Program Operations Manual System (POMS)
TN 35 (08-20)
DI 23022.720 Follicular Dendritic Cell Sarcoma -- metastatic or recurrent
COMPASSIONATE ALLOWANCES INFORMATION
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FOLLICULAR DENDRITIC CELL SARCOMA
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ALTERNATE NAMES
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Follicular Dendritic Cell Tumor/Sarcoma; FDCT/S; Dendritic Cell Sarcoma
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DESCRIPTION
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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, or has
come back after treatment.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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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.
Physical findings: Individuals with FCDS may experience:
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Swelling in the lymph nodes;
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Individuals with abdominal FDCS may have pain in the abdominal area.
ICD-9: 202.98
ICD-10: C96.4
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PROGRESSION
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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.
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TREATMENT
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Currently there is no optimal standard treatment for FDCS. Treatment is supportive.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Biopsy or needle aspiration information found in operative notes, pathology reports,
summaries of hospitalization or other medical reports that include details of the
surgical and pathological findings;
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Treatment records including surgical procedures, and up-to-date progress notes.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.04
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Adult FDCS with metastases meets the criteria in listing 13.04 A. Adult recurrent
FDCS meets the criteria in listing 13.04 B.
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113.03 |
Pediatric FDCS meets the criteria in listing 113.03 A, regardless of metastases. Pediatric
recurrent FDCS meets the criteria in listing 113.03 B.
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Equals
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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