Search Database ICD-O-3 Code Lists

Name

Subcutaneous panniculitis-like T-cell lymphoma

ICD-O-3 Morphology

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

Reportable

for cases diagnosed 2001 and later

Primary Site(s)

C490-C499
Subcutaneous (soft tissue) lymphoma which often presents as nodules in the trunk and extremities (arms and legs). See Module 7.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) presents with multiple subcutaneous nodules, usually in the absence of other sites of disease.

Skin nodules range in size from 0.5 cm to several centimeters in diameter. Larger nodules may become necrotic, ulceration is rare. The infiltrate involves the fat lobules, usually sparing the septa. The overlying dermis and epidermis are typically uninvolved. Dissemination to lymph nodes and other organs is rare.

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

SPTCL

Definition

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a cytotoxic T-cell lymphoma that preferentially infiltrates subcutaneous tissue. It is composed of atypical lymphoid cells of varying size, typically with prominent apoptotic activity of tumor cells and associated fat necrosis.

Definitive Diagnostic Methods

Bone marrow biopsy
Histologic confirmation
Immunophenotyping

Genetics Data

EBV sequences negative
TR genes are clonally rearranged

Immunophenotyping

Beta F1+ (expression/positive)
CD8+ (expression/positive)
CD56- (no expression/negative)
CD123- (no expression/negative)
Granzyme B+ (expression/positive)
TIA1+ (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-9 Codes

202.8 Other lymphoma

Corresponding ICD-10 Codes

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)

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
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

Age: 35 years median age (broad age range)
Incidence: <1% of all non-Hodgkin lymphomas
Sex: slight female predominance
Survival: 80% overall 5 year survival

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: Mature T- and NK-cell neoplasms
Pages: 383-384

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

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary