• General surgery
• Pathology
• Surgical oncology

• Male
• Humans
• Sarcoma, Ewing/diagnostic imaging
• Sarcoma, Ewing/surgery
• Neuroectodermal Tumors, Primitive, Peripheral
• Duodenum/diagnostic imaging
• Duodenum/surgery
• Biopsy
• Rare Diseases
• Lung Neoplasms

The management of retroperitoneal sarcomas can be challenging due to the variety of their presentation, histopathological types, and behaviours. This literature review provides a comprehensive and practical overview of the management of retroperitoneal sarcomas, focusing on diagnostic challenges, prognostic factors, multidisciplinary aspects of treatment and new research perspectives.

Retroperitoneal sarcomas (RPS) are rare cancers whose management can be challenging due to various presentation patterns, multiple organ involvement, and a high local and distant recurrence rate. Histopathology and prognostic factors analysis are essential to predict the behaviour of the disease and plan the best therapeutic strategy. To date, surgery is still the main therapeutic option that guarantees a chance of cure from the primary disease. While chemotherapy and radiotherapy seem to be good options for controlling metastatic and recurrent irresectable disease, their role in the treatment of primary RPS remains unclear. This literature review aims to provide a comprehensive overview of the multidisciplinary aspects of RPS management in high-volume centres, summarising the diagnostic path, the prognostic factors, and the most suitable therapeutic options.

• cancer
• literature review
• management
• multidisciplinary
• retroperitoneal sarcoma

Adrenal primitive neuroectodermal tumor (PNET) is an extremely rare malignant tumor with poor prognosis and of neural crest origin. Herein, we report a case of adrenal PNET and summarized its clinical and pathological characteristics on the basis of 16 patients reported recently.

A female patient aged 25 years presented with right lumbago for 12 mo, and pre-operative computed tomography showed a huge right adrenal mass. She received tumorectomy, and post-operative pathological examination showed adrenal PNET. After surgery, she underwent adjuvant chemotherapy and was followed up 31 mo after surgery. She received brachytherapy for right paracolic and hepatic metastases. She was alive and followed up for 60 mo. In available studies, only 57.14% (4/7) and 44.44% (4/9) were positive for the expression of neuron-specific enolase and synaptophysin, respectively, although CD99 expression was found in all the patients (100%; 10/10).

It is concluded that adrenal PNET is very rare and highly malignant, and histology is a golden standard in its diagnosis. Surgery and adjuvant therapy is the main treatment.

• Primitive neuroectodermal tumor
• Adrenal gland
• Case report

Patients with retroperitoneal sarcoma can present to a variety of clinicians with non‐specific symptoms and retroperitoneal sarcomas can be incidental findings. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in non‐specialist centers. Therefore it is critical that the possibility of retroperitoneal sarcoma should be considered with prompt referral to a soft tissue sarcoma unit. This review guides clinicians through a diagnostic pathway, introduces concepts in response assessment and new imaging developments.

• CT
• MRI
• diagnosis
• retroperitoneum
• soft tissue sarcoma

• CD99
• Ewings sarcoma
• Retroperitoneum

• Chemotherapy
• Extraosseous Ewing's sarcoma
• Multimodality care
• Surgery