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
Cutaneous (skin) lymphoma which presents with generalized skin lesions, mostly affecting the extremities (arms and legs). See Module 7.

Help me code for diagnosis year :

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.)

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
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
Deep dermal subcutaneous tumors with or without necrosis and ulceration
Drenching night sweats
Fatigue
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
CT (CAT) scan
Complete blood count (CBC)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Recurrence and Metastases
None
Epidemiology and Mortality
Age: mostly in adults
Incidence: <1% of cutaneous T-cell lymphomas
Sex: no male or female predominance
Survival: 15 months median survival