Relapsed extranodal NK/T-cell lymphoma in the mandible, Nasal Type: a case report and review of the literatures

Abstract

Extranodal NK/T-cell lymphoma (NTCL) is rare disease and has strong geographic prevalence. NTCL is an aggressive form of lymphoma which shows necrosis and ulceration. NTCL often shows an angiocentric and angiodestructive growth pattern . This disease is often found in extranodal sites. The nasal region is the most common involving area of nasal-type NTCL. However, nasal-type NTCL may involve the upper aerodigestive tract (UADT) to the level of the larynx, skin and subcutaneous tissue, gastrointestinal tract and testis. However, lung involvement is rare and this is the difference with pulmonary lymphomatoid granulomatosis, which has similar histological findings with nasal-type NTCL. A common complication of NTCL is a hemophagocytic syndrome and this badly affects survival in NTCL. EBV seemed to contribute to the pathogenesis of this syndrome. NTCL is highly fatal malignant neoplasm, but early pathologic diagnosis is highly difficult and has been no consensus for the treatment. The immunohistochemical findings have been major criteriar for the diagnosis and some molecular markers have been used for the evaluation of the prognosis. We recently experienced a case of Nasal-type NTCL in the mandible. Presented case might be a relapsed lesion from the neck. As the primary lesion was diagnosed as peripheral T-cell lymphoma at other hospital, the oral lesion was easily confused as a relapsed peripheral T-cell lymphoma. The case was presented with its immunohistochemical findings, treatment outcome, and review of literatures.