Tibial Dysplasia - Neurofibromatosis Type 1
Joseph Junewick, MD FACR
over 3 years ago
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Joseph Junewick, MD FACR
|Diagnostic Category: Neoplasia Benign
|Created: over 6 years ago
|Updated: over 6 years ago
4 year old female with 1 week of abdominal pain and fever.
US – Complex hyperemic right lower quadrant mass separate from the ovaries.
CT – Axial and coronal images demonstrate an enhancing solid mass with cystic components with apparent mural involvement of the urinary bladder. The appendix was not identified.
Inflammatory myofibroblastoma is an unusual, benign, chronic inflammatory lesion of unclear pathogenesis. It most frequently occurs in the lung and orbit, but it can also originate in many anatomic locations including the mesentery. Inflammatory myofibroblastoma may be a sequela of occult infection, minor trauma, or prior surgery. Inflammatory myofibroblasta has been referred to in the literature as inflammatory pseudotumor, inflammatory myofibroblastic pseudotumor, extrapulmonary inflammatory pseudotumor, plasma cell granuloma, plasma cell pseudotumor, and inflammatory fibrosarcoma. Inflammatory myofibroblastoma most commonly occurs in the pediatric or young adult population. Patients come to medical attention with complaints of fever, malaise, weight loss, or abdominal pain.
The imaging characteristics of inflammatory myofibroblastoma are nonspecific. On US, it appears as a well-defined or infiltrating, solid, mixed-echotexture mass within the mesentery. Hyperemia may be evident on Doppler ultrasound. On CT scans, the mass is typically heterogeneous in attenuation. Involvement of bowel or adjacent viscera is rare. The majority of lesions have been described as having well-defined margins, but can be infiltrative. Enhancement patterns are variable. Larger lesions may have central areas of hypoattenuation that are suggestive of necrosis and calcifications may also be present.
The differential diagnosis of inflammatory myofibroblastoma includes abscess, benign fibrous mesenteric tumors, lymphoma, metastatic disease, and soft-tissue sarcomas. Surgical resection is the treatment of choice and is curative in the majority of patients
Levy AD, Rimola J, Mehrotra AK, Sobin LH. Benign Fibrous Tumors and Tumorlike Lesions of the Mesentery: Radiologic-Pathologic Correlation. Radiographics (2006); 26:245-264.
Jessica Warners, RDMS