Abstract
Background
Primary renal angiosarcoma (PRA) is an extremely rare and aggressive neoplasm. Indeed,
due to its rarity, established clinical guidelines for PRA have not yet been established.
In this study, we attempted to investigate its prognostic factors and treatment options.
Methods
We systematically searched for articles describing PRA that had been published up
until December 2021. The final cohort included 113 patients in 103 articles. The starting
point of this study was the time of diagnosis and the end point was the time of recurrence
and disease-specific mortality.
Results
Metastasis at diagnosis was associated with poorer disease-specific survival (DSS)
(p = 0.001). Tumors of more than 5 cm had poorer DSS than tumors of 5 cm or less (p < 0.001).
Multivariate analysis demonstrated that primary metastatic status, and tumor size
were independent prognostic factors. In cases of localized PRA, tumor sizes exceeding
5 cm had also prognostic significance for recurrence-free survival (RFS) and DSS.
Surgical margins, postoperative radiation, and postoperative systemic therapy were
not associated with prognoses. However, in a subgroup analysis of tumors exceeding
5 cm, postoperative radiation therapy improved RFS and DSS (p = 0.022 and p = 0.031,
respectively). In cases of metastatic PRA, systemic therapy improved DSS (p < 0.001).
Conclusion
We identified several prognostic factors for PRA. Among them, primary metastatic status
and tumor size exceeding 5 cm were selected as independent prognostic factors. Postoperative
radiation therapy for large, localized PRA and systemic therapy for recurrent and
metastatic PRA might be a treatment option.
Keywords
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Article info
Publication history
Published online: September 07, 2022
Accepted:
September 2,
2022
Received in revised form:
August 29,
2022
Received:
April 20,
2022
Identification
Copyright
© 2022 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.
