest: molecular oncology, laparoscopic approach, management of GIST located at unusual locations, and management of advanced GIST. Advances in the field of molecular oncology lead to the discovery of new oncogenic mutations making the term Wil Type GIST obsolete. Moreover, these advances allow for the development of 2 new drugs: Avapritinib and Ripretinib, that added to the previous 3 commercially available drugs (imatinib, sunitinib and regorafenib) make possible the management of GIST with resistant mutations. The principles of the surgical management of primary GIST are well stablished which laparoscopic approach must accomplish. This approach is limited by 2 main factors: location and size. The diagnosis of GIST in unusual locations as esophagus, duodenum, rectum of out of the gastrointestinal tract (EGIST), implies an extraordinary therapeutic challenge, being imperative to manage them by surgeons and oncologist among others in the setting of a multidisciplinary team. The management of advanced/metastatic GIST has changed in a revolutionary fashion because surgery is now part of its treatment as adjuvant to tyrosine kinase inhibitors.

Los tumores del estroma gastrointestinal (GIST) suponen el 1-2% de los tumores digestivos, siendo su localización más frecuente el estómago (55-60%) y el intestino delgado (30%). Los avances más importantes sucedidos en los últimos años se centran en cuatro áreas: biología molecular, abordaje quirúrgico laparoscópico, manejo técnico del GIST en localizaciones inusuales y tratamiento e integración de la cirugía en el manejo del GIST avanzado. Los avances en el conocimiento de la biología molecular del GIST han dado lugar a la progresiva identificación de nueva mutaciones oncogénicas que hacen del concepto wild type obsoleto. Estos avances han permitido el desarrollo de dos nuevos fármacos, avapritinib y ripretinib, lo que permite el tratamiento de pacientes con mutaciones resistentes a las tres líneas terapéuticas clásicas. El tratamiento quirúrgico del GIST se rige por unos principios técnicos bien establecidos que el abordaje laparoscópico debe cumplir, abordaje que queda limitado por dos factores clave: localización y tamaño. El GIST de localización infrecuente (esófago, duodeno o recto, o extradigestivo) supone un reto terapéutico. Estos pacientes deben ser manejados en un contexto multidisciplinario. La cirugía queda integrada en el manejo del GIST avanzado, considerándose como adyuvante a los inhibidores de la tirosina cinasa.

Actualización en el manejo de sarcomas retroperitoneales y pélvicos; el papel de la cirugía compartimental / Update in pelvic and retroperitoneal sarcoma management: The role of compartment surgery

La cirugía de los sarcomas retroperitoneales debe ser compartimental «en bloque», lo que implica la resección de órganos adyacentes al tumor. Su empleo «de entrada» permite un elevado porcentaje de resecciones con márgenes negativos, lo que supone un mejor control local y mayor supervivencia en muchos pacientes. La preservación de órganos debe hacerse de forma personalizada, especialmente en la pelvis, y adaptarla a la agresividad histológica del tumor. La biopsia preoperatoria permite establecer el subtipo de sarcoma y una adecuada estrategia perioperatoria. Estos pacientes deben ser manejados por cirujanos expertos en centros de referencia, con unidades multidisciplinarias y comités oncológicos. El uso de quimioterapia y radioterapia aún no está bien definido, por lo que solo se recomienda en centros de referencia con ensayos clínicos. En la actualidad esta es la única opción para ofrecer las mejores tasas de morbimortalidad, y las posibles mejoras en la supervivencia de estos pacientes

Surgery for retroperitoneal sarcomas should be "en bloc" compartmental, which involves resection of unaffected organs. Its upfront use is key, providing a high percentage of resections with negative margins, resulting in a better local control and increased survival in many patients. Preservation of organs should be done in an individualized manner, especially in the pelvic location, and adapted to the histological aggressiveness of the tumor. Preoperative biopsy is able to establish the diagnosis of sarcoma subtype and consequently an adequate perioperative strategy. These patients should be managed by expert surgeons at referral centers with multidisciplinary units and oncology committees. The use of chemotherapy and radiotherapy is not yet well defined, so it is only recommended at referral centers with clinical trials. Currently, this is the only option to offer the best morbidity and mortality rates, as well as possible improvements in the survival of these patients

Update in pelvic and retroperitoneal sarcoma management: The role of compartment surgery. / Actualización en el manejo de sarcomas retroperitoneales y pélvicos; el papel de la cirugía compartimental.

