Interdigitating dendritic cell sarcoma

Name
Interdigitating dendritic cell sarcoma
ICD-O-3 Morphology
9757/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
See Module 7
Most common sites of involvement: lymph nodes, skin, soft tissue, other extranodal presentations

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Dendritic cell sarcoma, NOS
Dendritic cell tumor, NOS
IDC sarcoma
IDCS
Indeterminate dendritic cell tumor
Interdigitating cell sarcoma
Definition
IDC sarcoma is a neoplastic proliferation of spindle to ovoid cells with phenotypic features similar to those of interdigitating dendritic cells. Very rare neoplasm with most studies representing single case reports or very small series.

Rare sarcoma arising from the interdigitating reticulum cells in the T-cell area of the lymph node and forming a node-based tumor.

Indeterminate cells are the proposed precursor of Langerhans cells, so this malignancy is in myeloid-derived dendritic cells in contrast to fibroblastic reticular cell tumor (9759/3). These lesions are usually based in the dermis but may extend into the subcutaneous fat. The infiltrate is diffuse, comprising cells resembling Langerhans cells.
Abstractor Notes
Interdigitating dendritic cell sarcoma has shown wide variation.

Visceral organs that are commonly affected include the liver, spleen, kidney and lung. Stage may be an important prognostic factor; however, histological features have not been correlated with clinical outcome.

Indeterminate dendritic cell tumor is a variant of interdigitating dendritic cell sarcoma that should also be coded 9757/3. The clinical course has been highly variable, ranging from spontaneous regression to rapid progression.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Immunoglobulin heavy chain gene and T-cell receptor beta, delta and gamma chain genes are in germline configuration
Immunophenotyping
CD1a (IDC sarcoma) positive
CD1a-
CD21-
CD23-
CD30-
CD34-
CD35-
CD163 (for IDC sarcoma) negative
Fascin (IDC sarcoma) positive
Langerin negative
Myeloperoxidase negative
S100 protein positive
Vimentin (IDC sarcoma) positive
Treatments
Chemotherapy
Radiation
Surgery
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
Corresponding ICD-9 Codes
202.9 Other and unspecified malignant neoplasms
Corresponding ICD-10 Codes
C96.7 Other specified malignant neoplasms of lymphoid, hematopoietic
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C96.4 Sarcoma of dendritic cells (accessory cells)
Signs and Symptoms
Asymptomatic mass
Drenching night sweats
Fatigue
Fever
Generalized lymphadenopathy (rare)
Hepatomegaly (rare)
Splenomegaly (rare)
Diagnostic Exams
Bone scan
BRAF testing
Complete blood count (CBC)
CT (CAT) scan
Endoscopy
Liver function test
Neurological exam
PET (positron emission tomography) scan
Ultrasound exam
Urinalysis
Water deprivation test
Recurrence and Metastases
None
Epidemiology and Mortality
Age: predominantly adults
Incidence: very rare disease
Sex: slight male predominance
Survival: aggressive lymphoma, poor survival, ~1/2 of patients die of disease