Search Database ICD-O-3 Code Lists

Name

Primary cutaneous anaplastic large cell lymphoma (C-ALCL)

ICD-O-3 Morphology

9718/3: Primary cutaneous CD30-positive T-cell lymphoproliferative disorders
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C440-C449 , C510-C512 , C518-C519 , C600-C602 , C608-C609 , C632
Cutaneous (skin) lymphoma which presents with lesions on the skin of the trunk, face, extremities, and buttocks.

Assign C449 if specific primary site cannot be determined.

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is part of the Mature T-cell and NK-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B20)

C-ALCL is a primary cutaneous disease that can involve the skin in any location. The disease may have primary mucosal involvement or, rarely, can concurrently involve mucosal and cutaneous sites.

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.

Module Rule

None

Alternate Names

Primary cutaneous CD30 positive large T-cell lymphoma [OBS]
Primary mucosal CD30 positive large T-cell lymphoma
Ocular adnexal T-cell lymphoma

Definition

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) belongs to the spectrum of primary cutaneous CD30-positive T-cell lymphoproliferative disorders. C-ALCL is composed of large cells with an anaplastic, pleomorphic, or immunoblastic cytomorphology; > 75% of the tumour cells express CD30. (WHO 5th edition)

Definitive Diagnostic Methods

Genetic testing
Histologic confirmation
Immunophenotyping

Genetics Data

ALK gene at 2p23
t(2;5)(p23;q35)/NPM1::ALK and with other ALK fusion partners

Immunophenotyping

CD2 variable loss
CD3 variable loss
CD4+ (expression/positive) T-cell phenotype
CD5 variable loss
CD15+ (expression/positive)
CD30+ (expression/positive)
Granzyme B+ (expression/positive)
Perforin+ (expression/positive)
TIA1+ (expression/positive)

Treatments

Chemotherapy
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C84.4 Peripheral T-cell lymphoma

Corresponding ICD-10-CM Codes (U.S. only)

C86.6 Primary cutaneous CD30-positive T-cell proliferations (effective October 01, 2015 - September 30, 2024)
C86.60 Primary cutaneous CD30-positive T-cell proliferations not having achieved remission (effective October 01, 2024)
C86.61 Primary cutaneous CD30-positive T-cell proliferations in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Solitary or localized nodules or tumors
Ulcerated skin lesions
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
PET (positron emission tomography) scan
Skin biopsy

Progression and Transformation

Multifocal lesions are seen in about 20% of cases. These lymphomas frequently relapse in the skin.

Epidemiology and Mortality

Incidence: second most common type of cutaneous T-cell lymphoma
Age: 60 years median age (can occur in children, although rare)
Sex: M:F ratio is 2-3.1
Survival: 90% 10 year survival

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Mature T-cell and NK-cell neoplasms
Pages: Part B: 698-699

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 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

PDQ® Adult Treatment Editorial Board. PDQ Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment. Bethesda, MD: National Cancer Institute. Updated <02/19/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq. Accessed <03/31/2025>. [PMID: 2638928
Section: Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq
Glossary