Surgery for retroperitoneal sarcomas should be "en bloc" compartmental, which involves resection of unaffected organs. Its upfront use is key, providing a high percentage of resections with negative margins, resulting in a better local control and increased survival in many patients. Preservation of organs should be done in an individualized manner, especially in the pelvic location, and adapted to the histological aggressiveness of the tumor. Preoperative biopsy is able to establish the diagnosis of sarcoma subtype and consequently an adequate perioperative strategy. These patients should be managed by expert surgeons at referral centers with multidisciplinary units and oncology committees. The use of chemotherapy and radiotherapy is not yet well defined, so it is only recommended at referral centers with clinical trials. Currently, this is the only option to offer the best morbidity and mortality rates, as well as possible improvements in the survival of these patients.

Tumores mesenquimales-sarcomas: un nuevo grupo de trabajo en la Asociación Española de Cirujanos / Mesenchymal tumors-sarcoma: A new AEC work group

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Angiomixoma agresivo. Un raro tumor mesenquimal pélvico / Aggressive angiomyxoma. A rare mesenchymal pelvic tumor

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PEComa, un raro tumor de células epiteloides / PEComa, a rare epithelioid cell tumor

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Diferencias entre cirugía en bloque y enucleación en el tratamiento del sarcoma retroperitoneal / Differences between en bloc resection and enucleation of retroperitoneal sarcomas

OBJETIVO: La cirugía R0 con márgenes libres es el «gold-standard» en el tratamiento de los sarcomas de partes blandas, así como el mejor predictor de su recurrencia y de la supervivencia de estos pacientes. El objetivo del presente trabajo fue evaluar la evolución de los pacientes afectos de un SRP tratados mediante cirugía de resección «en bloque» frente a los tratados con enucleación en la primera cirugía. MÉTODOS: Una serie de 56 pacientes adultos fueron divididos en 2 grupos según la resección quirúrgica. El grupo A mediante una cirugía de enucleación y el grupo B mediante una cirugía «en bloque». Se valoran la supervivencia global (SG) y el tiempo a la recurrencia (SLE), de acuerdo al tipo histológico y a la estrategia quirúrgica inicial. RESULTADOS: El tiempo libre de enfermedad fue más prolongado tras una cirugía en bloque (p < 0,05); no hubo diferencias con relación a la supervivencia global. Cuando comparamos el tipo histológico con una cirugía de enucleación versus una cirugía en bloque, la SLE y la SG fueron superiores en el liposarcoma. En el análisis multivariado, solo la presencia de márgenes libres y la histología liposarcoma se asociaron significativamente con una mejor supervivencia. CONCLUSIÓN: El tratamiento de los pacientes con un sarcoma retroperitoneal debe ser claramente agresivo, requiriendo exéresis múltiviscerales. A partir de los resultados de SG y SLE, es importante que el tratamiento de estos pacientes se base en una primera cirugía lo más radical posible

AIM: Today, free margin surgery is the gold-standard management for soft-tissue sarcoma patients and one of the most important predictors of recurrence and survival. To obtain optimal results, a multidisciplinary approach is necessary. The aim of this study was to evaluate the evolution of patients with RPS treated by «en bloc«surgical resection versus those treated with enucleation in the first surgery. METHODS: Fifty-six adult patients were divided into 2 groups. Patients in Group A underwent enucleation surgery, and patients in Group B underwent en bloc surgery. The endpoints of the study were survival time and time to recurrence, according to histological type and first surgical strategy. RESULTS: Disease-free survival was longer for en bloc surgery (P < 0,05), but there was no difference in overall survival. When comparing the histology of patients who underwent enucleation surgery and en bloc resection surgery, the disease-free survival and overall survival rates were longer for liposarcoma. In the multivariate analysis, only free margins and histology of liposarcoma were significantly associated with a better survival. CONCLUSIONS: The surgical management of patients with retroperitoneal sarcoma must be very aggressive, often requiring multivisceral resection. Considering the disease-free survival and overall survival rates obtained, it is clear that it is critical to manage patients as early as possible by a radical en bloc surgery

Differences between en bloc resection and enucleation of retroperitoneal sarcomas.

