Primary cutaneous gamma-delta T-cell lymphoma

Name
Primary cutaneous gamma-delta T-cell lymphoma
ICD-O-3 Morphology
9726/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
C440-C449, C490-C499, C510-C519, C600-C609, C632

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Grade
5 - T-cell
Module Rule
None
Alternate Names
PCGD-TCL
Definition
This is a disease composed of a clonal proliferation of mature, activated gamma-delta T-cells with a cytotoxic phenotype. This group includes cases previously known as subcutaneous panniculitis-like T-cell lymphoma with a gamma-delta 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.) Primary cutaneous gamma-delta T-cell lymphoma (PCGD-TCL) often presents with generalized skin lesions, preferentially affecting the extremities. The clinical presentation is variable. The neoplasm may be predominantly epidermorphic and present with patches/plaques. Some patients present with deep dermal or subcutaneous tumors with or without epidermal necrosis and ulceration. Dissemination to mucosal or extranodal sites is frequently observed, but involvement of lymph nodes, spleen, or bone marrow is uncommon. B-symptoms such as fever, night sweats, and weight loss are seen in the majority of patients. PCGD-TCL is resistant to multiagent chemotherapy and/or radiation and has a poor prognosis with a median survival of approximately 15 months. Patients with subcutaneous involvement tend to have a more unfavorable prognosis.
Definitive Diagnostic Methods
Genetics testing
Histologic confirmation
Immunophenotyping
Genetics Data
Clonal rearrangement of TRG@ and TRD@ genes
EBV negative
TRB@ may be rearranged or deleted, but not expressed
Immunophenotyping
BetaF1-
CD2+
CD3+
CD4-
CD5-
CD7+/-
CD8-
CD56+
TCR-delta
Treatments
Chemotherapy
Radiation
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 (effective October 1, 2015 U.S. only)
C84.4 Peripheral T-cell lymphoma, not classified
C84.A Cutaneous T-cell lymphoma, unspecified
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None