Primary Synovial Sarcoma of Kidney - A Histological Surprise: A Rare Case Report from a Tertiary Care Centre

Case Report

Austin J Clin Case Rep. 2021; 8(10): 1234.

Primary Synovial Sarcoma of Kidney - A Histological Surprise: A Rare Case Report from a Tertiary Care Centre

Jayadeva Reddy S¹, Sourabh Reddy B¹, Pai K² and Zeeshan Hameed BM³*

1Registrar, Department Of Urology, Kasturba Medical College, Manipal, India

2Professor, Department Of Pathology, Kasturba Medical College, Manipal, India

3Professor, Department Of Urology, Father Muller Medical College, Mangalore, India

*Corresponding author: BM Zeeshan Hameed, Associate Professor, Department Of Urology, Father Muller Medical College and Hospital, Mangalore, 575002, India

Received: October 27, 2021; Accepted: November 17, 2021; Published: November 24, 2021

Abstract

Renal Synovial Sarcoma (SS) are rare type of tumors arising from the mesenchymal tissue of the kidney. Its presenting features overlap with other renal tumors there by creating a diagnostic dilemma. Very few cases of primary renal SS are described so far in the literature with no defined treatment protocol described. We hereby describe a case report of this rare tumor in a 12 year old girl who presented to us with hematuria and flank pain. Histopathological analysis and the Immunohistochemistry (IHC) confirmed the diagnosis of primary monophasic synovial sarcoma. Follow-up post nephrectomy at six months showed no evidence of metastasis or recurrence.

Keywords: Synovial sarcoma; SYT-SSX; Renal tumours; Renal spindle cell tumour; Pathological response; Survival

Abbreviations

SS: Synovial Sarcoma; CT: Computed Tomography; PCR: Polymerase Chain Reaction; FISH: Fluroscence In Situ Hybridization

Introduction

Synovial Sarcomas (SS) are a group of tumors of mesenchymal origin affecting predominantly young adults. SS commonly involves the proximal extremities with male predominance. Other sites of involvement are lungs, heart, head, neck and kidneys but these tumors are exceedingly rare [1]. The term “synovial” is a misnomer as morphology of the tumor and Immunohistochemistry (IHC) does not match with that of a normal synovium of joint [2].

SS can be classified into biphasic or monophasic variety histologically. Monophasic SS of kidney is rare and was initially described by Faria et al. [3]. Monophasic SS consists of only of spindle cells without any epithelial component and biphasic SS consists of spindle cells with epithelial cell components.

Primary SS of kidney is very rare with less than 50 cases being reported till date [4]. It accounts for only 5-10% of adult soft tissue sarcomas and even rare in pediatric population. There is no clinical or imaging findings that are characteristic of the tumor which can clinch the diagnosis [5]. Chromosomal rearrangement studies are required at times for confirmation of diagnosis along with a characteristic histology with IHC. IHC positivity for CD99, bcl2 and TLE11 is characteristic of SS and there is SYT-SSX gene fusion rearrangement, t (X; 18) (p11.2; q11.2) [5]. We hereby report a case of primary SS of the kidney, which was a histological surprise, thought to be a Wilms tumor preoperatively.

Case Presentation

A 12-year-old girl presented with complaints of pain in the left flank which was recurrent since 8 months, insidious in onset and colicky in nature, associated with gross hematuria with passage of clots for a period of 4 days before seeking medical care. Ultrasound imaging showed an upper pole mass indicating a further evaluation of the lesion using a Contrast Enhanced CT scan (CECT). CECT showed presence of heterogeneously enhancing lobulated mass which involved the upper pole of the left kidney along with renal pelvis measuring around 4.7 centimeters (Figure 1a). There was no evidence of metastasis to lung or liver. Based on the above findings, provisional diagnosis of Wilm’s tumor was considered and patient underwent left radical nephrectomy (Figure 1b).

Citation: Jayadeva Reddy S, Sourabh Reddy B, Pai K and Zeeshan Hameed BM. Primary Synovial Sarcoma of Kidney - A Histological Surprise: A Rare Case Report from a Tertiary Care Centre. Austin J Clin Case Rep. 2021; 8(10): 1234.