Primary synovial sarcoma of the kidney: a case report of complete pathological response at a Lebanese tertiary care center

Abstract

BackgroundPrimary synovial sarcoma of the kidney is a rare type of soft tissue sarcoma. Its presenting features can resemble those of other renal tumors; rendering its early diagnosis, a dilemma. Several cases of renal synovial sarcoma have been reported in the literature with varying treatment options and outcomes. This article describes a rare case of primary renal synovial sarcoma and reviews all cases in the literature.Case presentationA 26-year-old male presented with flank pain and hematuria. Initially diagnosed with Wilm’s tumor, revision of pathology and histology, along with the immunohistochemical profile, confirmed, nevertheless, the diagnosis of primary monophasic synovial sarcoma of the kidney with the SYT-SSX2 fusion transcript. Follow-up, post nephrectomy, revealed recurrence within the lungs and at the surgical bed. Surgical resection followed by adjuvant chemotherapy regimen constituting of Doxorubicin and Ifosfamide, achieved complete pathological response.ConclusionIn this case report, we emphasize the need for accurate diagnosis and prompt treatment. We propose multimodality treatment approach including surgery along with anthracycline-based chemotherapy to induce complete remission.

Doxorubicin and ifosfamide for recurrent renal synovial sarcoma: The first case report in Indonesia

“…Partial response was shown after six courses of treatment. Chediak et al reported a case where the patient had a complete pathological response after being given adjuvant doxorubicin and ifosfamide [12] . Park et al also reported a patient who was given six courses of this chemotherapy regimen with complete remission [13] .…”

A rare case of synovial sarcoma with lung, heart and adrenal gland metastasis: a caution for patients and clinicians

“…Cases of intraprostatic synovial sarcoma have also been reported in the literature. 7 – 10 The overall prognosis of primary metastatic synovial sarcoma remains poor, with a metastases rate of 25%, and a 48% rate of recurrence. 11 , 12 The standard approach to treating synovial sarcoma is wide surgical excision combined with radiotherapy, with or without (neo)adjuvant chemotherapy, as appropriate.…”

Primary renal synovial sarcoma: A case report

“…In addition, TLE1 antibody can also be used as an important tissue factor to distinguish PRSS from other sarcomas. Studies by Mishra et al have shown that TLE1 is more specific and sensitive to synovial sarcomas than any immunohistochemical factor[11,12]. Molecular biology is also providing further confirmation that t(X;18) translocation can lead to syt-ssx fusion, and SYT rearrangement has become the gold standard for PRSS diagnosis[4].…”