Extranodal T-cell lymphoma of the sinonasal tract presenting as severe rhinitis: case series

Systemic diseases such as Wegener granulomatosis, Churg-Strauss syndrome, and sarcoidosis can present initially as severe rhinitis alone. One clinical entity that mimics severe rhinitis and thus poses a particular challenge in this regard is extranodal T-cell lymphoma of the sinonasal tract. Sinonasal lymphoma has a vague initial presentation that masquerades as more common, benign causes of rhinitis but progresses as a "midfacial progressive destructive lesion" and is uniformly fatal if untreated. In the absence of systemic involvement, the precise etiology frequently defies diagnosis. We present a series of illustrative cases to raise awareness of this rare yet lethal disease to help reduce the therapeutic delays that are currently encountered clinically.

A retrospective case series of four patients was reviewed. Each patient presented initially with severe rhinitis but was later diagnosed with extranodal natural killer/T-cell non-Hodgkin lymphoma of the sinonasal tract.

The predominant presenting symptoms were unilateral nasal obstruction (four patients), rhinitis (three patients), and facial pain (two patients). Only one patient reported systemic B symptoms. Initial management of all four patients included repeated biopsies revealing nonspecific inflammation, which resulted in extensive therapeutic delays (mean time until diagnosis 21.5 months). The primary outcomes were two deaths and two complete remissions.

The challenge in making an early diagnosis of T-cell lymphoma of the sinonasal tract has been reaffirmed in our case series. We emphasize that diagnosis requires a high index of clinical suspicion in combination with multiple deep and appropriately processed biopsies that are submitted for immunohistochemistry and molecular studies.