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Primary Renal Synovial Sarcoma and Clinical and Pathological Findings: a Systematic Review

  • Kidney Diseases (G Ciancio, Section Editor)
  • Published:
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Abstract

Purpose of Review

To update epidemiological, diagnostic, and therapeutic information on primary synovial sarcoma of the kidney.

Recent Findings

A total of 96 studies were analyzed; age at presentation was 38.6±14.2 years, predominant location of tumor was right kidney; frequent reported symptoms at diagnosis were hematuria and pain. For definitive diagnosis, cytogenetic technique was used. Detected oncogene was available in 37.8% cases with fusion of SS18-SSX in most patients. Surgery is treatment of choice, with adjuvant chemotherapy; most frequently ifosfamide-based associated with doxorubicin or epirubicin. Overall median survival was 34 months. Mortality was 29% of the cases which reported death and the recurrence rate was 39.8%. Risk of death was increased in patients with metastases at diagnosis

Summary

Primary RSS occurs more often in young men. RSS often presents with symptoms and in an advanced stage. Surgical treatment is the most commonly used and chemotherapy for advanced or recurrent treatment.

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Abbreviations

AWD:

Alive with disease

ANED:

Alive with no evidence of disease

BCL2:

B-cell leukemia/lymphoma protein 2

RCC:

Renal cell carcinoma

CD34:

Differentiation cluster 34

CD99:

Differentiation cluster 99

CI:

Confidence interval

CT:

Computed axial tomography

DOD:

Died of disease

EMA:

Membrane epithelial antigen

F:

Female

FISH:

Fluorescence in situ hybridization, fluorescent hybridization in situ

IHQ:

Immunohistochemistry

IQR:

Middle and interquartile ranges

IVC:

Inferior vena cava

mo(s) :

Month(s)

M:

Male

MTS:

Metastasis

NMR:

Nuclear magnetic resonance

N:

No

NA:

Not available

PCR:

Polymerase chain reaction

PICO:

Population, intervention, comparator, outcome

QM:

Chemotherapy

RSS:

Renal synovial sarcoma

RT:

Radiotherapy

SD:

Standard deviation

SMA:

Smooth muscle actin

SS:

Synovial sarcoma

Tmt:

Treatment

w:

Weeks

Y:

Yes

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Authors and Affiliations

  1. Hospital Aleman de Buenos Aires, Buenos Aires, Argentina

    Leandro Blas

  2. National Scientific and Technical Research Council - CONICET, Buenos Aires, Argentina

    Javier Roberti

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  1. Leandro Blas
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Blas, L., Roberti, J. Primary Renal Synovial Sarcoma and Clinical and Pathological Findings: a Systematic Review. Curr Urol Rep 22, 25 (2021). https://doi.org/10.1007/s11934-021-01038-w

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