Primary cutaneous T-cell lymphoma

Name
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

Help me code for dx year :

Grade
5 - T-cell
Module Rule
None
Alternate Names
CTCL
Cutaneous lymphoma, NOS [OBS]
Cutaneous T-cell lymphoma, NOS
Primary cutaneous CD4 positive small/medium cell 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 is the old code for cutaneous T-cell lymphoma, NOS. This code should rarely be used for cutaneous T-cell lymphoma, NOS; use the code only for HISTORIC CASES, DCO cases, or path-only cases when additional information is not available.

Code 9709/3 is now used for two variants of primary cutaneous T-cell lymphomas; primary or cutaneous CD4-positive small/medium T-cell lymphoma and there is a pre-2010 tumor coded to 9709/3 there should be careful follow-back to determine whether this is a new primary or a more specific diagnosis of T-cell cutaneous lymphoma.

Primary cutaneous CD8-positive aggressive epidermotropic cytoxic T-cell lymphoma presents with generalized skin lesions. This neoplasm is characterized by localized or disseminated eruptive papules, nodules, and tumors showing central ulceration and necrosis or by superficial hyperkeratotic patches and plaques. This neoplasm may disseminate to other visceral sites (lung, testis, CNS, and oral mucosa) but lymph nodes are seldom affected. This lymphoma often has an aggressive clinical course with a median survival of 32 months. There is no difference in survival between cases with a small or large cell morphology.

Primary cutaneous CD4-positive small/medium T-cell lymphoma usually presents with a solitary plaque or nodule, most commonly on the face, neck, or upper trunk. Involvement of lower extremities is rare. By definition, there should be an absence of patches typical of Mycosis fungoides. Patients are generally asymptomatic with the detection of a single skin lesion being the sole manifestation of disease. A minority of patients may present with large tumors or multiple skin lesions. This lymphoma has a rather favorable prognosis with an estimated 5-year survival of approximately 80%. Those patients with a single lesion have a an excellent prognosis and are treated with surgical excision or radiotherapy.
Definitive Diagnostic Methods
Histologic confirmation
Immunophenotype
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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None