Resultados obtenidos tras el tratamiento quirúrgico de los pacientes con metástasis hepáticas de sarcomas / Surgical and oncological results after surgical treatment of patients diagnosed with sarcoma liver metastases

Introducción: La presencia de metástasis hepáticas en pacientes con sarcomas se asocia a peor pronóstico, aunque en casos seleccionados la resección de dichas metástasis se ha propuesto para aumentar la supervivencia. El objetivo de este estudio es describir la evolución postoperatoria y los resultados oncológicos tras la resección hepática. Métodos: Se presenta un estudio retrospectivo unicéntrico. Se incluyen pacientes diagnosticados de metástasis hepáticas de sarcoma intervenidos quirúrgicamente entre 2003-2019. Los criterios de inclusión fueron la presencia de enfermedad resecable, la presencia de enfermedad extrahepática controlada no se consideró criterio de irresecabilidad. Resultados: Diecinueve pacientes se sometieron a resección hepática de 7 tipos distintos de estirpes sarcomatosas. La mediana de edad fue de 58 años. Las metástasis se diagnosticaron 25 meses de mediana tras el primario, 6 (32%) presentaron lesiones sincrónicas y 12 (63%) estaban afectos de enfermedad extrahepática. Se realizó hepatectomía mayor en 5 (26%) pacientes; se describieron 8 (42%) complicaciones menores. La mediana de seguimiento fue de 33 meses. El análisis de supervivencia se realizó estratificando en 2 grupos, la supervivencia fue del 100%, 85,7% y del 42,9% al año, a los 3 años y a los 5 años, en los no-GIST, y del 100% y del 40% a los 5 y 10 años en los GIST. Conclusiones: El abordaje quirúrgico de las metástasis hepáticas de sarcoma parece aumentar la supervivencia en pacientes seleccionados, asociando pocas complicaciones. En nuestra serie, la tasa de enfermedad extrahepática es elevada en comparación con series previas, no obstante la supervivencia es equiparable. Dichos resultados apoyan la resección hepática en pacientes con enfermedad extrahepática estable. (AU)

Introduction: The presence of liver metastases in sarcomatous tumors is associated with poor prognosis. However, in selected patients, surgical resection has been suggested as a tool to improve survival rates. The aim of our study is to describe postoperative and oncological outcomes after liver resection. Methods: A retrospective unicentric study was conducted including patients diagnosed with hepatic metastases from soft tissue sarcoma who underwent hepatic resection between 2003 and 2019. The inclusion criteria were the presence of resectable disease, including synchronic and metachronic lesions. The presence of extra-hepatic controlled disease was not considered unresectable. Results: Nineteen patients underwent liver resection for liver metastasis of 7 different sarcomatous subtypes. Median age was 58-yo. Liver metastases were diagnosed a median 25 months after primary tumor diagnosis. Six patients (32%) suffered of synchronic metastases and 12 (63%) were affected of extrahepatic disease. Major hepatectomy was done in 5 (26%) patients, 8 (42%) minor complications were described. Median follow-up was 33 months. Survival analysis was performed independently for, GIST tumors and non-GIST sarcomas. One, three and five-year survival rate was 100%, 85.7% and 42.9% in non-GIST sarcomas, while five and ten-year survival rate was 100% and 40% in GIST, respectively. Conclusions: Surgical approach of liver metastases of sarcomatous tumors seems to be useful in order to improve survival in selected patients, while associated to be of low complications rate. In our cohort, extrahepatic disease rate is high in comparison with series published before, nevertheless survival is comparable. These results support performing surgical resection in selected patients with stable extrahepatic disease. (AU)

Surgical and oncological results after surgical treatment of patients diagnosed with sarcoma liver metastases.

INTRODUCTION: The presence of liver metastases in sarcomatous tumors is associated with poor prognosis. However, in selected patients, surgical resection has been suggested as a tool to improve survival rates. The aim of our study is to describe postoperative and oncological outcomes after liver resection. METHODS: A retrospective unicentric study was conducted including patients diagnosed with hepatic metastases from soft tissue sarcoma who underwent hepatic resection between 2003-2019. The inclusion criteria were the presence of resectable disease, including synchronic and metachronic lesions. The presence of extra-hepatic controlled disease was not considered unresectable. RESULTS: Nineteen patients underwent liver resection for liver metastasis of 7 different sarcomatous subtypes. Median age was 58-years. Liver metastases were diagnosed a median 25 months after primary tumor diagnosis. Six patients (32%) suffered of synchronic metastases and 12 (63%) were affected of extrahepatic disease. Major hepatectomy was done in 5 (26 %) patients, 8 (42%) minor complications were described. Median follow-up was 33 months. Survival analysis was performed independently for, GIST tumors and non-GIST sarcomas. One, three and five-year survival rate was 100%, 85.7% and 42.9% in non-GIST sarcomas, while Five and ten-year survival rate was 100% and 40% in GIST, respectively. CONCLUSION: Surgical approach of liver metastases of sarcomatous tumors seems to be useful in order to improve survival in selected patients, while been associated to low complications rate. In our cohort, extrahepatic disease rate is high in comparison with series published before, nevertheless survival is comparable. These results support performing surgical resection in selected patients with stable extrahepatic disease.

