Name
Follicular dendritic cell sarcoma
ICD-O-3 Morphology
9758/3: Follicular 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 (primarily cervical), tonsil, oral cavity, GI tract, soft tissue, skin, mediastinum, liver, and spleen.
Coding Manual:
Hematopoietic Coding Manual (PDF)
Abstractor Notes
This is an indolent sarcoma, much like a low or intermediate-grade soft tissue sarcoma. Cases showing high-grade features (significant cytologic atypia, extensive coagulative necrosis, and a high proliferative index), large tumor size (greater than 6 cm), or intra-abdominal location can pursue a rapidly fatal course.
Systemic symptoms are uncommon in conventional follicular dendritic cell sarcoma, but are common in the inflammatory pseudo-tumor-like variant.
Most patients are treated by complete surgical excision, with or without adjuvant radiotherapy or chemotherapy.
Systemic symptoms are uncommon in conventional follicular dendritic cell sarcoma, but are common in the inflammatory pseudo-tumor-like variant.
Most patients are treated by complete surgical excision, with or without adjuvant radiotherapy or chemotherapy.
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
Definition
Follicular dendritic cell (FDC) sarcoma is a neoplastic proliferation of spindled to ovoid cells showing morphological and immunophenotypic features of FDCs.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Immunophenotyping
CD3- (no expression/negative)
CD21+ (expression/positive)
CD23+ (expression/positive)
CD34- (no expression/negative)
CD35+ (expression/positive)
CD79a- (no expression/negative)
Clusterin+ (expression/positive)
CXCL13+ (expression/positive)
Desmoplakin+ (expression/positive)
Epidermal growth factor receptor (EGFR)+ (expression/positive)
Fascin+ (expression/positive)
HLA-DR+ (expression/positive)
HMB45- (no expression/negative)
Lysozyme- (no expression/negative)
MPO- (no expression/negative)
Vimentin+ (expression/positive)
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
Drenching night sweats (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
Epidemiology and Mortality
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: 476-479
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Histiocytic and dendritic cell neoplasms
Pages: 476-479
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
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
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