FOLLICULAR DENDRITIC CELL SARCOMA

ALTERNATE NAMES

Follicular Dendritic Cell Tumor/Sarcoma; FDCT/S; Dendritic Cell Sarcoma

DESCRIPTION

Follicular Dendritic Cell Sarcoma (FDCS) is part of a family of extremely rare soft tissue malignant neoplasms that have a significant recurrent and metastatic potential. Although most people with FDCS respond to initial treatment, most have recurrence of the cancer later. Most cases of FDCS develop in the lymph nodes, but approximately 30% of these sarcomas originate in extranodal sites (areas outside of the lymph nodes). Follicular Dendritic Cell Sarcoma that is metastatic or recurrent indicates that the malignant tumor has spread to other parts of the body, and has come back after treatment.

Symptoms vary and are dependent on the part of the body where the tumor develops. The most common symptom is painless swelling in the lymph nodes. Other symptoms may include cough, sore throat, swallowing difficulty, weight loss, and tiredness. Individuals with abdominal FDCS may have pain in the abdominal area.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Diagnostic testing: Diagnosing FDCS is difficult as the tumor closely mimics other tumors and tumor like conditions. Cases may need confirmation of diagnosis by immunohistochemistry. Imaging tests are used to detect recurrence or metastasis.

ICD-9: 202.98

ONSET AND PROGRESSION

Age at onset is variable, with median age of 41 years of age. Current data show overall recurrence and metastatic rates of 43% and 24%, respectively.

TREATMENT

Currently there is no optimal standard treatment for FDCS. Treatment is supportive.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for evaluation: The adjudicator needs medical evidence from treating sources and hospitals. It should include clinical history and examination that describe the diagnostic features of the impairment, biopsies, pathology reports, surgical procedures, imaging tests, pertinent treatment records, and up-to-date progress notes.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

13.04

113.03

Adult FDCS with metastases meets listing 13.04A. Adult recurrent FDCS meets listing 13.04B. Pediatric FDCS meets listing 113.03A, regardless of metastases. Pediatric recurrent FDCS meets listing 113.03B.

Medical Equals