Subcutaneous panniculitis-like T-cell lymphoma

Name
Subcutaneous panniculitis-like T-cell lymphoma
ICD-O-3 Morphology
9708/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C490-C499

Help me code for dx year :

Grade
5 - T-cell
Module Rule
None
Alternate Names
SPTCL
Definition
This is a cytotoxic T-cell lymphoma, which preferentially infiltrates subcutaneous tissue, primarily involving the extremities. It is composed of atypical lymphoid cells of varying size, often with marked tumor necrosis and karyorrhexis. Per "The Lymphomas," typically, biopsies show lymphoid cells mixed with reactive histiocytes.
Abstractor Notes
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) presents with multiple subcutaneous nodules, usually in the absence of other sites of disease. The most common sites of localization are the extremities and the trunk. The nodules range in size from 0.5 cm to several centimeters in diameter. Larger nodules may become necrotic, however 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.

The 5-year survival overall is 80%, but if a hemophagocytic syndrome is present the prognosis is poor.

SPTCL is a rare form of lymphoma, slightly more common in females than males and has a broad age range. Up to 20% may have associated autoimmune disease, most commonly systemic lupus erythematosus.
Definitive Diagnostic Methods
Bone Marrow biopsy
Histological confirmation
Immunophenotyping
Genetics Data
None
Immunophenotyping
BetaRF1 expression
CD3-
CD5-
CD8+
CD30+
CD56-
Express CLA
Expression of T1A1
Expression perforin
Expression T-cell intracellular antigen
Granzyme B
Perforin
T-cell intracellular antigen (TIA1)
Treatments
Chemotherapy
Hormone
Immunotherapy
Radiation
Transformations to
None
Transformations from
None
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 (effective October 1, 2015 U.S. only)
C86.3 Subcutaneous panniculitis-like T-cell lymphoma
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None