INTRACRANIAL HEMANGIOPERICYTOMA
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ALTERNATE NAMES
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Primary Intracranial Hemangiopericytoma; Infantile Intracranial Hemangiopericytoma;
Multifocal Intracranial Hemangiopericytoma; Solitary Fibrous Tumor; SFT; Mesenchymal
Tumor; Non-Meningothelial Tumor
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: Diagnostic testing for HPC includes:
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A biopsy to confirm the diagnosis;
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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
ICD-10:
M85.00
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PROGRESSION
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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.
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TREATMENT
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HPC is treated with surgery, chemotherapy and radiotherapy, depending on the grade,
size, and location of the tumor.
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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 disorder
and laboratory findings are needed to confirm the diagnosis;
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Oncology consultation reports;
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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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13.13 A
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Evaluate under 13.13 A 1 if grade III. Evaluate under 13.13 A 2 if grade II. When
evaluating under 13.13 A 2, the cancer must be progressive or recurrent following
initial antineoplastic therapy.
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113.13
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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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