Presentation
Gradually increasing right-sided facial swelling associated with limited mouth opening and pain for the last two months. Past history of chronic ulcer in the right upper buccal vestibule.
Patient Data
T2 hyperintense diffuse infiltrative disease process involving several right sided head and neck spaces (buccal, masticator, parotid, submandibular & parapharyngeal spaces and pterygopalatine & infratemporal fossae), showing no restriction on DWI and mild to moderate enhancement on the postcontrast study. A few enlarged, enhancing bilateral level I & II lymph nodes, showing restricted diffusion. Small fluid collection in the right buccal space. No marrow abnormality is seen in the underlying mandible. Opacified right sided paranasal sinuses.
Infiltrative heterogeneously hypermetabolic (SUV=24.4) disease involving multiple spaces of the right side of the head and neck (buccal, masticator, parotid, carotid & parapharyngeal spaces), submandibular area and pterygopalatine fossa with extension to the right nasal cavity, right maxillary sinus and right orbit. Hypermetabolic parapharyngeal & bilateral level II lymph nodes. Small air locules in the right masticator space (superimposed infection). No hypermetabolic lymphadenopathy is seen in the chest, abdomen & pelvis.
Significant interval regression of the previously described infiltrative hypermetabolic disease in the right side of the face and neck, leaving behind residual hypermetabolic process in the masticator space (SUV=9.2). Mild residual hypermetabolic disease is also noted in the right nasal cavity and right submandibular region. Small residual right cervical level II lymph node (SUV=4.8).
Procedure: Incisional biopsy from right buccal space ulcerative lesion.
Diagnosis: Extra-nodal NK/T-cell lymphoma, nasal type.
Immunostains: The immunostains with the antibodies anti-CD3, CD2, CD43, CD45, granzyme B, p53, EBV (LMP1) are positive in the tumor cells; however, CD7, CD25, CD56, CD99, & CD20 are negative. EBV-EBER and TIA CD30 are also positive. Ki-67 is highly expressed (>90% of cells). The PAS and Grocott special stains are negative for microorganisms.
Case Discussion
Sinonasal lymphoma (both T & B-cell types) is famous for producing large soft tissue masses with a tendency of remodeling of the adjacent bones; however, bone destruction can be seen in nasal T/NK-cell variety 1. The soft tissue masses are usually isointense to muscle on T1, mildly hyperintense to muscle & hypointense to the mucosa on T2-weighted imaging and enhance moderately on the post-contrast T1-weighted imaging 1. Other diseases with similar imaging features as lymphoma (involving the nasal cavity and paranasal sinuses) are granulomatosis with polyangiitis, extramedullary plasmacytoma/ multiple myeloma, chloroma and Rosai-Dorfman disease 1.
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