The spectrum of primary mucosal CD30-positive T-cell lymphoproliferative disorders of the head and neck.

Objective To retrospectively investigate the clinicopathologic spectrum of primary mucosal CD30-positive T-cell lymphoproliferative disorders (PTCLDs) of the head and neck. Study Design Archives of PTCLDs primarily arising in head and neck mucosa were reviewed. Immunostaining of CD20, CD3, CD4, CD8, CD30, CD56, anaplastic lymphoma kinase (ALK), epithelial membrane antigen (EMA), cytotoxic molecules (TIA-1, granzyme B, or perforin), and Ki67; in situ hybridization for Epstein-Barr virus; and T-cell receptor gene rearrangement analysis were performed. Results Fourteen cases of primary mucosal anaplastic large cell lymphoma (M-ALCL) were identified, and no lymphomatoid papulosis (LyP) cases were found. All cases demonstrated atypical mononuclear neoplastic cells with diverse histology and cytomorphology. The typical immunophenotype of neoplastic cells was CD3-positive, CD4-positive, CD8-negative, CD30-positive, ALK-negative, and cytotoxic molecules–positive. Infiltration of inflammatory cells was common. All cases presented an indolent course, regardless of therapy. Conclusions PTCLDs of the head and neck provisionally included M-ALCL alone.