Hydroa vacciniforme-like lymphoma

Hydroa vacciniforme-like lymphoma
ICD-O-3 Morphology
9725/3: Hydro vacciniforme-like lymphoma
Effective 2010 and later
for cases diagnosed 2010 and later
Primary Site(s)
C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632
Cutaneous (skin) lymphoma condition that primarily affects sun exposed skin, most commonly the face. See Module 7.

Not Applicable
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. (See also 9724/3: Systemic EBV-positive T-cell lymphoproliferative disease of childhood)

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.

Systemic spread and mosquito bite allergy/hypersensitivity result in a much more aggressive clinical course.

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 (U.S. only)
C84.Z Other mature T/NK-cell lymphomas (effective October 01, 2015)
Signs and Symptoms
Papulovesicular eruption resulting in ulceration and scarring
Diagnostic Exams
Progression and Transformation
Recurrent skin lesions for 10-15 years before progression to systemic involvement
Epidemiology and Mortality
Age: mainly children and adolescents
Country: Asia, Native Americans from Central and South America, Mexico