Primary Head and Neck Mucosal CD30-Positive T-Cell Lymphoproliferative Disorder

**Semantic type:** Disease or Syndrome

**Definition:** A spectrum of T-cell lymphoproliferative disorders that arise from the mucosal sites of the head and neck. They are characterized by the presence of CD30-positive neoplastic lymphoid cells with pleomorphic nuclei and abundant cytoplasm. Cells that resemble the hallmark cells of anaplastic large cell lymphoma are often seen. The lesions usually arise from the oral cavity and, less frequently, the conjunctiva, orbit, and nasopharynx. In most cases complete remission is achieved, following excision, with or without radiotherapy, and with or without systemic chemotherapy.

**Synonyms:** - Primary Mucosal CD30-Positive T-Cell Lymphoproliferative Disorder