Oncology outcomes in Retroperitoneal sarcomas: Prognostic factors in a Retrospective Cohort study.

INTRODUCTION: Retroperitoneal sarcomas are a rare group of malignant soft tissue tumors with a generally poor prognosis. The aim of the study was to define the demographic characteristics and prognostic factors for patients with retroperitoneal sarcomas (RPS) in a Tertiary Referral Center at Mexico. METHODS: A retrospective study of patients with RPS treated from January 2005 to December 2012 at the National Cancer Institute at Mexico. Patient, tumor and treatment variables were analyzed including use of adjuvant therapy and survival status. Survival and local recurrence curves were estimated using the Kaplan-Meier method. RESULTS: Ninety-five patients with a mean age of 47 years with retroperitoneal sarcoma were included. Median follow-up was 25 months (range 1-108 months). The average tumor size was 23.7 cm. Histology, 58 (61.1%) were liposarcoma, 14 (14.7%), leiomyosarcomas and 23 (24.2%) were from other histologies. In 64 (67.4%) patients were high-grade malignancies. The median survival was 51 months for patients with complete resection, 25.1 months for those with incomplete resection, and 4.4 months for those with unresectable tumors. Complete resection (p = 0.0001), and liposarcoma (p = 0.03) were prognostic factors for overall survival. CONCLUSION: In this study of patients with retroperitoneal, complete resection and liposarcoma histology are prognostic factors related to the disease-free and overall survival. Patients approached with curative intent should undergo aggressive attempts at complete surgical resection.

Survival of high-risk pediatric neuroblastoma patients in a developing country.

Little information is available about survival of high-risk pediatric neuroblastoma patients in developing countries. We aimed to assess survival among high-risk pediatric neuroblastoma patients in La Plata, Argentina. Individuals eligible for our cohort were aged <20 yr when diagnosed with high-risk neuroblastoma and received cancer-directed therapy including stem cell transplantation at Hospital de Niños Sor Maria Ludovica between February 1999 and February 2015. We estimated overall survival probabilities using an extended Kaplan-Meier approach. Our study population comprised 39 high-risk neuroblastoma patients, of whom 39% were aged >4 yr at diagnosis, 54% were male, and 62% had adrenal neuroblastoma. We observed 18 deaths, and the median survival time of our study population was 1.7 yr. The five-yr overall survival probability was 24% (95% CL: 10%, 41%). In contrast, five-yr survival of high-risk neuroblastoma patients ranges between 23% and 76% in developed countries. Survival among high-risk neuroblastoma patients is generally poor regardless of geographic location, but our results illustrate dramatically worse survival for patients in a developing country. We speculate that the observed survival differences could be attenuated or eliminated with improvements in treatment and supportive care, but addressing these issues will require creative solutions because of resource limitations.

Compartment surgery in treatment strategies for retroperitoneal sarcomas: a single-center experience.

BACKGROUND: Retroperitoneal sarcomas (RPS) are rare tumors and radical surgery is still the only curative treatment. We aim to estimate postoperative morbidity and mortality and identify significant prognostic factors for survival of patients with RPS and then evaluate the effect of en bloc resection on survival. METHODS: This was a retrospective follow-up study of 91 patients with RPS who underwent surgery at the Section of Abdominopelvic Surgery of the National Cancer Institute-Brazil (INCA) between June 1992 and January 2008. Overall and 2-, 5-, and 10-year disease-free survival rates were calculated and univariate and Cox multivariate analyses were performed. RESULTS: The most common complaints were abdominal pain and mass. Resection was possible in 83.5% and curative resection in 55.3%. Six patients died within the postoperative period (mortality = 6.6%) and 28 had complications (30.8%). Leiomyosarcomas and liposarcomas predominated, as well as G3. The median tumor diameter was 20.5 cm. There were 124 organs resected in association, with only 42 proven invaded. The 5-year overall survival and disease-free survival rates were 32.0 and 36.8%, respectively. Cell differentiation, curative or palliative resection, blood transfusion, and re-resection were significant variables. Compartment surgery had no impact on survival, but it increased the duration of surgery, the need for blood transfusion, and overall morbidity. CONCLUSIONS: This study suggests that early diagnosis and curative resection of retroperitoneal sarcomas can improve long-term survival. Adjacent organs with evidence of direct invasion must be removed en bloc; others should be spared.

Retroperitoneal soft tissue sarcomas: experience at a single institution in Mexico.

