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 an EBV-positive cutaneous T-cell lymphoma (sometimes can be NK-cells) occurring in children, and associated with sensitivity to insect bites and sun. Like other EBV-positive T-cell and NK-cell lymphomas, predisposition for this condition may be related to a defective cytotoxic immune response to EBV.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9702/3). Hydroa vacciniforme-like lymphoma is one of two major types of Epstein-Barr (EBV) associated T-cell lymphoproliferative disorders that have been reported in the pediatric age group. (The other is systemic EBV-positive T-cell lymphoproliferative disease of childhood, 9724/3.) Both occur with increased frequency in Asians and in Native Americans from Central and South America and Mexico.
This is a cutaneous condition that primarily affects sun exposed skin, in particular the face. The neoplastic cells are generally small to medium in size without significant atypia. The infiltrates show extension from the epidermis to the subcutis, showing necrosis, angiocentricity and angioinvasion. The overlying epidermis is frequently ulcerated. This condition is seen mainly in children and adolescents from Asia, or in Native Americans from Central and South America and Mexico. This disease is rare in adults. The clinical course is variable and patients my have recurrent skin tumors for some time, up to 10-15 years, before progression to systemic involvement. With systemic spread, the clinical course is much more aggressive. Mosquito bite allergy/hypersensitivity is clinically more aggressive.
This disease is treated with UV (ultra-violet light) or PUVA (psoralen plus ultraviolet light). UV and PUVA are both coded as "Other Treatment."