Lymphomatoid granulomatosis presenting with gingival involvement in an immune competent elderly male.
Lymphomatoid granulomatosis (LYG) represents a B cell lymphoproliferative disorder that appears to be driven by infection of the lesional cells by Epstein-Barr virus (EBV). Although not a common condition, the overwhelming majority of cases affect the lungs and mediastinal lymph nodes. Oral mucosal involvement has been documented in only one other report. We describe an 82-year-old man who developed a chronic oral ulcer following extraction of a mandibular molar tooth. Biopsy of the ulcer identified large atypical mononuclear cells that had a B cell immunophenotype and were associated with the walls of several arterioles in the sample. In situ probes for EBV-encoded small RNA showed prominent labeling of these large cells, suggesting the possibility of LYG. Imaging studies identified mediastinal and hilar lymphadenopathy on CT imaging, while PET scans showed hypermetabolic activity in the lymph nodes as well as the left mandible. Based on these findings, a diagnosis of LYG was made and the patient was treated with rituximab, an anti-CD20 monoclonal antibody, combined with a chemotherapeutic regimen consisting of etoposide, prednisone, vincristine, cyclophosphamide and doxorubicin. After three cycles of therapy, the oral ulcer resolved significantly, as well as the areas of hypermetabolic nodal activity. Remission continued for 3 years, however the patient eventually developed non-small cell carcinoma of the lung and expired as a result of that tumor.
Division of Hematology Oncology, Department of Internal Medicine, College of Medicine and Public Health, The Ohio State University, Columbus, OH 43210, USA.
Lapo
L
Alinari
Shubham
S
Pant
Kristin
K
McNamara
John R
JR
Kalmar
William
W
Marsh
Carl M
CM
Allen
Robert A
RA
Baiocchi
D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002289:Carcinoma, Non-Small-Cell Lung / Q000473:pathology; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005887:Gingival Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D006801:Humans; D008175:Lung Neoplasms / Q000473:pathology; D008230:Lymphomatoid Granulomatosis / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D008297:Male; D016609:Neoplasms, Second Primary / Q000473:pathology