Search Database ICD-O-3 Code Lists

Name

Anaplastic large cell lymphoma, ALK-negative (ALK-ALCL)

ICD-O-3 Morphology

9715/3: Anaplastic large cell lymphoma, ALK-negative / Breast implant-associated anaplastic large cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

No primary site specified
Most common sites are lymph nodes, soft tissue, mediastinum, GI tract.
Common metastatic sites: liver, bone marrow
Spleen involvement is common due to dissemination of disease

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

(This code is effective for cases diagnosed 2021 and later. For cases diagnosed prior to 2021 see code: 9702/3.)

Anaplastic large cell lymphoma, ALK-negative (ALK-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)

ALK-ALCL involves the lymph nodes and extranodal sites. Sites include soft tissue, mediastinum, bone marrow, liver, spleen, gastrointestinal tract.

Most patients present with advanced disease and B symptoms.

BIA-ALCL is mostly restricted to the peri-implant space or as superficial deposits on the luminal side of the peri-implant fibrous capsule. BIA-ALCL can also present as a mass within or beyond the capsule, infiltrating the surrounding soft tissue or breast parenchyma. Regional lymph nodes may be involved.

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

ALK-negative anaplastic large cell lymphoma
Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL)
Seroma-associated anaplastic large cell lymphoma

Definition

ALK-negative anaplastic large cell lymphoma (ALCL) is a mature T-cell lymphoma with uniform strong expression of CD30, without ALK expression or ALK rearrangement. (WHO 5th edition).

Breast implant–associated anaplastic large cell lymphoma (BIA-ALCL) is a mature CD30-positive T-cell lymphoma that arises in relation to a breast implant as an effusion confined by a fibrous capsule, and less commonly forming an invading mass. (WHO 5th edition).

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

DUSP22 locus at 6p25.3
JAK1 mutations
STAT3 mutations
TR genes are clonally rearranged

Immunophenotyping

ALK negative
CD2+ (expression/positive)
CD3+ (expression/positive)
CD4+ (expression/positive)
CD5+ (expression/positive) BIA-ALCL)
CD7+ (expression/positive) (BIA-ALCL)
CD30+ (expression/positive)
C43+ (expression/positive)
Granzyme B+ (expression/positive) (BIA-ALCL)
Perforin+ (expression/positive) (BIA-ALCL)
TIA1+ (expression/positive) (BIA-ALCL)

Treatments

None

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)

C84.5 Other and unspecified T-cell lymphomas

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

C84.7_ Anaplastic large cell lymphoma, ALK-negative (effective October 01, 2015)
C84.7A Anaplastic large cell lymphoma, ALK-negative, breast (effective October 01, 2024)
C84.7B Anaplastic large cell lymphoma, ALK-negative, 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)
Peripheral and abdominal lymphadenopathy
Peri-implant effusion (breast)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Flow cytometry
Immunohistochemistry
Immunophenotyping
Lymph node biopsy
PET (positron emission tomography) scan

Progression and Transformation

None

Epidemiology and Mortality

Incidence: 5.5-15.5% of mature T-cell neoplasms (ALK- ALCL)
Age: median age 54 years, range 18-89 (ALK- ALCL)
Sex: M:F ratio of 1.6-1 (ALK- ALCL)
Survival: 5-year survival of 50% (ALK- ALCL)
Incidence: 1 in 30,000 women with implants (BIA-ALCL)
Age: median age 50, range 21->80 (BIA-ALCL)
Sex: female (BIA-ALCL)
Survival: 5-year survival 84% (BIA-ALCL)

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- and NK-cell neoplasms
Pages: Part B: 734-741

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

PDQ® Adult Treatment Editorial Board. PDQ Peripheral T-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <03/13/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq. Accessed <03/31/2025>. [PMID: 37437079]
Section: Peripheral T-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/peripheral-t-cell-lymphoma-pdq#_2289
Glossary