Angiocentric T-cell lymphoma presenting as lethal midline granuloma

Abstract

Background: Lethal midline granuloma (LMG) is a rare condition characterized by rapidly progressive midfacial destruction. Most LMG cases are angiocentric T-cell lymphomas and an association with Epstein-Barr virus (EBV) has been reported. Cutaneous involvement is poorly described and the prognosis not well documented.

Methods: We report a case of angiocentric T-cell lymphoma of the palate that presented as LMG with concurrent discrete skin lesions composed of two distinct morphologic appearances: indurated nodules and annular plaques. The English language literature for LMG-type angiocentric T-cell lymphoma is reviewed and a survival analysis of 58 cases with follow-up data (including our own case) is performed.

Results: The 1- and 5-year survival rates were 45% +/- 7% and 22% +/- 9%, respectively. Poor survival was associated with advanced age and stage. EBV DNA was detected in 16 out of 21 reported cases in which it was sought (including our case).

Conclusions: We present photographic documentation of a broader spectrum of cutaneous lesions in the LMG-type angiocentric T-cell lymphoma than has previously been described, and have confirmed the association with EBV. The prognosis is poor. Aggressive therapy such as bone marrow transplantation should be considered early in the course.