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Review

Primary Synovial Sarcoma of the Kidney: Diagnostic Approach and Therapeutic Modalities for a Rare Nosological Entity

by
Aikaterini Mastoraki
1,
Dimitrios Schizas
1,
Despoina Maria Karavolia
1,
Antonios Smailis
1,
Nikolaos Machairas
2,*,
Michail Vailas
1,
Adamantios Michalinos
3,
Dimitrios Tsapralis
4,
Ioannis Anastasiou
5 and
George Agrogiannis
6
1
First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, 11527 Athens, Greece
2
Second Department of Propaedeutic Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, 11527 Athens, Greece
3
Department of Anatomy, European University of Cyprus, 1516 Nicosia, Cyprus
4
Department of Surgery, General Hospital of Ierapetra, 72200 Ierapetra, Greece
5
First University Urology Clinic, National and Kapodistrian University of Athens, Laikon General Hospital, 11527 Athens, Greece
6
First Department of Pathology, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece
*
Author to whom correspondence should be addressed.
J. Pers. Med. 2022, 12(9), 1450; https://doi.org/10.3390/jpm12091450
Submission received: 10 August 2022 / Revised: 27 August 2022 / Accepted: 31 August 2022 / Published: 2 September 2022
(This article belongs to the Section Clinical Medicine, Cell, and Organism Physiology)

Abstract

Synovial sarcoma (SS) is a rare mesenchymal entity that represents 5–10% among soft tissue sarcomas (STS). Primary renal synovial sarcoma (PRSS) is an uncommon, rapidly growing tumor, with potential metastatic dissemination. The main prognostic factors of PRSS include tumor size and histologic grade, while translocation t (X; 18) (p11.2; q11.2) (fusion of SYT gene -chromosome 18- with SSX genes (1, 2 or 4)-chromosome X) is the most common pathognomonic sign. Aggressive surgical resection of the tumor along with concomitant regional lymphadenectomy is the treatment of choice for PRSS, while additional en bloc resection of the adjacent affected organs is often performed. To date, the role of preoperative or postoperative chemotherapy remains equivocal. The prognosis of patients with PRSS is poor, as the 5-year survival rate is only 20–30% and further deteriorates when a high mitotic activity is detected. Local recurrence even after complete R0 surgical excision remains the most frequent cause of death. The aim of this review was to meticulously discuss clinical features, histogenesis, and morphological and immunochemical findings of PRSS, while the role of current diagnostic and therapeutic management of this aggressive neoplasm was emphasized.
Keywords: retroperitoneal; synovial; sarcoma; therapeutic management; prognostic parameters retroperitoneal; synovial; sarcoma; therapeutic management; prognostic parameters
Graphical Abstract

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MDPI and ACS Style

Mastoraki, A.; Schizas, D.; Karavolia, D.M.; Smailis, A.; Machairas, N.; Vailas, M.; Michalinos, A.; Tsapralis, D.; Anastasiou, I.; Agrogiannis, G. Primary Synovial Sarcoma of the Kidney: Diagnostic Approach and Therapeutic Modalities for a Rare Nosological Entity. J. Pers. Med. 2022, 12, 1450. https://doi.org/10.3390/jpm12091450

AMA Style

Mastoraki A, Schizas D, Karavolia DM, Smailis A, Machairas N, Vailas M, Michalinos A, Tsapralis D, Anastasiou I, Agrogiannis G. Primary Synovial Sarcoma of the Kidney: Diagnostic Approach and Therapeutic Modalities for a Rare Nosological Entity. Journal of Personalized Medicine. 2022; 12(9):1450. https://doi.org/10.3390/jpm12091450

Chicago/Turabian Style

Mastoraki, Aikaterini, Dimitrios Schizas, Despoina Maria Karavolia, Antonios Smailis, Nikolaos Machairas, Michail Vailas, Adamantios Michalinos, Dimitrios Tsapralis, Ioannis Anastasiou, and George Agrogiannis. 2022. "Primary Synovial Sarcoma of the Kidney: Diagnostic Approach and Therapeutic Modalities for a Rare Nosological Entity" Journal of Personalized Medicine 12, no. 9: 1450. https://doi.org/10.3390/jpm12091450

APA Style

Mastoraki, A., Schizas, D., Karavolia, D. M., Smailis, A., Machairas, N., Vailas, M., Michalinos, A., Tsapralis, D., Anastasiou, I., & Agrogiannis, G. (2022). Primary Synovial Sarcoma of the Kidney: Diagnostic Approach and Therapeutic Modalities for a Rare Nosological Entity. Journal of Personalized Medicine, 12(9), 1450. https://doi.org/10.3390/jpm12091450

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