Search Database ICD-O-3 Code Lists

Name

Follicular dendritic cell sarcoma (FDCS)

ICD-O-3 Morphology

9758/3: Follicular dendritic cell sarcoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes (primarily cervical), tonsil, oral cavity, GI tract, soft tissue, skin, mediastinum, liver, and spleen.

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Follicular dendritic cell sarcoma (FDCS) is part of the Mesenchymal dendritic cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B21)

FDCS occurs in extranodal sites, lymph nodes. Extranodal disease occurs most commonly in the abdomen and upper aerodigestive tract, but it may occur at almost any extranodal site.

EBV+ inflammatory FDCS occurs almost exclusively in the liver or spleen, sometimes with synchronous or metachronous involvement of both organs. It may uncommonly occur in the colon, tonsils, bronchus, pancreas, and mesentery

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

None

Alternate Names

EBV-positive inflammatory follicular dendritic cell
Follicular dendritic cell tumor

Definition

Follicular dendritic cell sarcoma (FDCS) is a malignant neoplasm showing morphological and phenotypic characteristics of follicular dendritic cells (FDCs), which are stroma-derived cells normally found in germinal centres. (WHO 5th edition)

EBV-positive inflammatory follicular dendritic cell sarcoma (FDCS) is an indolent malignant neoplasm characterized by neoplastic follicular dendritic cell (FDC) proliferation, a prominent lymphoplasmacytic infiltrate, and a consistent association with EBV. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Recurrent alterations in NF-κB pathway genes (BIRC3, NFKBIA, TRAF3, SOCS3, CYLD, and TNFAIP3)
IG rearrangement

Immunophenotyping

Expresses antigens CD21, CD23, CD35., clusterin, CXCL13, podoplanin and SSTR2

Treatments

Chemotherapy
Radiation therapy
Surgery

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C96.7 Other specified malignant neoplasms of lymphoid, hematopoietic, and related tissue
C96.9 Malignant neoplasms of lymphoid, hematopoietic, and related tissue, unspecified

Corresponding ICD-10-CM Codes (U.S. only)

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

Signs and Symptoms

Abdominal pain (due to abdominal disease)
Drenching night sweats (inflammatory pseudo-tumor-like variant)
Fatigue (inflammatory pseudo-tumor-like variant)
Fever (inflammatory pseudo-tumor-like variant)
Lymphadenopathy
Paraneoplastic pemphigus (rare)
Slow growing painless mass

Diagnostic Exams

Biopsy
Blood chemistry studies
Bone marrow aspiration and biopsy
Bone scan
BRAF testing
CT (CAT) scan
Endoscopy
Flow cytometry
Immunophenotyping
Liver function test
Neurological exam
PET (positron emission tomography) scan
Ultrasound exam
Urinalysis
Water deprivation test

Progression and Transformation

Local recurrences occur in more than 50% of cases
Metastases occurs in ~25% of cases

Epidemiology and Mortality

Incidence: rare disease
Age: median 50 years, can occur at any age
Sex: no male or female predilection
Survival: low mortality

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Mesenchymal dendritic cell neoplasms
Pages: Part B: 787-793

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Glossary