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Childhood Gastrointestinal Stromal Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI]

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Incidence

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasms of the gastrointestinal tract in adults.[1] These tumors are rare in children.[2] Approximately 2% of all GIST occur in children and young adults.[3,4,5] In one series, pediatric GIST accounted for 2.5% of all pediatric nonrhabdomyosarcomatous soft tissue sarcomas.[6] Previously, these tumors were diagnosed as leiomyomas, leiomyosarcomas, and leiomyoblastomas.

In pediatric patients, GIST are most commonly located in the stomach and almost exclusively affect adolescent females.[5,7,8]

References:

  1. Corless CL, Fletcher JA, Heinrich MC: Biology of gastrointestinal stromal tumors. J Clin Oncol 22 (18): 3813-25, 2004.
  2. Pappo AS, Janeway K, Laquaglia M, et al.: Special considerations in pediatric gastrointestinal tumors. J Surg Oncol 104 (8): 928-32, 2011.
  3. Prakash S, Sarran L, Socci N, et al.: Gastrointestinal stromal tumors in children and young adults: a clinicopathologic, molecular, and genomic study of 15 cases and review of the literature. J Pediatr Hematol Oncol 27 (4): 179-87, 2005.
  4. Miettinen M, Lasota J, Sobin LH: Gastrointestinal stromal tumors of the stomach in children and young adults: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases with long-term follow-up and review of the literature. Am J Surg Pathol 29 (10): 1373-81, 2005.
  5. Benesch M, Wardelmann E, Ferrari A, et al.: Gastrointestinal stromal tumors (GIST) in children and adolescents: A comprehensive review of the current literature. Pediatr Blood Cancer 53 (7): 1171-9, 2009.
  6. Cypriano MS, Jenkins JJ, Pappo AS, et al.: Pediatric gastrointestinal stromal tumors and leiomyosarcoma. Cancer 101 (1): 39-50, 2004.
  7. Pappo AS, Janeway KA: Pediatric gastrointestinal stromal tumors. Hematol Oncol Clin North Am 23 (1): 15-34, vii, 2009.
  8. Benesch M, Leuschner I, Wardelmann E, et al.: Gastrointestinal stromal tumours in children and young adults: a clinicopathologic series with long-term follow-up from the database of the Cooperative Weichteilsarkom Studiengruppe (CWS). Eur J Cancer 47 (11): 1692-8, 2011.
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