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.