Hydroa vacciniforme-like lymphoma

Hydroa vacciniforme-like lymphoma
ICD-O-3 Morphology
Effective 2010 and later
for cases diagnosed 2010 and later
Primary Site(s)
C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632

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5 - T-cell
Module Rule
Alternate Names
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.
Abstractor Notes
(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."
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Genetics Data
Clonally rearranged T-cell receptor (TCR) genes
EBER in situ hybridization
Monoclonal EBV
Other RX
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
202.8 Other lymphoma
Corresponding ICD-10 Codes
C84.5 Other and unspecified T-cell lymphomas
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C84.Z Other mature T/NK-cell lymphomas
Signs and Symptoms
Diagnostic Exams
Recurrence and Metastases
Epidemiology and Mortality