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Review
. 2024 Mar 5;9(3):190-201.
doi: 10.1530/EOR-23-0193.

Synovial sarcoma: the misdiagnosed sarcoma

Chengxiang Li  1   2 , Fatime Krasniqi  3   4 , Ricardo Donners  4   5 , Christoph Kettelhack  4   6 , Andreas H Krieg  1   4
Affiliations
Review

Synovial sarcoma: the misdiagnosed sarcoma

Chengxiang Li et al. EFORT Open Rev. .

Abstract

Synovial sarcoma is a rare and highly malignant soft tissue sarcoma. The inconspicuous and diversity of its early symptoms make it a highly misdiagnosed disease. The management of synovial sarcomas is challenging as they are rare and have a poor prognosis. Early and correct diagnosis and treatment are critical for clinical outcomes. Misdiagnosis or delayed diagnosis can have devastating consequences for the patient. The detection of SS18 gene rearrangement is considered a powerful tool in establishing the diagnosis of synovial sarcomas. Biopsies and testing for gene rearrangements are recommended for all patients in whom SS cannot be excluded. Surgery is the mainstay of treatment for synovial sarcomas. Neoadjuvant/adjuvant radiotherapy is recommended for patients with big tumors (>5 cm) or positive resection margins, and neoadjuvant/adjuvant chemotherapy is recommended for patients with high-risk tumors or advanced diseases. This article reviews synovial sarcomas from the perspectives of clinical and radiological presentation, histological and cytogenetic analysis, differential diagnosis, treatment, and prognosis.

Keywords: malignant; misdiagnosed; sarcoma; synovial sarcoma.

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Conflict of interest statement

The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the work reported here.

Figures

Figure 1
Figure 1
Synovial sarcoma of the right plantar in a 36-year-old man. X-rays showed very subtle interdigital calcification and a corresponding large mass on MRI. (A, B) X-rays of the right foot; (C, D) T2 fat-saturated axial and coronal; (E) T1 sagittal.
Figure 2
Figure 2
Histological images of synovial sarcoma. (A) Monophasic synovial sarcoma showing spindle cells arranged in a haphazard pattern (H&E staining, 50×); (B) Biphasic synovial sarcoma comprising a mixture of spindle cells and pseudoacinic epithelial proliferations (H&E staining, 100×).
Figure 3
Figure 3
2.4 × 2 cm synovial sarcoma of the right anterior tibial tendon in a 63-year-old man, which was initially suspected to be a ganglion without the application of contrast. (A–C) T2 fat-saturated axial, sagittal, and coronal; (D) T1 paraaxial.
Figure 4
Figure 4
10 × 7 cm synovial sarcoma of the left thigh in a 34-year-old man. (A, B) T1 coronal and axial; (C) T2 fat-saturated coronal; (D) T2 axial; (E, F) T1 fat-saturated coronal and axial after contract administration; (G, H): enhanced subtraction coronal and axial. This case initially presented with left thigh swelling and tenderness and MRI revealed a heterogeneous mass. A tumor or intramuscular hematoma was suspected. The initial biopsy suggested skeletal muscles without histopathological findings, which increased the suspicion of a hematoma. However, given that the patient was not clearly traumatized, it was insufficient to explain the extensive hemorrhage within the lesion. Therefore, a second biopsy was conducted promptly, revealing a synovial sarcoma with extensive hemorrhage and necrosis. Neoadjuvant chemotherapy, neoadjuvant radiotherapy, and tumor resection were implemented. At the time of writing this article, the patient is 15 months postoperative with no local or distant recurrence.
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