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Establishment of a Primary Culture of Patient-derived Soft Tissue Sarcoma
Published on: April 11, 2018
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Primary Orbital Synovial Sarcoma Mimicking a Periocular Cyst
Francesca Portelli1, Giulia Pieretti2, Nicola Santoro2
1Histopathology and Molecular Diagnostics, Department of Health Sciences, Careggi University Hospital, Florence, Italy.
The American Journal of Dermatopathology
|January 10, 2019
Summary
Synovial sarcoma (SS), a rare soft-tissue cancer, can occur in the head and neck. This case highlights an unusual orbital location of monophasic SS, initially misdiagnosed as a cyst.
Area of Science:
- Oncology
- Pathology
- Ophthalmology
Background:
- Synovial sarcoma (SS) is a high-grade soft-tissue sarcoma primarily affecting young adults.
- Head and neck SS is rare, accounting for <0.1% of head and neck cancers.
- Orbital synovial sarcoma is exceptionally rare, with limited documented cases.
Observation:
- A case of monophasic synovial sarcoma is presented, originating in the left supero-nasal orbit.
- The patient was a 24-year-old woman.
- The tumor presented with a clinically benign appearance, initially mistaken for a periocular cyst.
Findings:
- Synovial sarcoma is characterized by a specific translocation t(X;18)(p11.2;q11.2) in over 95% of cases.
- Histologically, SS can be monophasic, biphasic, or poorly differentiated.
- This case involved monophasic SS.
Implications:
- The unusual orbital location and benign clinical presentation underscore the importance of considering rare diagnoses.
- Synovial sarcoma is known for late recurrences and metastases, necessitating vigilant follow-up.
- Accurate diagnosis is crucial for appropriate management of orbital soft-tissue sarcomas.
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