Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma

Apte, SS; Mor, E; Mitchell, C; Gyorki, DE

Author(s): Apte, SS; Mor, E; Mitchell, C; Gyorki, DE;
Details: Publication Year 2023-06-21,Volume 30,Issue #7,Page 5953-5972
Journal Title: Current Oncology
Publication Type: Review
Abstract: With the exception of well-differentiated liposarcoma, dedifferentiated liposarcoma, leiomyosarcoma, solitary fibrous tumour, malignant peripheral nerve sheath tumour, and undifferentiated pleomorphic sarcoma, the majority of the approximately 70 histologic subtypes of retroperitoneal sarcoma are defined as 'ultra-rare' sarcomas, with an incidence of </=1-5/1,000,000 persons/year. For most of these ultra-rare RPS subtypes, diagnosis and treatment follows international guidelines for the management of more common RPS histologies, with en bloc surgical resection as the mainstay of curative treatment, and enrolment in clinical trials where possible. Because the treatment of RPS is heavily driven by histology, the surgeon must be familiar with specific issues related to the diagnosis and management of ultra-rare sarcoma subtypes. Expert radiological and surgeon reviews are required to differentiate similarly presenting tumours where surgery can be avoided (e.g., angiomyolipoma), or where upfront systemic therapy is indicated (e.g., extraosseous Ewing's sarcoma). Thus, the management of all retroperitoneal sarcomas should occur at a sarcoma referral centre, with a multidisciplinary team of experts dedicated to the surgical and medical management of these rare tumours. In this focused review, we highlight how diagnosis and management of the ultra-rare primary RPS histologies of malignant perivascular epithelioid cell tumour (PEComa), extraosseous Ewing sarcoma (EES), extraosseous osteosarcoma (EOS), and rhabdomyosarcoma (RMS) critically diverge from the management of more common RPS subtypes.
Publisher: MDPI
Keywords: Adult; Humans; *Sarcoma, Ewing/diagnostic imaging/therapy; *Sarcoma/diagnosis/therapy/pathology; *Retroperitoneal Neoplasms/diagnosis/therapy/pathology; *Soft Tissue Neoplasms; *Kidney Neoplasms; adult rhabdomyosarcoma; extraosseous Ewing sarcoma; extraosseous osteosarcoma; perivascular epithelioid tumour; retroperitoneal sarcoma; ultra-rare sarcoma
Department(s): Surgical Oncology; Pathology
PubMed ID: 37504306
Publisher's Version: https://doi.org/10.3390/curroncol30070445
Open Access at Publisher's Site: https://doi.org/10.3390/curroncol30070445
Terms of Use/Rights Notice: Refer to copyright notice on published article.

Creation Date: 2023-09-12 07:43:54

Last Modified: 2023-09-12 07:44:44