Myofibroma, Solitary and Multicentric
Definition
- Generally biphasic tumor composed of mature and immature myofibroblastic cells with hemangiopericytoma-like vessels. May be solitary or multicentric
Alternate / Historical Names
- Congenital fibrosarcoma
- Congential generalized fibromatosis
- Infantile hemangiopericytoma
- Infantile myofibromatosis
- Myofibromatosis
Diagnostic Criteria
- Superficial lesions circumscribed, deep lesions infiltrative
- Both may be microscopically infiltrative
- Generally biphasic nodules (see below for exceptions)
- Light staining bundles of mature nyoid cells usually on periphery of nodules
- Moderate amount of well defined cytoplasm
- Generally vesicular nuclei
- Peripheral chronic inflammation may be present
- Dark staining areas of primitive oval to spindle cells usually in center of nodules
- Scant indistinct cytoplasm
- Light staining bundles of mature nyoid cells usually on periphery of nodules
- Small dark nuclei
- Hemangiopericytoma-like vascular pattern
- Mitotic figures may be frequent
- No atypical figures
- Atypical features are of no clinicall significance
- Intravascular growth frequent
- Primtive spindle cell areas may be very cellular and predominate
- May approach cellularity of fibrosarcoma but lacks atypia
- Mild pleomorphism may be present
- Central necrosis may be present
- Rare in infants
- Both cell types actin positive
- Exceptions to usual pattern
- Occasional domination of either type
- Mature cell type more predominant in lung
- Immature cell type more predominant in bone
- Reversal of zonation occasionally seen in adults
- Infantile lesions show common features with infantile hemangiopericytoma
- We consider them to be the same entity
- Adult lesions have been proposed as part of a spectrum including glomangiopericytoma and myopericytoma
Richard L Kempson MD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Original posting / last update: 8/10/08, 11/23/14