Primary synovial cell sarcoma of the heart: A rare case

Abstract

A synovial sarcoma (also known as malignant synovioma) is a rare form of cancer that usually occurs near the joints of the arm, neck, or leg. It is one of the soft tissue sarcomas. Primary cardiac neoplasms are rare. Most common site for synovial sarcoma is lower limb. Synovial sarcoma of the heart is extremely rare. Occurrence of synovial sarcoma at extra synovial site is very uncommon. It is two times common in right side heart than left side so left side synovial sarcoma is rarest of rare, only very limited cases are reported. Here, we report a case of primary synovial cell sarcoma of the left side of the heart in a 26-year-old female. She presented with dyspnea and weakness only. She was operated for left-sided mass covering mitral valve; further histo-pathology revealed the mass as synovial cell sarcoma. In Indian scenario, this type of rare case needs documentation.

Monophasic Synovial Sarcoma of the Left Ventricle of the Heart: An Extremely Rare Case and Literature Review

“…Surgery has been the primary treatment of the SS cases since wide excision is required for a better outcome, followed by chemotherapy. The prognosis of SS is poor, with the most common causes of death as tumor recurrence and metastasis [8], [11]. Favorable factors for better survival are the young age when the diagnosis was made, the absence of chromosomal abnormalities, and the pericardium origin tumor.…”

Infiltrating Cardiac Synovial Sarcoma Presenting as Acute Cerebrovascular Accident

“…The hallmark for diagnosing synovial sarcoma is the cytogenetic detection via FISH of the translocated chromosome t(X;18)(p11.2;q11.2) which is present on approximately 90% of synovial sarcomas [ 9 – 12 ]. This translocation results in the fusion of the SYT gene on chromosome 18q11 with the SSX1 , SSX2 , or SSX4 gene on chromosome Xp11 [ 10 , 11 , 13 ].…”

Primary cardiac atrial sarcomas. Report of two histologically different cases and review of the literature

“…In case of SS, the differential diagnosis includes mesothelioma, fibrosarcoma, MPNST and myxoid sarcoma/myxoma [50,72]. For biphasic SS it is challenging to differentiate with mesothelioma with histology and immunohistochemistry with the support of molecular testing might be necessary [50].…”

“…The role of immunohistochemical studies in the differential diagnosis of synovial sarcoma is limited, so the detection of t(X;18)(p11.2;q11.2) translocation resulting in fusion of SYT with SSX1, 2 or 4 present in 90% of SS cases is a hallmark in diagnosis of this malignancy [50,73]. The diagnosis is based on FISH, but this genetic abnormality can also be detected with reverse transcription polymerase chain reaction (RT-PCT) next generation sequencing (NGS) [20,72].…”