Primary cutaneous T-cell lymphoma

ICD-O-2 Morphology
9709/3
Effective 1992 - 2000
ICD-O-3 Morphology
9709/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632
Cutaneous (skin) lymphoma which presents with generalized skin lesions. See Module 7.

Help me code for diagnosis year :

Grade
5 - T-cell
Module Rule
None
Alternate Names
Cutaneous lymphoma, NOS [OBS]
P-CTCL
Primary cutaneous CD4-positive small/medium T-cell lymphoma
Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma
Definition
Umbrella term for more than 10 discrete T-cell non-Hodgkin lymphomas.

A group of lymphoproliferative disorders characterized by localization of neoplastic T lymphocytes to the skin.
Abstractor Notes
This code should rarely be used for cutaneous T-cell lymphoma, NOS. Diagnosis of a more specific T-cell neoplasm should be possible. Use this NOS term only for HISTORIC CASES, DCO cases, or path-only cases when additional information is not available.

There are two variants for this code:
1. Primary or cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma. Characterized by localized or disseminated eruptive papules, nodules with tumors showing central ulceration and necrosis or by superficial hyperkeratotic patches and plaques. Dissemination to other visceral sites (lung, testis, CNS, and oral mucosa) possible. Lymph nodes seldom affected. Aggressive clinical course with median survival of 32 months.

2. Primary cutaneous CD4-positive small/medium T-cell lymphoma. Clinical presentation is usually a solitary plaque or nodule, commonly on the face, neck, or upper trunk. Involvement of lower extremities is rare. There should be an absence of patches (if patches are seen, see Mycosis Fungoides, 9700/3).

Note: When there is a pre-2010 tumor coded to 9709/3 there should be careful follow-back to determine whether this new diagnosis is a new primary or a more specific diagnosis of T-cell cutaneous lymphoma.

Patients with a single lesion have an excellent prognosis and are treated with surgical excision or radiotherapy.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
T-cell receptor (TCR) genes are clonally rearranged
Immunophenotyping
Beta F1+
CD2-/+
CD3+
CD3- (CD4-positive variant)
CD4+ (CD4-positive variant)
CD4-
CD45RA+/-
CD45RO-
CD5-
CD7+/-
CD8+
CD8- (CD4-positive variant)
Granzyme B+
Perforin+
T1A1+
Treatments
Bone marrow transplant
Chemotherapy
Immunotherapy
Other RX
Radiation
Surgery
Transformations from
None
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.A Cutaneous T-cell lymphoma, unspecified
Signs and Symptoms
Drenching night sweats
Fatigue
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Ulcerated/disseminated eruptive papules, nodules and tumors
Weight loss (for no known reason)
Diagnostic Exams
Bone marrow aspiration and biopsy
CT (CAT) scan
Complete blood count (CBC)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Progression and Transformation
None
Epidemiology and Mortality
Age: mostly occurs in adults
Incidence: <1% of cutaneous T-cell lymphomas
Sex: no male or female predominance
Survival: 32 months median survival