Synovial Sarcoma

It is crucial to distinguish an OFD and an adamantinoma from their various mimics, given treatment-associated implications. A long-term follow-up is suggested, as recurrences and metastases can occur late during the disease course.

Although the diagnosis was established based on morphology and immunoprofile, adverse histologic features, such as increased mitotic activity and high cellularity, suggest potential malignant behavior, warranting close clinical follow-up. This case underscores the importance of thorough histopathological and immunohistochemical evaluation in distinguishing SFT from other spindle cell lesions of the prostate, particularly given its extreme rarity and potential implications for patient management and follow-up.

Postoperatively, the patient received adjuvant chemotherapy with the AIM (doxorubicin, ifosfamide, and mesna) regimen. Given the extreme rarity of PRSS, we discuss the diagnostic challenges, molecular characteristics, and treatment approach adopted at our institution, contributing to the limited but growing body of knowledge on this rare entity.

P2, N=110, Recruiting, Oslo University Hospital | Not yet recruiting --> Recruiting

P2, N=40, Recruiting, Oslo University Hospital | Not yet recruiting --> Recruiting

Eleven years after treatment, the patient remains free of recurrence. Understanding BSNS is essential to avoid excessive intervention, and confirmation of PAX3 rearrangement by FISH or equivalent molecular testing is crucial for accurate diagnosis.

Genomics-guided therapy in sarcoma is feasible and impactful. Expanding timely access to molecular profiling is essential for advancing precision oncology in the MENA region.

The chemotherapy regimen of ifosfamide, mesna and doxorubicin proved effective for this condition, leading to a significant reduction in tumour size and metabolic activity. However, due to disease recurrence and the presence of a KDM5A-positive marker, second-line therapy with trabectedin and pazopanib became necessary.

Resection subsequently revealed a biphasic morphology, and molecular testing confirmed the presence of an SS18::SSX fusion, confirming the diagnosis of synovial sarcoma. This case highlights a potential diagnostic pitfall in dermatopathology and stresses the importance of confirmatory molecular testing in cases where both sarcoma and carcinoma are included in the differential diagnosis.

The patient underwent wide surgical excision with negative margins and recovered uneventfully. This case emphasizes the importance of maintaining synovial sarcoma in the differential diagnosis of deep thigh masses in older adults, where imaging and molecular confirmation are essential to avoid misdiagnosis and treatment delay.

Talazoparib combined with ATR inhibitor possesses potential application as a therapeutic option for SS.