Name
Primary cutaneous T-cell lymphoma
ICD-O-2 Morphology
9709/3: Cutaneous T-cell lymphoma, NOS
Effective
1992 - 2000
ICD-O-3 Morphology
9709/3: Cutaneous T-cell lymphoma, NOS
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.
Coding Manual:
Hematopoietic Coding Manual (PDF)
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.
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.
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.
Diagnostic Confirmation
This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.
Grade
Not Applicable
Module Rule
None
Alternate Names
Cutaneous T-cell lymphoma, NOS
P-CTCL
Primary cutaneous acral CD8+ T-cell lymphoma
Primary cutaneous CD8+ T-cell lymphoma
Primary cutaneous T-cell lymphoma, NOS
Definition
Primary cutaneous CD8+ T-cell lymphoma is a rare cutaneous tumor characterized by skin infiltration of clonal atypical medium-sized cytotoxic lymphocytes,. The tumor is clinically characterized by preferential involvement of acral sites (in particular the ear).
Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma is a cutaneous T-cell lymphoma characterized by proliferation of epidermotropic CD8+ cytotoxic T cells and aggressive clinical behavior. Differentiation from other types of cutaneous T-cell lymphomas with a CD8+ cytotoxic T-cell phenotype is based on the clinical presentation, clinical behavior, and certain histological features, such as marked epidermotropism with epidermal necrosis.
Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma is a cutaneous T-cell lymphoma characterized by proliferation of epidermotropic CD8+ cytotoxic T cells and aggressive clinical behavior. Differentiation from other types of cutaneous T-cell lymphomas with a CD8+ cytotoxic T-cell phenotype is based on the clinical presentation, clinical behavior, and certain histological features, such as marked epidermotropism with epidermal necrosis.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
EBV negative
TR genes are clonally rearranged
Immunophenotyping
BCL6- (no expression/negative)
Beta F1+ (expression/positive)
CD2+ (expression/positive)
CD3+ (expression/positive)
CD4- (no expression/negative)
CD5+ (expression/positive)
CD7+ (expression/positive)
CD8+ (expression/positive)
CD10- (no expression/negative)
CD30- (no expression/negative)
CD56- (no expression/negative)
CXCL13- (no expression/negative)
CD57 - (no expression/negative)
Granzyme B+ (expression/positive)
PDI- (no expression/negative)
Perforin+ (expression/positive)
TdT- (no expression/negative)
T1A1+ (expression/positive)
Treatments
Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Immunotherapy
Other therapy
Radiation therapy
Surgery
Transformations to
Transformations from
None
Same Primaries
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 (U.S. only)
C84.A Cutaneous T-cell lymphoma, unspecified (effective October 01, 2015)
Signs and Symptoms
Diagnostic Exams
Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Flow cytometry
Immunohistochemistry
Immunophenotyping
PET (positron emission tomography) scan
Skin biopsy
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
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: 399-401
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 399-401
International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq