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

Abstract

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.

• Authors: R Marianowski, M Wassef, L Amanou, P Herman, P Tran-Ba-Huy
• Journal: Archives of otolaryngology--head & neck surgery (Arch Otolaryngol Head Neck Surg) Vol. 124 Issue 9 Pg. 1037-40 (Sep 1998) ISSN: 0886-4470 [Print] United States
• PMID: 9738818 (Publication Type: Case Reports, Journal Article)

Chemical References

• RNA, Viral
• Receptors, Antigen, T-Cell, gamma-delta

Topics

• Aged
• Aged, 80 and over
• Herpesviridae Infections (pathology)
• Herpesvirus 4, Human (isolation & purification)
• Humans
• Laryngeal Neoplasms (pathology, virology)
• Larynx (pathology)
• Lymphoma, T-Cell (pathology, virology)
• Lymphoma, T-Cell, Cutaneous (pathology, virology)
• Male
• RNA, Viral (isolation & purification)
• Receptors, Antigen, T-Cell, gamma-delta (analysis)
• Skin Neoplasms (pathology, virology)
• Tumor Virus Infections (pathology)