COMPASSIONATE ALLOWANCE INFORMATION

INTRACRANIAL HEMANGIOPERICYTOMA

ALTERNATE NAMES

Primary Intracranial Hemangiopericytoma; Infantile Intracranial Hemangiopericytoma; Multifocal Intracranial Hemangiopericytoma

DESCRIPTION

Intracranial Hemangiopericytoma (HPC) is a rare, malignant meningothelial tumor with a high proclivity toward recurrence and metastasis. Hemangiopericytomas are tumors of vascular origin, usually occurring in the musculoskeletal system and the skin; intracranial location is uncommon. HPC can occur at any age, but tumors are rare in childhood; and are even rarer in the first year of life (infantile intracranial HPC). Sometimes HPCs are accompanied by paraneoplastic syndrome and hypoglycemia. Clinical presentation relates to CNS tumor mass effect or seizures, intracranial hemorrhage, nausea, headache, projectile vomiting and focal neurological deficit. The World Health Organization classifies HPC as a grade II and grade III cancer.

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

Diagnostic testing: Diagnostic testing for HPC includes a biopsy to confirm the diagnosis, blood tests and diagnostic imaging scans which may include computerized tomography (CT) scan, Magnetic Resonance Imaging (MRI), PET scans, and nucleotide scans.

Physical findings: Physical findings of HPC depend on the site of the tumor and degree of metastases.

ICD-9: 191.X and other codes depending upon site

ONSET AND PROGRESSION

HPC may occur in children or adults. The prognosis is poor if there are metastases to other sites. HPC may metastasize many years after initial onset and requires long-term follow-up.

TREATMENT

HPC is treated with surgery, chemotherapy and radiotherapy, depending on the grade, size and location of the tumor.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for evaluation:

• Clinical history and examination that describes the diagnostic features of the disorder and laboratory findings are needed to confirm the diagnosis.

• Oncology consultation reports.

• Imaging studies.

• Biopsy reports.

• Pathology reports.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

13.13A

113.13

Evaluate under 13.13A1 if grade III. Evaluate under 13.13A2 if grade II. When evaluating under 13.13A2, the cancer must be progressive or recurrent following initial antineoplastic therapy.

Equals Listing

* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.