Hernia enlargement and pancreatitis in a patient with short bowel syndrome treated with teduglutide.

INTRODUCTION: Introduction: teduglutide (TED) is indicated for the treatment of patients with short-bowel syndrome (SBS) who are dependent on parenteral support. Case report: we report the case of a 60-year-old woman with SBS treated with TED. She had previously undergone multiple surgical resections due to Crohn's disease. Her remnant bowel included only the duodenum and 50-60 centimeters of jejunum. The patient was dependent on intravenous fluids (2,320 mL/48 h) and had a high stoma output (3,000 mL/day). After four months of TED the jejunostomy output had decreased to 2,200 mL/day with a thicker consistency, and intravenous fluid therapy was reduced to 2,010 mL/48 h. TED was withdrawn due to acute pancreatitis and enlargement of two supraumbilical hernias with high strangulation risk. Discussion: pancreatitis has been reported in clinical studies, and determination of amylase and lipase is recommended in all patients receiving TED. In contrast, there are no recommendations for the surveillance of hernia enlargement in patients on TED therapy, but we suggest the need for surveillance based on this case report.

INTRODUCCIÓN: Introducción: la teduglutida (TED) está indicada para el tratamiento de pacientes con síndrome de intestino corto (SBS) que precisen soporte parenteral. Caso clínico: mujer de 60 años con SBS tratada con TED. Previamente se había sometido a múltiples resecciones quirúrgicas por su enfermedad de Crohn. Su intestino remanente incluía el duodeno y 50-60 centímetros de yeyuno. La paciente era dependiente de líquidos por vía intravenosa (2320 ml/48 h) y tenía una ostomía de alto débito (3000 ml/día). Después de cuatro meses de TED, el débito de la yeyunostomía disminuyó a 2200 ml/día, con una consistencia más espesa, y la fluidoterapia intravenosa se redujo a 2010 ml/48 h. Se retiró la TED por pancreatitis aguda y agrandamiento de dos hernias supraumbilicales con alto riesgo de estrangulamiento. Discusión: se han descrito casos de pancreatitis en estudios previos, por lo que se recomienda la determinación de la amilasa y la lipasa en los pacientes tratados con TED. Sin embargo, no hay recomendaciones específicas sobre la vigilancia del agrandamiento de hernias, pero sugerimos su idoneidad basada en este caso clínico.

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.

Hemorragia digestiva aguda en paciente con neurofibromatosis tipo 1 afecto de múltiples GIST y ganglioneuromatosis intestinal / Acute gastrointestinal bleeding, multiple GIST and intestinal ganglioneuromatosis in a patient with neurofibromatosis

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Leiomyosarcoma of the Right Gonadal Vein: Review of the Approach and Prognostic in a Rare Case.

BACKGROUND: Venous leiomyosarcoma is a mesenchymal tumour that represents 5-7% of soft tissue sarcomas. It originates in the smooth muscle cells of the vessel wall and is frequently located in the inferior vena cava. Primary leiomyosarcomas of the gonadal vein are rare, with only 10 cases reported in the literature. CASE REPORT: We present the case of a 51-year-old woman diagnosed with a right retroperitoneal mass by computed tomography (CT). The differential diagnosis was between a neurogenic tumour and a mesodermic tumour. The tumour was dissected from the vena cava and right ureter by laparoscopy without performing resection en bloc. Histologic examination of the surgical specimen showed a high-grade leiomyosarcoma of the right gonadal vein. The postoperative course was uneventful. Three cycles of chemotherapy with epirubicin-ifosfamide were performed. DISCUSSION AND CONCLUSIONS: Venous leiomyosarcoma is an aggressive tumour, and prognosis is poor due to haematogenous spread. No chemotherapy or radiotherapy has yet proven effective in improving survival, and complete surgical excision is currently considered to offer the best chance of cure. Despite the more conservative treatment approach used in the present case, the patient is alive three years after surgery and has a good quality of life. Although it was not used in this patient, the standard procedure for optimal survival is resection en bloc.

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