Abstract
Introduction and importance
Synovial sarcoma is a malignant soft tissue tumor typically found near joints; its occurrence in the inguinal region is very rare.
Case presentation
We report a 23-years-old who presented with lower limb swelling. Imaging studies revealed a tumor in the groin area, compressing the femoral vein. A trucut biopsy concluded a synovial sarcoma. A complete resection was performed and the patient had adjuvant radiotherapy and chemotherapy with no evidence of reccurrence at 2-years follow-up.
Clinical discussion
Synovial sarcoma accounts for approximately 8 to 10 % of all soft tissue sarcomas. It is predominantly localized near the large joints in the limbs, with the inguinal location being extremely rare.
Clinical diagnosis of the mass can sometimes be challenging. A needle biopsy, followed by histological analysis, is necessary to establish the diagnosis. MRI is considered the gold standard radiological examination for local staging of the tumor.
The main treatment approach for synovial sarcoma is wide-margin resection, involving en-bloc resection of the tumor with clear margins. Vascular resection and reconstruction should be considered for involved vessels.
Some authors argue that resection alone is sufficient for treating primary synovial sarcoma. However, adjuvant chemotherapy may be effective in cases where surgery quality is poor, making it a non-standard treatment. Others have highlighted the potential benefits of adjuvant radiotherapy, particularly in high-grade tumors.
Conclusion
Surgical excision remains the mainstay of treatment. Therefore, it is necessary to be aware of the different clinical presentations, which can sometimes be unusual.
Keywords: Case report, Synovial sarcoma, Inguinal region, Surgical resection, Adjuvant treatments
Highlights
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Synovial sarcoma is a malignant soft tissue tumor typically found near joints, its occurrence in the inguinal region is very rare.
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Clinical diagnosis of the mass can be challenging.
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The main treatment approach for synovial sarcoma is complete surgical excision
1. Introduction and importance
Synovial sarcoma is a slow-growing malignant tumor primarily affecting the soft tissues near the extremities and joints. In rare cases, it can occur in the groin area. It is locally invasive and can metastasize to distant sites [1]. While this tumor typically presents as a firm mass adhering to deep planes, in some instances, it may manifest as a mass effect on adjacent structures, such as vascular compression. Here, we report a case of synovial sarcoma in the groin area of a 23-year-old male, presenting as limb swelling due to femoral vein compression. This work has been reported in line with the SCARE criteria [2].
2. Case presentation
A 23-year-old male with no prior medical history presented with swelling of the entire right lower limb. Clinical examination revealed edematous infiltration and taut skin throughout the limb, without signs of inflammation. A tumefaction adhering to the deep planes was found at the groin fold, hindering thigh flexion. Ultrasound showed a hypoechoic mass with heterogeneous areas, irregular margins, and decreased but permeable flow in the femoral vein on Doppler. A trucut biopsy confirmed synovial sarcoma.
To assess the locoregional extent, magnetic resonance imaging (MRI) was performed, revealing a tissular process measuring 37x58x62mm within the distal portion of the ilio-psoas muscle, near its insertion on the minor trochanter. The mass exhibited lobulated contours, high signal intensity on T1, and moderate enhancement. It was in contact with the common femoral artery and femoral vein, causing its compression. No lymphatic spread or distant metastases were detected. (See Fig. 1.)
Fig. 1.
MRI section showing a mass with high signal on T1 WIexerting a mass effect on femoral pedicle.
After a multidisciplinary consultation, an initial conservative treatment was decided. Surgery was performed, revealing a solid, well-limited tumor with close contact to the femoral pedicle (Fig. 2). The femoral vein and artery were meticulously dissected and freed. The entire tumor was resected en bloc (Fig. 3). The patient was discharged after 4 days with an uneventful postoperative course and progressive regression of leg swelling.
Fig. 2.
Per-operative view showing the tumor in contact with the femoral pedicle before dissection.
Fig. 3.
The surgical specimen measuring 8 × 6 × 4cm which presents macrospically necrotic, hemorrhagic and cystic remodelings.
Pathological examination of the surgical specimen confirmed a biphasic synovial sarcoma. Resection margins were clear. Macroscopically, the mass consisted of a sarcomatous proliferation comprising spindle and epithelial cell components, surrounded by a fibrous pseudo capsule with focal necrosis (Fig. 4).
Fig. 4.
Microscopic section of the tumor showing sarcomatous proliferation made up of two components: a spindle cell component and an epithelial component.
The patient received adjuvant radiotherapy and chemotherapy and was followed up for 2 years without recurrence.
3. Clinical discussion
Synovial sarcoma, a high-grade primary mesenchymal malignancy tumor, accounts for approximately 8 to 10 % of all soft tissue sarcomas [3]. It carries a risk of distant metastasis and is predominantly localized near the large joints in the limbs, with the inguinal location being extremely rare. This tumor primarily affects young adults, particularly those in the second and third decades of life, with a slight male predominance [4].
The most common clinical presentation of synovial sarcoma is a deep, firm, slowly growing mass that may result in limb mobility limitations. Clinical diagnosis of the mass can sometimes be challenging. In our case, the diagnosis was guided by clinical signs of venous compression.
Synovial sarcoma exhibits a cellular component consisting of epithelial cells and fibrosarcomatoid spindle cells, and it can present in three forms: biphasic, monophasic, and undifferentiated. In our case, it was presented as the biphasic form. This tumor is associated with a chromosomal translocation t(X;18) (p11;q11) [5].
While ultrasound of the soft tissue serves as a fast and simple diagnostic tool, a needle biopsy followed by histological analysis is necessary to establish the diagnosis. MRI is considered the gold standard radiological examination for local staging of the tumor, and computed tomography of the chest or bone scan is usually used to evaluate distant metastasis [6].
The main treatment approach for synovial sarcoma is wide-margin resection, involving en-bloc resection of the tumor with clear margins. However, when the tumor is in close proximity to neurovascular structures, careful dissection is required to preserve these structures. Vascular resection and reconstruction should be considered for involved vessels [7].
Some authors argue that resection alone is sufficient for treating primary synovial sarcoma. A study by A. Le Cesne et al. reported no significant improvement in overall survival for young patients who received adjuvant chemotherapy. However, adjuvant chemotherapy may be effective in cases where surgery quality is poor, making it a non-standard treatment [8]. Others have highlighted the potential benefits of adjuvant radiotherapy, particularly in high-grade tumors. Neoadjuvant or adjuvant radiotherapy is recommended for tumors exceeding 5 cm, as it improves local control and may provide an overall survival benefit [9].
4. Conclusion
Inguinal synovial sarcoma is a soft tissue tumor with a widely varying prognosis. Surgical excision remains the mainstay of treatment. Therefore, it is necessary to be aware of the different clinical presentations, which can sometimes be unusual, such as the venous compression described in our clinical case.
Consent
Written informed consent was obtained from the patient for publication and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.
Ethical approval
Ethics clearance was not necessary. It is a case report describing a rare clinical presentation.
Funding
N/A.
Author contribution
J.C: conceived the work, contiubuted to writing, reviewing and finalization of the manuscript.
I.K: contiubuted to writing and finalization of the manuscript.
I.B: contiubuted to writing, reviewing and finalization of the manuscript.
A.B: contiubuted to writing and finalization of the manuscript.
M.B: approval of the final version.
A.J: approval of the final version.
Guarantor
Imen Bouassida.
Research registration number
It is not a first in Man. It is a case report about a rare clinical presentation.
Declaration of competing interest
N/A.
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