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 (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)
Paraneoplastic pemphigus (rare)
Slow growing painless mass
Diagnostic Exams
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