Retromolar monophasic synovial sarcoma: a rare entity in a rare location

Synovial sarcoma (SS) is a rare tumour that arises mainly in the peri articular tissues of the extremities. The majority of SS are biphasic on histology. Monophasic synovial sarcoma (MSS) of the mandible is a rare, aggressive malignancy having non-specific clinical and histological features with a tendency for distant metastasis. A 17- year-old girl presented to the surgical clinic with a gradually enlarging, painless ulcerated, right retro molar mass. Biopsy of the lesion revealed mitotically active, elongated, spindle cells arranged haphazardly and as fascicles with a hemangiopericytoma-like vascular pattern. The differential diagnosis included both odontogenic and non-odontogenic tumours. Immunohistochemistry showed positivity for AE1/AE3, CD99 and BCL2. CD34, CD31, SMA, CD117 and p63 were negative, thereby confirming a diagnosis of MSS. This case highlights key features of MSS and focuses on the importance of including it in the working differential diagnosis of malignant spindle cell tumours of the oral cavity.

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Publication Details

Journal
Journal of Diagnostic Pathology
Published
2024-12-31
DOI
https://doi.org/10.4038/jdp.v19i2.7869
Primary Topic
Sarcoma Diagnosis and Treatment
Type
article
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article

Retromolar monophasic synovial sarcoma: a rare entity in a rare location

Iranthi Kumarasinghe
Journal of Diagnostic Pathology
Sarcoma Diagnosis and Treatment
article

Retromolar monophasic synovial sarcoma: a rare entity in a rare location

Iranthi Kumarasinghe
article en

Abstract

Synovial sarcoma (SS) is a rare tumour that arises mainly in the peri articular tissues of the extremities. The majority of SS are biphasic on histology. Monophasic synovial sarcoma (MSS) of the mandible is a rare, aggressive malignancy having non-specific clinical and histological features with a tendency for distant metastasis. A 17- year-old girl presented to the surgical clinic with a gradually enlarging, painless ulcerated, right retro molar mass. Biopsy of the lesion revealed mitotically active, elongated, spindle cells arranged haphazardly and as fascicles with a hemangiopericytoma-like vascular pattern. The differential diagnosis included both odontogenic and non-odontogenic tumours. Immunohistochemistry showed positivity for AE1/AE3, CD99 and BCL2. CD34, CD31, SMA, CD117 and p63 were negative, thereby confirming a diagnosis of MSS. This case highlights key features of MSS and focuses on the importance of including it in the working differential diagnosis of malignant spindle cell tumours of the oral cavity.

Journal of Diagnostic PathologyVol. 19(2)
Openalex Percentile: Top 54%
Sarcoma Diagnosis and Treatment
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Retromolar monophasic synovial sarcoma: a rare entity in a rare location — Iranthi Kumarasinghe · Journal of Diagnostic Pathology (2024) | TGRS Research Map | TGRS