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Monday, August 09, 2004
Inflammatory myofibroblastic tumour (IMT)
Synonyms: plasma cell granuloma, plasma cell pseudotumor, inflammatory myofibrohistiocytic proliferation, omental myxoid hamartoma, inflammatory pseudo tumour
IMT is a distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate, in children and young adults, with mean age of 10 years.
Common sites are the lungs, mesentery, omentum. Constitutinal symptoms is frequent, fever, growth failure, malaise, weight loss, anemia are seen in up to one-third of patients. Imaging findings include lobulated solid mass, inhomogenous, calcification.
Mean diameter is 6 cm with a range of 1 to 17 cm. ICH: positive for vimentin (diffuse cytoplasmic), SMA, MSA, desmin. Focal reactivity for cytokeratin is seen in approximately one- third of cases. Negative for myogenin, myoglobin, S100 protein, CD117 (KIT). In younger cases, ALK (Anaplastic Lymphoma Kinase) 1 rearrangement is frequently detected. Subset of IMT without ALK activation may show the rearrangements of HMGIC (HMGA2) gene.
IMT is a distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate, in children and young adults, with mean age of 10 years.
Common sites are the lungs, mesentery, omentum. Constitutinal symptoms is frequent, fever, growth failure, malaise, weight loss, anemia are seen in up to one-third of patients. Imaging findings include lobulated solid mass, inhomogenous, calcification.
Mean diameter is 6 cm with a range of 1 to 17 cm. ICH: positive for vimentin (diffuse cytoplasmic), SMA, MSA, desmin. Focal reactivity for cytokeratin is seen in approximately one- third of cases. Negative for myogenin, myoglobin, S100 protein, CD117 (KIT). In younger cases, ALK (Anaplastic Lymphoma Kinase) 1 rearrangement is frequently detected. Subset of IMT without ALK activation may show the rearrangements of HMGIC (HMGA2) gene.