Inflammatory myofibroblastic tumor of the central nervous system and its relationship to inflammatory pseudotumor

摘要: Inflammatory myofibroblastic tumor (IMT) is a distinctive spindle cell lesion and occurs primarily in soft tissue. Recent evidence suggests neoplastic nature, although historically, both nonneoplastic processes were combined this category. Originally described as process, the term inflammatory pseudotumor (IP) has been used synonymously with IMT. IMTs have linked to ALK gene (2p23) rearrangements, some suggested an association human herpesvirus 8 (HHV-8). IMT central nervous system (CNS) rare, its characteristics are poorly defined, relation similar tumors at other sites unclear. To better characterize within CNS, we studied clinicopathologic features of 6 compared them 18 lesions originally classified IP. The group consisted 2 male 4 female patients median age 29 years. Of six IMTs, 5 occurred cerebral hemispheres, one was posterior fossa. All composed cells variable amount infiltrate. Eighteen IPs included study predominantly masses occasionally seen setting systemic diseases. Only 1 none recurred during follow-up period. Four had either protein overexpression or 2p23 rearrangement, case demonstrated both. None positive for ALK. Neither nor IP cases HHV-8 expression. We suggest that CNS distinct from IP, distinguishing should enable decisions patient management.