Retroperitoneal Tumors in Adults

Abstract

Retroperitoneal tumors are a heterogeneous group of rare tumors arising in the retroperitoneum, but not from the retroperitoneal organs. Most retroperitoneal tumors are malignant and derive from soft tissue, sarcomas being the most common. Primary extragonadal germ cell tumors and primary retroperitoneal lymphomas are rare differential diagnoses. A hallmark of malignant retroperitoneal soft tissue tumors is their large size upon diagnosis and their poor prognosis. CT scans and core needle biopsies are the mainstay of diagnosis. Extended radical and complete en bloc surgery is the only potentially curative treatment. Surgery should always be imageguided and never be exploratory. Despite surgery, local recurrence is common and accounts for most of the disease’s morbidity. Even though the scientific evidence is weak, neoadjuvant chemotherapy and/or preoperative radiation might be of some value for selected patients and histological subtypes. Guidelines strongly recommend a multidisciplinary treatment approach at a specialized center and data collection in international registries. Since all these cases are rare, advances can only be made in collaboration.