Primary T-cell non-Hodgkin lymphoma of the larynx with subsequent cutaneous involvement.

BACKGROUND

T lymphocytes expressing the gammadelta T-cell receptor represent a minority of normal T lymphocytes and are mostly located in the spleen or mucosa. Lymphomas expressing the gammadelta T-cell receptor are rare and usually present as hepatosplenic (negative for Epstein-Barr virus) disease. Primary lymphomas of the larynx are also rare.

OBJECTIVE

To report the first case of primary laryngeal gammadelta T-cell lymphoma related to Epstein-Barr virus infection.

DESIGN

Single-case study, including clinical, histological, immunohistochemical, and ultrastructural analysis, and in situ hybridization for Epstein-Barr virus-encoded small nuclear RNA.

PATIENT: An 88-year-old man presenting with a 6-month history of a cough followed by progressive dysphonia and a thickening of the left aspect of the aryepiglottic fold.

INTERVENTION: Two weeks of treatment with corticosteroids and antibiotics, followed by radiotherapy and then chemotherapy with chlorambucil and corticosteroids.

OUTCOME: The patient died of heart failure 10 months after the onset of the disease.

RESULTS

The tumor was laryngeal and disseminated to the skin over the parotid gland. Tumor cells were medium-sized T cells of cytotoxic immunophenotype, expressed the gammadelta T-cell receptor, and contained azurophilic granules and cytotoxiclike granules detected on electron microscopy. Epstein-Barr virus-encoded small nuclear RNA was detected in most tumor cells.

CONCLUSIONS

Lymphomas with a T-cell cytotoxic phenotype expressing the gammadelta T-cell receptor are rare, and this case appears to be the first to involve the larynx. The association between Epstein-Barr virus and T-cell lymphomas has been shown to be frequent in the upper respiratory tract and is confirmed in this case. This finding suggests that T cells in the upper respiratory tract may be more exposed to Epstein-Barr virus infections, perhaps because of their anatomical location.