Name

Primary cutaneous gamma-delta T-cell lymphoma

ICD-O-3 Morphology

9726/3: Primary cutaneous gamma delta T-cell lymphoma
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

C440-C449, C490-C499, C510-C519, C600-C609, C632
Cutaneous (skin) lymphoma which presents with generalized skin lesions, mostly affecting the extremities (arms and legs). See Module 7.

Grade

Not Applicable

Module Rule

None

Alternate Names

PCGD-TCL

Definition

Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) is a lymphoma involving primarily the skin, composed of a clonal proliferation of mature, activated gamma delta T cells with a cytotoxic phenotype.

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see codes 9702/3 or 9709/3.)

1. Epidermorphic with presentation of patches and/or plaques.
2. Deep dermal or subcutaneous tumors with or without epidermal necrosis and ulceration.
3. Dissemination to mucosal or extranodal sites. Involvement of lymph nodes, spleen, or bone marrow is uncommon.

Resistant to multiagent chemotherapy and/or radiation and has a poor prognosis. Patients with subcutaneous involvement tend to have a more unfavorable prognosis.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

EBV negative
TR genes are clonally rearranged

Immunophenotyping

Beta F1 negative
CD2 positive
CD3 positive
CD4 negative
CD5 negative
CD7 positive and negative
CD8 negative
CD56 positive
Granzyme B expression
Perforin expression
TCR positive
TIA1 expression

Treatments

Chemotherapy
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Corresponding ICD-9 Codes

202.7 Peripheral T-cell lymphoma

Corresponding ICD-10 Codes

C84.4 Peripheral T-cell lymphoma

Corresponding ICD-10-CM Codes (U.S. only)

C84.A Cutaneous T-cell lymphoma, unspecified (effective October 01, 2015)

Signs and Symptoms

Deep dermal subcutaneous tumors with or without necrosis and ulceration
Drenching night sweats
Fever (for no known reason)
Pain in the chest, abdomen or bones (for no known reason)
Skin patches/plaques
Weight loss (for no known reason)

Diagnostic Exams

Progression and Transformation

None

Epidemiology and Mortality

Age: mostly in adults
Sex: no male or female predominance

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 397-399

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary