Search Database ICD-O-3 Code Lists

Name

Fibroblastic reticular cell tumor

ICD-O-3 Morphology

9759/3: Fibroblastic reticular cell tumor
Effective 2010 and later

Reportable

for cases diagnosed 2010 and later

Primary Site(s)

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

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later.) Fibroblastic reticular cell tumor is extremely rare.

Clinical outcome is variable with some patients dying of the disease. The tumor 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.

Grade

Not Applicable

Module Rule

None

Alternate Names

Cytokeratin-positive interstitial reticulum cell tumor
Fibroblastic dendritic cell tumor

Definition

Fibroblastic reticular cell tumor is very rare. Tumors diagnosed as cytokeratin-positive interstitial reticulum cell tumors, probably constitute the same entity.

Definitive Diagnostic Methods

Histologic confirmation
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-9 Codes

202.9 Other and unspecified malignant neoplasm of lymphoid and histiocytic tissue

Corresponding ICD-10 Codes

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
Survival: limited data, spontaneous regression to rapid progression has been seen

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: 479-480

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

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