AIM: Today, free margin surgery is the gold-standard management for soft-tissue sarcoma patients and one of the most important predictors of recurrence and survival. To obtain optimal results, a multidisciplinary approach is necessary. The aim of this study was to evaluate the evolution of patients with RPS treated by «en bloc«surgical resection versus those treated with enucleation in the first surgery. METHODS: Fifty-six adult patients were divided into 2 groups. Patients in Group A underwent enucleation surgery, and patients in Group B underwent en bloc surgery. The endpoints of the study were survival time and time to recurrence, according to histological type and first surgical strategy. RESULTS: Disease-free survival was longer for en bloc surgery (P<0,05), but there was no difference in overall survival. When comparing the histology of patients who underwent enucleation surgery and en bloc resection surgery, the disease-free survival and overall survival rates were longer for liposarcoma. In the multivariate analysis, only free margins and histology of liposarcoma were significantly associated with a better survival. CONCLUSIONS: The surgical management of patients with retroperitoneal sarcoma must be very aggressive, often requiring multivisceral resection. Considering the disease-free survival and overall survival rates obtained, it is clear that it is critical to manage patients as early as possible by a radical en bloc surgery.

Tratamiento percutáneo de una obstrucción tumoral de asa aferente de hepaticoyeyunostomía / Percutaneous treatment of a tumour obstruction of the afferent loop of a hepaticojejunostomy

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Cirugía de los tumores del estroma gastrointestinal primarios no metastásicos. Resultados del estudio GRISK / Primary, non-metastatic gastrointestinal stromal tumour surgery: Results of the GRISK study

La cirugía radical es el tratamiento estándar en el GIST primario localizado. Se valora una serie de GIST primarios, su tratamiento y el riesgo preestablecido de recaída tras seguimiento de los mismos. Material y métodos Estudio retrospectivo multicéntrico y descriptivo de GIST primarios no metastásicos intervenidos entre junio de 2007 y diciembre de 2008. Se analizan las variables de mayor relevancia: localización, tamaño, índice mitótico y criterios pronóstico de recidiva NHI y AFIP y su correlación con la supervivencia libre de enfermedad (SLE) de los pacientes. Resultados Serie de 141 pacientes, edad media 65 años, con GIST de localización más frecuente en estómago (70,8%) e intestino delgado (22,9%) y con tamaño medio tumoral de 6,7cm (0,5-35). La cirugía fue R0 en el 97,2% de los casos (abordaje laparoscópico 21,5%). Distribución según criterios de NHI/Flescher: alto (31,95) e intermedio (26,4%), y según criterios AFIP/Miettinen: alto (22,9%) e intermedio (12,5%). Tras un seguimiento medio de 20,3 meses se detectó recaída en 7,1% (10 casos) perteneciendo tan solo 2 casos al grupo de «bajo riesgo» por criterios pronóstico NHI y AFIP. La SLE a un año fue del 95,5% y a los 2 años del 91,5%.ConclusionesLa serie mostró una alta SLE y una buena correlación con los criterios pronóstico tanto de Flescher como de Mietinen. No obstante, el riesgo de recaída varió siguiendo los criterios de NIH (intermedio/alto 58,3%) o los criterios de AFIP (intermedio/alto 35,4%) que incluyen la localización del tumor. Por ello consideramos estos últimos criterios como los más adecuados para la valoración prónostica de riesgo de recidiva del GIST (AU)

Introduction and objectives: Radical surgery is the standard treatment for localised gastrointestinal stromal tumours (GIST). A series of primary GIST, their treatment and pre-established risk of recurrence after their follow-up is evaluated. Material and methods: A retrospective, descriptive and multicentre study was conducted on primary, non-metastatic GIST operated on between June 2007 and December 2008. The variables of greater relevance were analysed, including, location, size, mitotic index, and NHI and AFIP recurrence prognostic criteria, and their correlation with the disease-free survival (DFS) of the patients. Results: The series included 141 patients with a mean age of 65 years. The most frequent GIST location was in the stomach (70.8%) and small intestine (22.9%), and with a mean tumour size of 6.7 cm (0.5-35 cm). The surgery was R0 in 97.2% of cases ( laparoscopic approach, 21.5%). The distribution according to NHI/Flescher criteria was, high (31.95%),and intermediate (26.4%), and according to AFIP/Miettinen criteria it was, high (22.9%) and intermediate (12.5%). After a mean follow-up of 20.3 months, there was a 7.1% (10 cases)recurrence, with only 2 cases belonging to the group with a «low risk» using the NHI and AFIP prognostic criteria. The DFS at one year was 95.5% and 91.5% at 2 years. Conclusions: The series showed a high DFS and a good correlation with both the Flescher and the Miettinen criteria. However, the risk of recurrence varied according to the AFIP criteria(intermediate/high, 58.3%), or the AFIP criteria (intermediate/high, 35.4%) which included the tumour location. For this reason, we consider these latter criteria as the most adequate for assessing the prognostic risk of GIST recurrence (AU)