Name

Indeterminate dendritic cell tumor

ICD-O-3 Morphology

9757/3: Interdigitating dendritic cell sarcoma
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

Grade

Not Applicable

Module Rule

None

Alternate Names

Dendritic cell sarcoma, NOS
IDC sarcoma
IDCS
Interdigitating dendritic cell sarcoma
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

Visceral organs 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. 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 expression
CD4 positive
CD21 negative
CD23 negative
CD30 negative
CD35 negative
CD45 positive
CD68 positive
CD163 negative
Ki-67 proliferation index highly variable
Langerin negative
Lysozyme positive
S100 protein expression

Treatments

Chemotherapy
Radiation therapy
Surgery

Transformations to

There are no known transformations

Transformations from

There are no known transformations

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 (U.S. only)

C96.4 Sarcoma of dendritic cells (accessory cells) (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
Generalized lymphadenopathy (rare)

Progression and Transformation

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

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: Histiocytic and dendritic cell neoplasms
Pages: 475-476

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 Langerhans Cell Histiocytosis (LCH)
Pages: https://www.cancer.gov/types/langerhans/hp/langerhans-treatment-pdq#section/_178%20or%20http://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq
Glossary