Abstract Details

Title Recurrence of a Chondroid Tenosynovial Giant Cell Tumor in the Temporomandibular Joint

Topic Neuro-oncology

Presentation(s) P9 - Poster Session 9 (11:45 AM-12:45 PM)

Poster/Presentation
Number 6-013

Objective To describe radiologic, pathologic, and clinical findings associated with a chondroid tenosynovial giant cell tumor (CTGCT), harboring a fibronectin 1 (FN1) tyrosine endothelial kinase (TEK) gene fusion that recurred within 1 year of initial resection.

Background

Chondroid tenosynovial giant cell tumors (CTGCT) are indolent tumors with predilection for the temporomandibular joint (TMJ) region. These tumors can be locally aggressive and invade the synovium of joints, tendons, or bursa. CTGCTs can harbor FN1::TEK fusions, which may define a distinct entity with a synoviocyte cell of origin. FN1 encodes fibronectin, a glycoprotein participating in cell adhesion and migration processes. When fused with a receptor tyrosine kinase, like TEK, it can lead to aberrant cell signalization and promote tumorigenesis. Recurrence rate is low (10-15%) following gross total resection (primary treatment).

Design/Methods Case report and review of literature.

Results A 48-year-old male with history of multiple concussions underwent surveillance MRI. Imaging identified a 3.9x2.8 cm extra-axial intracranial lesion with heterogeneous enhancement and T2 hyperintensity. It was difficult to tell whether origin was bone or joint and therefore, differential diagnosis included chondrosarcoma, chondroma, metastatic disease, meningioma, solitary fibrous tumor, and calcium pyrophosphate deposition disease. Patient underwent gross total resection. Histological evaluation demonstrated proliferation of epithelioid cells with eccentric nuclei and eosinophilic cytoplasm admixed with osteoclast-type giant cells. Molecular testing was done to assist with classification. This identified an FN1::TEK fusion, consistent with diagnosis of CTGCT. Ten months after surgery, imaging demonstrated tumor recurrence. Additional tissue analysis was undertaken, which identified positivity for colony stimulating factor 1 (CSF-1). Treatment options include repeat resection and radiation therapy.

Conclusions CTGCT should be included in the differential diagnosis of intracranial extra-axial osseous mass lesions. Optimal management of recurrent tumors is unclear, but surgical resection is the only option that has been successfully trialed.

Authors/Disclosures
Anusha Khawaja, MD PRESENTER	Dr. Khawaja has nothing to disclose.
Giuseppe Lanzino, MD (Mayo Clinic)	Dr. Lanzino has nothing to disclose.
Jorge Torres-Mora, MD	Dr. Torres-Mora has nothing to disclose.
Andrew Folpe, MD	Dr. Folpe has nothing to disclose.
Derek Johnson, MD (Mayo Clinic)	Dr. Johnson has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Telix.
Ugur Sener, MD	Dr. Sener has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Servier Pharmaceuticals. Dr. Sener has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Dr. Sener has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion.

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