Follicular dendritic cell sarcoma

Name
Follicular dendritic cell sarcoma
ICD-O-3 Morphology
9758/3
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.

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Dendritic reticulum cell sarcoma
FDC
FDC sarcoma
Sarcoma of follicular dendritic cells
Definition
FDC sarcoma is a neoplastic proliferation of spindled to ovoid cells showing morphologic and phenotypic features of follicular dendritic cells.

This is a rare sarcoma arising from the follicular reticulum cells in the B-cell area of the lymph node and forming a node-based tumor.

The inflammatory pseudo-tumor-like variant- originates as tumors in the liver or spleen, with the neoplastic spindled cells dispersed in a lymphoplasmacytic infiltrate.
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.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
Immunoglobulin and T-cell receptor genes in germline configuration
Immunophenotyping
CD1a-
CD3-
CD21+
CD23+
CD30-
CD34-
CD35+
CD79a-
CD163-
Clusterin strongly positive
CNA 42+
Desmoplakin+
Epidermal growth factor receptor +
Fascin+
HLA-DR+
HMB45-
KiM4p+
Lysozyme-
Myeloperoxidase-
Vimentin+
Treatments
Chemotherapy
Radiation
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 neoplasms
Corresponding ICD-10 Codes
C96.7 Other specified malignant neoplasms of lymphoid, hematopoietic
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C96.4 Sarcoma of dendritic cells (accessory cells)
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)
Paraneoplastic pemphigus (rare)
Slow growing painless mass
Diagnostic Exams
BRAF testing
Complete blood count (CBC)
CT (CAT) scan
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Water deprivation test
Progression and Transformation
Local recurrences occur in more than 50% of cases
Metastases occurs in ~25% of cases
Epidemiology and Mortality
Age: 44 years median age (wide age range)
Incidence: rare disease
Sex: no male or female predominance
Survival: 10-20% of patients die from disease after long period of time