Search Database ICD-O-3 Code Lists

Name

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL)

ICD-O-3 Morphology

9708/3: Subcutaneous panniculitis-like T-cell lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C490-C499
Subcutaneous (soft tissue) lymphoma which often presents as nodules in the trunk and extremities (arms and legs).

Do not assign skin (C44_) as primary site.

Assign C499 if specific primary site cannot be determined.

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) 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)

SPTCL primarily involves subcutaneous tissue, with a predilection for the lower extremities, followed by the upper extremities and trunk.

Do not assign skin as primary site (C44_)

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

None

Definition

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a lymphoproliferative disorder with adipotropism that mostly involves the subcutaneous tissue, but occasionally other sites. It is predominantly composed of activated cytotoxic medium-sized CD8+ lymphocytes expressing the TCRαβ heterodimer. (WHO 5th edition).

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Germline variants of HAVCR2
Epigenetic modifiers, the PI3K/AKT/mTOR pathway, and the JAK/STAT pathway

Immunophenotyping

Beta F1+ (expression/positive)
CD8+ lymphocytes expressing the TCRαβ heterodimer
CD30+ and EBV encoded small RNA (EBER) are negative
Cytotoxic markers expressed (TIA1, perforin, granzyme B)
Granzyme B+ (expression/positive)

Treatments

Chemotherapy
Hormone therapy
Immunotherapy
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)

C84.5 Other and unspecified T-cell lymphomas

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

C86.3 Subcutaneous panniculitis-like T-cell lymphoma (effective October 01, 2015 - September 30, 2024)
C86.30 Subcutaneous panniculitis-like T-cell lymphoma not having achieved remission (effective October 01, 2015)
C86.31 Subcutaneous panniculitis-like T-cell lymphoma, in remission (effective October 01, 2015)

Signs and Symptoms

Autoimmune disease (~20%), commonly systemic lupus erthematosus
Cytopenia
Drenching night sweats
Fatigue
Fever (for no known reason)
Frank hemophagocytic syndrome
Hepatosplenomegaly
Pain in the chest, abdomen, or bones (for no known reason)
Subcutaneous nodules
Weight loss (for no known reason)

Diagnostic Exams

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

Progression and Transformation

None

Epidemiology and Mortality

Incidence: 1.5 cases per 10-million-person years
Age: 35 years median age (broad age range)
Sex: M:F ratio 1.2-3
Survival: 80% overall 5 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: 700-701

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