BACKGROUND: Retroperitoneal soft tissue sarcomas (RSTS) are a rare and uncommon entity with a poor 5-year overall survival (OS) of approximately 50%, even though they rarely metastasize. The aim of the present study was to analyze the survival of patients with RSTS treated at our institution. PATIENTS AND METHODS: Patients with RSTS treated between 1990 and 2008 were included. Variables analyzed were age, sex, histological type, TNM stage, tumor size, grade of differentiation, and treatment (surgery, chemotherapy only, radiotherapy only, adjuvant radiotherapy, and best supportive care). R0, R1, and R2 resection were analyzed. Overall survival, recurrence-free survival (RFS), cancer-specific survival (CSS), and survival comparison by stage, grade, and type of resection were analyzed. RESULTS: Data for 46 patients were analyzed, 20 men and 26 women. Median age was 51.1 years. Surgery with a curative intent was performed in 32 patients, and R0 resection was feasible in 19 (59.4%). The 5- and 10-year OS rates were 47 and 30%, respectively, for the entire group. The median CSS for resected patients was 102 months; the 5-year OS for stages I, III, and IV was 83, 37, and 0%, respectively; the 5-year OS for low histological grade disease and high histological grade disease was 82 and 35%, respectively; and for R0, R1, and R2 resection, the 5-year OS was 81, 56, and 14%, respectively. Median RFS was 79 months. CONCLUSIONS: Incomplete surgical resection, unresectable disease, high histological grade, and advanced TNM stage are associated with a poor survival in patients with RSTS. Complete resection is still the treatment of choice.

Masas retroperitoneales / Retroperitoneal Masses

Antecedentes: la cirugía de lesiones ubicadas en el retroperitoneo, han demostrado similitudes tanto en las vías de acceso y la antomía, como en algunas dificultades técnicas y complicaciones quirúrgicas. Objetivos: analizar todos los casos consecutivos operados de masas ubicadas en retroperitoneo, incluso cuando muchas de ellas no respondían al término tumor retroperitoneal. Lugar de aplicación: hospital público y práctica privada. Diseño: estudio primariamente retrospectivo y luego prospectivo. Material y métodos: se analizan 41 pacientes en los que se resecaron 49 masas retroperitoneales. Resultados: se han agrupado como lesiones hereditarias, infecciosas, traumáticas, litiásicas (urinarias) y neoplásicas primarias y secundarias, recidivadas o metastásicas. En todos se describen las dificultades técnicas, especialmente con los grandes vasos, con 7 despegamientos dificultosos, dos lesiones suturadas de aorta y 3 de cava; el colon izquierdo con 5 resecciones segmentarias y los riñones, que siendo la causa primaria de intervención en 19 casos, debieron ser removidos en otros 5. Conclusiones: las masas retroperitoneales se abordan habitualmente según técnicas quirúrgicas conocidas, encontrándose elementos anatómicos que le confieren cierta dificultad; esto puede incrementarse en lesiones invasivas, pero son comunes a todas en mayor o menor medida. (AU)

Resección de tumores retroperitoneales / Retroperitoneal tumors resection

Antecedentes: Los Tumores Retroperitoneales (TRP), constituyen entidades de escasa observación, frecuentemente malignas y, generalmente, de diagnóstico tardío. Su exéresis completa obliga, asiduamente, a resecar órganos vecinos. Objetivo: Analizar las resecciones de TRP realizadas personalmente durante 5 años (1992 a 1996). Lugar de Aplicación: Hospital Angel C. Padilla y práctica privada. Diseño: Estudio retrospectivo. Material y Métodos: se analizan 9 resecciones de TRP en ocho pacientes, cuatro de ellos mujeres. Tres de los diagnósticos fueron intraoperatorios, en tumores benignos; los restantes, clínico, ecográfico y tomográfico. Resultados: No hubo mortalidad peri-operatoria. Las resecciones de lesiones benignas fueron completas. 5 de 8 pacientes presentaban Sarcomas; 4 requirieron resección de órganos vecinos, incluyendo colon izquierdo, bazo y riñón derecho. 3 de 5 Sarcomas fueron Liposarcomas. Ninguno fue considerado irresecable. Conclusiones: Se han resecado ocho TRP primitivos y uno recidivado. Hallazgo intraoperatorio: dos casos. Los restantes se diagnosticaron con Ecografía y TAC. Los Sarcomas representaron 5 de 8 pacientes; de ellos, 3 fueron Liposarcomas. Los dos restantes fueron Condrosarcoma y Fibrohistiocitoma maligno. Un Liposarcoma fue reoperado antes de los 8 meses por recidiva; pero globalmente haan mostrado mejor respuesta al tratamiento. Los dos fallecidos por sus tumores fueron portadores de las otras formas histológicas. La resección de vísceras vecinas fue necesaria en 4 de 5 Sarcomas, no siempre relacionadas con el volumen. Se resecaron colon izquierdo, bazo y riñón derecho. (AU)