Fibroblastic reticular cell tumor

Summary

• Fibroblastic reticular cell tumor is a very rare dendritic cell tumor affecting the lymph nodes, spleen, and soft tissues.
• Morphologically it is similar to the interdigitating dendritic cell sarcoma or follicular dendritic cell sarcoma.
• The tumor cells are positive for cytokeratin and cd68 with variable clinical outcome.
• Tumors diagnosed as cytokeratin-positive interstitial reticulum cell tumors, probably constitute the same entity.

Signs and Symptoms

Signs and symptoms are:

• Diarrhea

• Dyspnea

• Easy bruising or bleeding

• Edema

• Fever

• Generalized papules (single or multiple)

• Painful bone lesion

• Polydipsia

• Polyuria

• Skin rash

• Weight loss

Diagnosis

Diagnosis involves many studies including imaging studies, biopsy for histology, flow cytometry, PET scan, USG

• Biopsy

• Blood chemistry studies

• Bone marrow aspiration and bone marrow biopsy

• Bone scan

• BRAF testing

• Complete blood count (CBC)

• CT scan

• Endoscopy

• Flow cytometry

• Immunophenotyping

• Liver function test

• MRI (magnetic resonance imaging)

• Neurological exam

• Positron emission tomography scan

• Physical examination and history (H&P)

• Ultrasound exam

• Urinalysis

• Water deprivation test

Diagnosis

Definitive diagnosis requires histologic confirmation and immunophenotyping

Immunophenotyping

• The immunophenotyping shows positive for CD68, it is variably immunoreactive
• Other immunophenotyping involves desmin and SMA, which are variably immunoreactive

Treatments

• Chemotherapy
• Radiation therapy
• Surgery

ICD codes

• ICD-9 Code: 202.9, other and unspecified malignant neoplasms
• 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)

Epidemiology and Mortality

• Incidence: very rare
• Survival: limited data is available, spontaneous regression to rapid progression has been seen