Primary cutaneous T-cell lymphoma
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A group of lymphoproliferative disorders characterized by localization of neoplastic T lymphocytes to the skin
Code 9709/3 is now used for two variants of primary cutaneous T-cell lymphomas; primary or cutaneous CD4-positive small/medium T-cell lymphoma and there is a pre-2010 tumor coded to 9709/3 there should be careful follow-back to determine whether this is a new primary or a more specific diagnosis of T-cell cutaneous lymphoma.
Primary cutaneous CD8-positive aggressive epidermotropic cytoxic T-cell lymphoma presents with generalized skin lesions. This neoplasm is characterized by localized or disseminated eruptive papules, nodules, and tumors showing central ulceration and necrosis or by superficial hyperkeratotic patches and plaques. This neoplasm may disseminate to other visceral sites (lung, testis, CNS, and oral mucosa) but lymph nodes are seldom affected. This lymphoma often has an aggressive clinical course with a median survival of 32 months. There is no difference in survival between cases with a small or large cell morphology.
Primary cutaneous CD4-positive small/medium T-cell lymphoma usually presents with a solitary plaque or nodule, most commonly on the face, neck, or upper trunk. Involvement of lower extremities is rare. By definition, there should be an absence of patches typical of Mycosis fungoides. Patients are generally asymptomatic with the detection of a single skin lesion being the sole manifestation of disease. A minority of patients may present with large tumors or multiple skin lesions. This lymphoma has a rather favorable prognosis with an estimated 5-year survival of approximately 80%. Those patients with a single lesion have a an excellent prognosis and are treated with surgical excision or radiotherapy.