Priya Sarv,  Watal Pankaj,  Sato T Shawn,  Sato Yutaka

Final Pr. ID: Poster #: EDU-067

The prevalence of fibrosing diseases is uncommon in adult patients, and significantly more rare in the pediatric population. The spectrum of fibrosing diseases may be subdivided into two sub-categories: Inflammatory pseudotumors (IMT) and multifocal fibrosclerotic diseases. IMT has a predilection for visceral soft tissues and the most common sites of involvement include lung, abdominopelvic region, but virtually any site may be involved, including the somatic soft tissues, bone, larynx, uterus and CNS. Multifocal fibrosclerotic diseases encompasses retroperitoneal fibrosis, mediastinal fibrosis, reidel’s thyroiditis, orbital pseudotumor, and sclerosing cholangitis to name a few. IMT’s are predominantly neoplastic but may be post-traumatic or post-infectious. Fibrosclerosing diseases may be associated with inflammatory diseases (inflammatory bowel disease), autoimmune conditions (juvenile rheumatoid arthritis, systemic lupus erythematosus), malignant tumors (lymphoma), vasculitis and may arise secondary to drugs, toxins, trauma or radiation. Some may be idiopathic with no underlying cause.
The clinical presentation can be quite variable and often depends upon the site of involvement as well which adjacent structures are affected by the fibrosis.
Initial diagnosis can be suggested by imaging, but imaging findings are often non-specific. They can appear as mass forming and may be mistaken for more aggressive malignancy. Tissue is often needed for confirmation. Histopathology shows evidence of lymphocytic infiltration, activated fibroblasts, spindle shaped cells and granulation tissue.
The key issue for the pediatric radiologist is to be aware of these rare conditions and thus include them in their differential diagnosis. This diagnosis should be considered to avoid over aggressive biopsy, operation and chemotherapy. In addition, it may warrant work up for other associated fibrosing diseases in the appropriate clinical scenario.
In this presentation, we will provide a case based review of the features of pediatric inflammatory pseudotumors and fibrosclerotic diseases including myofibroblastic tumors, fibromatosis, fibrosarcomas, nodular and cranial fascitis as well as fibrotic conditions involving mediastinum, retroperitoneum, biliary tract and thyroid gland with their appropriate diagnostic work-up.
Our aim is to make people aware of these rare presentations, so that they are not lost in the long differential! Read More

Authors:  Priya Sarv , Watal Pankaj , Sato T Shawn , Sato Yutaka

Keywords:  Fibrosclerosis, inflammatory pseudotumors