Peripheral T-cell lymphoma, NOS

Name
Peripheral T-cell lymphoma, NOS
ICD-O-2 Morphology
9702/3
Effective 1992 - 2000
ICD-O-3 Morphology
9702/3
Effective 2001 and later
Reportable
for cases diagnosed 1992 and later
Primary Site(s)
See Abstractor Notes and Module 7

Help me code for diagnosis year :

Grade
5 - T-cell
Module Rule
None
Alternate Names
ALCL, ALK-
Anaplastic large cell lymphoma, ALK-negative
Expanded mantle-zone T-cell lymphoma
Follicular T-cell lymphoma, NOS
Intrafollicular T-cell lymphoma
Large cell peripheral T-cell
Lennert lymphoma
Lymphoepithelioid lymphoma
Mature T-cell lymphoma, NOS
Paracortical nodular T-cell lymphoma
Perifollicular T-cell lymphoma, NOS
Peripheral T-cell lymphoma, large cell
Peripheral T-cell lymphoma, NOS
Peripheral T-cell lymphoma, pleomorphic medium and large cell
Peripheral T-cell lymphoma, pleomorphic small cell
PTCL
T-cell lymphoma, NOS
T-immunoblastic lymphoma
T-zone lymphoma
T-zone T-cell lymphoma
Definition
A large group of lymphomas which we collectively refer to as peripheral T-cell lymphomas with the optional addition of "unspecified" to emphasize that these cases do not belong to any better defined entities. Attempts to distinguish between them on morphological basis have met with poor reproducibility.
Abstractor Notes
Peripheral T-cell lymphoma, NOS (PTCL) presents with peripheral lymph node involvement but any site may be affected. Disease is often generalized with infiltrates in the bone marrow, liver, spleen, and extranodal tissue. Peripheral blood is sometimes involved, but leukemic presentation is uncommon. Extranodal presentations may occur with the skin and GI tract representing the most commonly affected areas. Other less frequently involved sites include the lung, salivary gland, and CNS. The diagnosis of PTCL, NOS would be made ONLY when other specific entities have been excluded. Patients most often present with LN enlargement and a majority have advanced disease with B-symptoms. Paraneoplastic features such as eosinophilia, pruritis, or rarely hemophagocytic syndrome may be seen.

There are four variants of PTCL, NOS: Lymphoepitheliod (Lennert lymphoma); follicular T-cell lymphoma, NOS; T-zone lymphoma, and anaplastic large cell lymphoma, ALK-negative.

Lymphoepitheliod (Lennert lymphoma) shows diffuse or more rarely interfollicular growth. There can be scattered Reed-Sternberg-like cells (usually EBV+). In the majority of cases the neoplastic cells are CD8 positive.

Follicular T-cell lymphoma, NOS (perifollicular T-cell lymphoma, intrafollicular T-cell lymphoma, paracortical nodular T-cell lymphoma, expanded Mantle zone T-cell lymphoma) usually consists of atypical clear cells forming intrafollicular aggregates (mimicking follicular lymphoma), small nodular aggregates in a background of progressively-transformed germinal center (mimicking nodular lymphocyte-predominant Hodgkin lymphoma), or enlarged perifollicular zones/nodular aggregates surrounding hyperplastic follicles (mimicking nodal Marginal zone lymphoma). Partial lymph node involvement, lack of enlarged follicular dendritic cells and meshworks and lack of prominent high endothelial venules distinguish it from typical angioimmunoblastic T-cell lymphoma.

T-zone T-cell lymphoma variant is characterized by a perifollicular growth pattern throughout the lymph node. Some studies suggest this variant may have a more indolent course than other PTCL, NOS.

Anaplastic large cell lymphoma, ALK-negative (ALCL-ALK negative involves both lymph nodes and extranodal tissue although the latter sites are less commonly involved than in ALCL-ALK+.) Extranodal sites include bone, soft tissues, and skin.

These are highly aggressive lymphomas with a poor response to therapy. There are frequent relapses and the 5-year overall survival and failure-free survival is 20-30%.
Definitive Diagnostic Methods
Genetic testing
Histological confirmation
Immunophenotype
Genetics Data
Recurrent chromosomal genes 7q, 8q, 17q, and 22q
Recurrent losses in chromosomes 4q, 5q, 6q, 9p, 10q, 12q, and 13q
T-cell receptor (TCR) genes are clonally rearranged
Immunophenotyping
CD3+ (T-zone variant)
CD4+ (NOS and T-zone variant)
CD8+ (lymphoepitheliod variant)
CD8- (NOS)
CD30+
CD30+ (alcl, ALK negative)
CD57- (NOS)
Downregulation CD5
Downregulation CD7
Scattered Reed-Stermberg-like cells usually EBV+ (Lymphoepitheliod variant)
TCR+ (NOS)
Treatments
Chemotherapy
Hormone
Stem cell transplant
Transformations to
No Transformations
Transformations from
No Transformations
Corresponding ICD-9 Codes
202.7 Peripheral T-cell lymphoma
Corresponding ICD-10 Codes
C84.4 Peripheral T-cell lymphoma
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C84.4 Peripheral T-cell lymphoma, not classified
C84.7 Anaplastic large cell lymphoma, ALK-negative
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None