Fibroblastic reticular cell tumor

Name
Fibroblastic reticular cell tumor
ICD-O-3 Morphology
9759/3
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

Help me code for diagnosis year :

Grade
9 - Grade/differentiation unknown, not stated, or not applicable
Module Rule
None
Alternate Names
Cytokeratin-positive interstitial reticulum cell tumor
Definition
This tumor is very rare and may also be reported as "cytokeratin-positive interstitial reticulum cell tumor".

The tumor is histologically similar to follicular dendritic cell sarcoma or Interdigitating dendritic cell sarcoma (9757/3), but lacks the immunophenotypic profile of these tumor types. It derives from stroma-derived dendritic cells.
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.
Definitive Diagnostic Methods
Histologic confirmation
Immunophenotyping
Genetics Data
None
Immunophenotyping
CD68
Variable reactivity for smooth muscle actin, desmin, cytokeratin (dendritic pattern)
Treatments
Chemotherapy
Radiation
Surgery
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Same Primaries
There are no known 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
Easy bruising or bleeding
Generalized papules (single or multiple)
Painful bone lesion
Polydipsia
Polyuria
Skin rash
Weight loss
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
None
Epidemiology and Mortality
Incidence: very rare
Survival: limited data, spontaneous regression to rapid progression has been seen