Diffuse swelling of the buccal mucosa and palate as first and only manifestation of an extranodal non-Hodgkin 'double-hit' lymphoma: report of a case.
BACKGROUND
Most of the lymphomas arising in the oral cavity are of B-cell origin. Among these, diffuse large B-cell lymphomas are the most common. Diffuse large B-cell lymphomas may exhibit more than one chromosomal rearrangement and are then referred to as 'double-hit' or 'triple-hit' lymphomas.


METHODS
We present a case of an intraoral 'double-hit' lymphoma in a 76-year-old male who had been referred by an oral surgeon in private practice. Intraoral examination exhibited a firm, exophytic lesion in the region of the right hard palate and buccal mucosa with extension to the soft palate. Radiographic examination exhibited a massive thickening of the right sinus membrane, and arrosion of the lateral and basal cortical sinus walls in the right maxilla. After diagnosis of the lesion, the patient was treated with six cycles of chemotherapy.


CONCLUSIONS
Lymphomas arising within the oral cavity account for less than 5% of all oral malignancies and typically affect the palatine tonsils and the palate. 'Double-hit' lymphomas are associated with older age, usually present with an advanced stage of disease, and show an aggressive clinical behaviour. They normally have a poor prognosis, even when treated with intensive chemotherapy regimens. Nevertheless, in the case presented, the patient was free of symptoms 1 year after initial diagnosis.
Frei
Marc
M
Department of Oral Surgery and Stomatology, School of Dental Medicine, University of Bern, Freiburgstrasse 7, 3010 Bern, Switzerland.
Dubach
Patrick
P
Reichart
Peter A
PA
Schmitt
Anja M
AM
Mueller-Garamvölgyi
Esther
E
Bornstein
Michael M
MM
D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000235:genetics; D002610:Cheek; D004268:DNA-Binding Proteins / Q000235:genetics*; D005500:Follow-Up Studies; D015322:Gene Rearrangement, B-Lymphocyte / Q000235:genetics*; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D008297:Male; D009061:Mouth Mucosa / Q000473:pathology*; D009062:Mouth Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D009367:Neoplasm Staging; D010157:Palatal Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000235:genetics*; D051560:Proto-Oncogene Proteins c-bcl-6; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics*; D014057:Tomography, X-Ray Computed
C084103:BCL6 protein, human; D014408:Biomarkers, Tumor; D004268:DNA-Binding Proteins; C489427:MYC protein, human; D019253:Proto-Oncogene Proteins c-bcl-2; D051560:Proto-Oncogene Proteins c-bcl-6; D016271:Proto-Oncogene Proteins c-myc