SEER is an authoritative source of information on cancer incidence and survival in the United States. SEER currently collects and publishes cancer incidence and survival data from population-based cancer registries covering approximately 28 percent of the U.S. population.
This is a disease composed of a clonal proliferation of mature, activated gamma-delta T-cells with a cytotoxic phenotype. This group includes cases previously known as subcutaneous panniculitis-like T-cell lymphoma with a gamma-delta phenotype.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see codes 9702/3 or 9709/3.) Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) often presents with generalized skin lesions, preferentially affecting the extremities. The clinical presentation is variable. The neoplasm may be predominantly epidermorphic and present with patches/plaques. Some patients present with deep dermal or subcutaneous tumors with or without epidermal necrosis and ulceration. Dissemination to mucosal or extranodal sites is frequently observed, but involvement of lymph nodes, spleen, or bone marrow is uncommon. B-symptoms such as fever, night sweats, and weight loss are seen in the majority of patients. PCGD-TCL is resistant to multiagent chemotherapy and/or radiation and has a poor prognosis with a median survival of approximately 15 months. Patients with subcutaneous involvement tend to have a more unfavorable prognosis.
Definitive Diagnostic Methods
Clonal rearrangement of TRG@ and TRD@ genes
TRB@ may be rearranged or deleted, but not expressed