ICD-O-2 Morphology

Effective 1992 - 2000

ICD-O-3 Morphology

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.

Grade

Not Applicable

Module Rule

None

Alternate Names

Cutaneous lymphoma, NOS [OBS]
P-CTCL
Primary cutaneous acral CD8+ T-cell lymphoma
Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma

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.

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.

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

EBV negative
TR genes are clonally rearranged

Immunophenotyping

BCL6 negative
Beta F1 expression
CD2 positive
CD3 expression
CD4 negative
CD5 positive
CD7 positive
CD8 positive
CD10 negative
CD30 negative
CD56 negative
CXCL13 negative
CD57 negative
Granzyme B+
PDI negative
Perforin+
TdT negative
T1A1 expression

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Immunotherapy
Other therapy
Radiation therapy
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 (U.S. only)

C84.A Cutaneous T-cell lymphoma, unspecified (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
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

Progression and Transformation

None

Epidemiology and Mortality

Age: mostly occurs in adults
Sex: no male or female predominance

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

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary