Search Database ICD-O-3 Code Lists

Name

Fibroblastic reticular cell tumor

ICD-O-3 Morphology

9759/3: Fibroblastic reticular cell tumor
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

See Module 7
Most common sites of involvement: lymph nodes, spleen, soft tissue.

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9755/3).

Fibroblastic reticular cell tumor 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)

Isolated nodal disease is common. Extranodal sites, such as spleen, soft tissue, liver, lung, kidney breast, adrenal gland, and bone may also be involved.

This histology is similar to follicular dendritic cell sarcoma (9758/3) and Interdigitating dendritic cell sarcoma (9757/3) but lacks the immunophenotypic profile of these tumor types.

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

Fibroblastic dendritic cell tumor
Fibroblastic reticular cell sarcoma

Definition

Fibroblastic reticular cell tumour (FRCT) is a neoplasm of putative stromal fibroblastic reticular cell origin. (WHO 5th edition).

Definitive Diagnostic Methods

Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

None

Immunophenotyping

CD68 variably immunoreactive
Cytokeratin (in dendritic pattern) variably immunoreactive
Desmin variably immunoreactive
SMA variably immunoreactive

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

Diarrhea
Dyspnea
Easy bruising or bleeding
Edema
Fever
Generalized papules (single or multiple)
Painful bone lesion
Polydipsia
Polyuria
Skin rash
Weight loss

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

None

Epidemiology and Mortality

Incidence: very rare
Age: median 61 years, range 13-80
Sex: No male or female predilection
Survival: limited data, spontaneous regression to rapid progression has been seen

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: 794-795

International Classification of Diseases for Oncology, Third Edition, Second Revision. Geneva: World Health Organization, 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