Subcutaneous panniculitis-like T-cell lymphoma

Name
Subcutaneous panniculitis-like T-cell lymphoma
ICD-O-2 Morphology
9708/3
Effective 1995 - 2000
ICD-O-3 Morphology
9708/3
Effective 2001 and later
Reportable
for cases diagnosed 1995 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.

Help me code for diagnosis 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 and trunk. It is composed of atypical lymphoid cells of varying size, often with marked fat 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.

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
None
Immunophenotyping
BetaF1 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
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 (effective October 1, 2015 U.S. only)
C86.3 Subcutaneous panniculitis-like T-cell lymphoma
Signs and Symptoms
Autoimmune disease (~20%), commonly systemic lupus erthematosus
Cytopenia
Drenching night sweats
Elevated liver function tests
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
Bone marrow aspiration and biopsy
CT (CAT) scan
Complete blood count (CBC)
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
MRI (magnetic resonance imaging)
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