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ICD-O-3 Morphology
9715/3: Anaplastic large cell lymphoma, ALK-negative / Breast implant-associated anaplastic large cell lymphoma
Effective
2021 and later
Reportable
for cases diagnosed
2021 and later
Primary Site(s)
No primary site specified
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.)
ALK- ALCL involved both lymph nodes and extranodal tissue (e.g., the bone, soft tissue, and skin), although extranodal sites are less commonly involved than in ALK+ ALCL.
Most patients present with advanced stage (III-IV) disease, with peripheral and/or abdominal lymphadenopathy and B symptoms.
For breast implant-associated anaplastic large cell lymphomas, the tumor cells may be localized to the seroma cavity or may involve the pericapsular fibrous tissue, sometimes forming a mass. Locoregional lymph nodes may be involved. For this diagnosis, primary site is the breast (C50_)
ALK- ALCL involved both lymph nodes and extranodal tissue (e.g., the bone, soft tissue, and skin), although extranodal sites are less commonly involved than in ALK+ ALCL.
Most patients present with advanced stage (III-IV) disease, with peripheral and/or abdominal lymphadenopathy and B symptoms.
For breast implant-associated anaplastic large cell lymphomas, the tumor cells may be localized to the seroma cavity or may involve the pericapsular fibrous tissue, sometimes forming a mass. Locoregional lymph nodes may be involved. For this diagnosis, primary site is the breast (C50_)
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
ALK-ALCL
Definition
Anaplastic large cell lymphoma, ALK-negative (ALK-) is defined as a CD30+ T-cell neoplasm that is not reproducibly distinguishable on morphological grounds from ALK-positive (ALK+) ALCL, but lacks ALK protein expression.
ALK-ALCL must be distinguished from primary cutaneous ALCL (C-ALCL), other subtypes of CD30+ T-cell or B-cell lymphoma with anaplastic features, and classic Hodgkin lymphoma (CHL).
Breast implant-associated anaplastic large cell lymphoma is a provisional entity that is a T-cell lymphoma with morphological and immunophenotypic features indistinguishable from those of ALK-negative anaplastic large cell lymphoma (ALCL), arising primarily in association with a breast implant.
ALK-ALCL must be distinguished from primary cutaneous ALCL (C-ALCL), other subtypes of CD30+ T-cell or B-cell lymphoma with anaplastic features, and classic Hodgkin lymphoma (CHL).
Breast implant-associated anaplastic large cell lymphoma is a provisional entity that is a T-cell lymphoma with morphological and immunophenotypic features indistinguishable from those of ALK-negative anaplastic large cell lymphoma (ALCL), arising primarily in association with a breast implant.
Definitive Diagnostic Methods
Bone marrow biopsy
Genetic testing
Immunophenotyping
Genetics Data
Immunophenotyping
CD2+ (expression/positive)
CD3+ (expression/positive)
CD4+ (expression/positive)
CD30+ (expression/positive)
C43+ (expression/positive)
Granzyme B+ (expression/positive)
Perforin+ (expression/positive)
TIA1+ (expression/positive)
Treatments
None
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
Corresponding ICD-9 Codes
200.6 Anaplastic large cell lymphoma
Corresponding ICD-10 Codes
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)
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
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
Age: 40-65 median age, can occur at any age
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: Section: Mature T- and NK-cell neoplasms
Pages: 418-422
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Section: Mature T- and NK-cell neoplasms
Pages: 418-422
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
