Name

Subcutaneous panniculitis-like T-cell lymphoma

ICD-O-3 Morphology

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.

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.

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.

Definitive Diagnostic Methods

Bone marrow biopsy
Histologic confirmation
Immunophenotyping

Genetics Data

EBV sequences negative
TR genes are clonally rearranged

Immunophenotyping

Beta F1 expression
CD8 positive
CD56 negative
CD123 negative
Granzyme B expression
TIA1 expression

Treatments

Chemotherapy
Hormone therapy
Immunotherapy
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

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, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

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