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 Abstractor Notes

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
IDC sarcoma
IDCS
Indeterminate cell histiocytosis
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.
Abstractor Notes
Interdigitating dendritic cell sarcoma has shown wide variation. Solitary lymph node involvement is most common, but extranodal presentations, particularly the skin and soft tissue, have been reported. Patients usually present with an asymptomatic mass, although systemic symptoms such as fatigue, fever, and night sweats have been reported. Rarely, there may be generalized lymphadenopathy, splenomegaly, or hepatomegaly. The clinical course is generally aggressive, with about one half of patients dying of their disease. 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. 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. The clinical course has been highly variable, ranging from spontaneous regression to rapid progression.
Definitive Diagnostic Methods
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 (for IDC sarcoma) positive
CD1a-
CD21-
CD23-
CD30-
CD34-
CD35-
Fascin positive
CD163 (for IDC sarcoma) negative
Langerin negative
Myeloperoxidase negative
S100 protein positive
Vimentin (for IDC sarcoma) positive
Treatments
Chemotherapy
Radiation
Surgery
Transformations to
No Transformations
Transformations from
No Transformations
Same Primaries
None
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
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None