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Diseases

Genetic and Rare Diseases Information Center (GARD)

Inflammatory myofibroblastic tumor


Other Names for this Disease
  • Inflammatory pseudotumor
  • Inflammatory fibrosarcoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

An inflammatory myofibroblastic tumor (IMT) is an uncommon, presumably benign (non-cancerous) tumor made up of cells called myofibroblastic spindle cells. It usually develops in children or young adults, but can affect people of any age. An IMT can occur in almost any part of the body but is most commonly found in the lung, orbit (eye socket), peritoneum (lining of the abdominal cavity and internal organs), and mesentery. Signs and symptoms vary depending on the site of the tumor. Some people with an IMT are asymptomatic, while others may have nonspecific respiratory symptoms, fever, or pain. IMTs may recur, and occasionally become locally invasive and/or spread (metastasize) to other parts of the body. The underlying cause of IMTs is poorly understood.[1][2][3][4] Some cases have been linked to translocations involving the ALK gene.[5] Treatment involves surgical removal when possible, although there are reports of treatment with oral steroids and radiation therapy.[1][2][3][4]
Last updated: 2/8/2016

References

  1. Coffin CM, Hornick JL, Fletcher CD. Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. American Journal of Surgical Pathology. April 2007; 31(4):509-520. http://www.ncbi.nlm.nih.gov/pubmed/17414097.
  2. Jindal A, Bal A, Agarwal R. Inflammatory myofibroblastic tumor of the trachea in the pediatric age group: case report and systematic review of the literature. Journal of Bronchology and Interventional Pulmonology. January, 2015; 22(1):58-65. http://www.ncbi.nlm.nih.gov/pubmed/?term=25590486.
  3. Chun YS, Wang L, Nascimento AG, Moir CR, Rodeberg DA. Pediatric inflammatory myofibroblastic tumor: anaplastic lymphoma kinase (ALK) expression and prognosis. Pediatr Blood Cancer. November, 2005; 45(6):796-801. http://www.ncbi.nlm.nih.gov/pubmed/?term=15602716.
  4. Ales Tomazic, Diana Gvardijancic, Joze Maucec, and Matjaz Homan. Inflammatory myofibroblastic tumor of the pancreatic head – a case report of a 6 months old child and review of the literature. Radiol Oncol. September, 2015; 49(3):265-270. http://www.ncbi.nlm.nih.gov/pubmed/?term=26401132.
  5. ALK. Genetics Home Reference (GHR). March 2011; http://ghr.nlm.nih.gov/gene/ALK.
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In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Inflammatory myofibroblastic tumor. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Inflammatory pseudotumor
  • Inflammatory fibrosarcoma
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.