Lung tumor
Benign tumors
Childhood inflammatory pseudotumor

Authors: Roseann Wu, M.D., M.P.H. (see Authors page)

Revised: 13 May 2016, last major update May 2016

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PubMed Search: Childhood inflammatory pseudotumor

Related topics: Inflammatory pseudotumor
Cite this page: Childhood inflammatory pseudotumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lungtumorinflammatorypseudotumorchild.html. Accessed December 17th, 2017.
Definition / general
  • Most common isolated lung lesion in children, usually asymptomatic
  • Mostly neoplastic with frequent ALK gene rearrangements (in inflammatory myofibroblastic tumor)
  • Benign, although rare cases of malignant behavior have been reported
  • Essential features
  • Inflammatory myofibroblastic tumor (IMT) occurs more often in childhood and in young adults and shows morphology similar to the adult form
  • IMT in children is more often associated with ALK gene rearrangement than in adults
  • Terminology
  • Renamed inflammatory myofibroblastic tumor
  • Epidemiology
  • Most common lung tumor in children age 16 and younger
  • Sites
  • Lung, mediastinum, trachea, bronchus, abdomen, orbit
  • Radiology description
  • Solid masses with homogeneous or heterogeneous enhancement, may contain calcification (Pediatr Radiol 2015;45:1672)
  • Case reports
  • 3 year old child with cough and recurrent respiratory infections (Diagn Pathol 2012;7:83)
  • 5 year old girl with recurrent pyrexia, dry cough and shortness of breath (Interact Cardiovasc Thorac Surg 2010;10:805)
  • Treatment
  • Excision or radiation therapy
  • Gross description
  • Solitary, small peripheral nodules, yellow, firm, covered by intact pleura or polypoid bronchial mass
  • Gross images

    Images hosted on other servers:

    Right lower lobe

    Microscopic (histologic) description
  • Plasma cells (often abundant), lymphocytes, histiocytes and myofibroblasts
  • May have vascular proliferation, collagenous or hyalinized stroma, myxoid change, xanthoma cells, hemosiderin
  • May resemble nodular fasciitis, fibrous histiocytoma or fibromatosis
  • Microscopic (histologic) images

    Images hosted on other servers:

    3 year old child

    Molecular / cytogenetics description
  • More often associated with ALK rearrangement than adult cases