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.


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


METHODS
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.


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


RESULTS
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.
Marianowski
R
R
Department of Oto-Rhino-Laryngology and Head and Neck Surgery, Hôpital Lariboisière, Paris, France.
Wassef
M
M
Amanou
L
L
Herman
P
P
Tran-Ba-Huy
P
P
D000368:Aged; D000369:Aged, 80 and over; D006566:Herpesviridae Infections / Q000473:pathology*; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D007822:Laryngeal Neoplasms / Q000473:pathology* / Q000821:virology; D007830:Larynx / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000473:pathology* / Q000821:virology; D016410:Lymphoma, T-Cell, Cutaneous / Q000473:pathology* / Q000821:virology; D008297:Male; D012367:RNA, Viral / Q000302:isolation & purification; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis*; D012878:Skin Neoplasms / Q000473:pathology* / Q000821:virology; D014412:Tumor Virus Infections / Q000473:pathology*
D012367:RNA, Viral; D016692:Receptors, Antigen, T-Cell, gamma-delta