Retroperitoneal Sarcoma Care in 2021

Abstract

Soft-tissue sarcomas are biologically heterogenous tumors arising from connective tissues with over 100 subtypes. Although sarcomas account for <1% of all adult malignancies, retroperitoneal sarcomas are a distinct subgroup accounting for <10% of all sarcomatous tumors. There have been considerable advancements in the understanding and treatment of retroperitoneal sarcoma in the last decade, with standard treatment consisting of upfront primary surgical resection. The evidence surrounding the addition of radiation therapy remains controversial. There remains no standard with regards to systemic therapy, including immunotherapy. Adjunctive therapy remains largely dictated by expert consensus and preferences at individual centers or participation in clinical trials. In this 2021 review, we detail the anatomical boundaries of the retroperitoneum, clinical characteristics, contemporary standard of care and well as recent advancements in retroperitoneal sarcoma care. Ongoing international collaborations are encouraged to advance our understanding of this complex disease.

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

“…Operative procedure aspects are crucial and must be designed based on the patient’s condition, histology, and tumor location in the retroperitoneum. Technically, the surgical approach must be standardized to maximize the chances of obtaining a complete R0 resection with negative microscopic margins [ 19 , 22 , 44 ].…”

“…The retroperitoneum is a complex anatomic space located behind the peritoneal cavity and bordered by great muscles: the diaphragm, the abdominal wall, and paraspinous muscles. For the sarcoma surgeon a profound knowledge of the retroperitoneal anatomy and its relationship with major vessels and organs, the surgical dissection planes of this space, and the continuity with the extra-retroperitoneal spaces is crucial [ 44 ]. When we define RPS, we are including sarcomas located in the retroperitoneal space, but also those located in the pelvis, the psoas muscle, the inguinal canal, and the great vessels and nerves of the retroperitoneum.…”

Hallazgos incidentales en cirugía abdominal: masas y sarcomas retroperitoneales

“…El hallazgo intraoperatorio de una masa retroperitoneal plantea algunas consideraciones para tener en cuenta: las características de la lesión como única o múltiple, quística, solida o mixta, su localización, el componente infiltrativo de órganos adyacentes, las adherencias a estructuras vasculares o nerviosas críticas, el compromiso multicompartimental o unicompartimental y la limitación por planos fasciales 13 . Algunas lesiones específicas, como en el caso de los liposarcomas bien diferenciados, pueden verse muy similares al tejido graso circundante, mientras los leiomiosarcomas pierden el plano de clivaje con músculos como el psoas o cuadrado lumbar, entre otros, y definir un plano de resección o determinar la extensión de la resección puede ser aún más complejo 16 .…”

Case report and literature review: Conversion surgery for initially unresectable huge retroperitoneal liposarcoma after preoperative radiotherapy

“…Unfortunately, the rare nature of RPSs and their heterogeneity make high level evidence difficult to come by (2,5). To date, the roles of neoadjuvant and adjuvant therapies for RPLS have remained controversial (6). In this report, we present a case of huge RPLS, whose greatest diameter reached 44.5cm at presentation.…”