Human Pathology
Volume 39, Issue 8 , Pages 1252-1257 , August 2008

Gastrointestinal stromal tumors of the vermiform appendix: clinicopathologic, immunohistochemical, and molecular study of 2 cases with literature review

  • Abbas Agaimy, MD

      Affiliations

    • Institute of Pathology, Klinikum Nürnberg, 90419 Nürnberg, Germany
    • Corresponding Author InformationCorresponding author. Consultant histopathologist, Institute of Pathology, Klinikum Nürnberg, Prof.-Ernst-Nathan-Strasse 1, 90419 Nürnberg, Germany.
    • ,
  • Antje-Friederike Pelz, PhD

      Affiliations

    • Institute of Human Genetics, University of Magdeburg, 39120 Magdeburg, Germany
    • ,
  • Peter Wieacker, MD

      Affiliations

    • Institute of Human Genetics, University of Magdeburg, 39120 Magdeburg, Germany
    • Institute of Human Genetics, University of Münster, 48149 Münster, Germany
    • ,
  • Albert Roessner, MD

      Affiliations

    • Institute of Pathology, University of Magdeburg, 39120 Magdeburg, Germany.
    • ,
  • Peter H. Wünsch, MD

      Affiliations

    • Institute of Pathology, Klinikum Nürnberg, 90419 Nürnberg, Germany
    • ,
  • Regine Schneider-Stock, PhD

      Affiliations

    • Institute of Pathology, University of Magdeburg, 39120 Magdeburg, Germany.

Received 1 October 2007 ,Revised 9 November 2007 ,Accepted 7 December 2007.

References 

  1. Miettinen M, Lasota J. Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol. 2006;23:70–83
  2. Miettinen M, Sobin LH. Gastrointestinal stromal tumors of the vermiform appendix. Clinicoapthologic, and immunohistochemical study of four cases. Am J Surg Pathol. 2001;25:1433–1437
  3. Yap WM, Tan HW, Goh SGN, Chuah KL. Appendiceal gastrointestinal stromal tumor. Am J Surg Pathol. 2005;29:1545–1547
  4. Schneider-Stock R, Boltze C, Lasota J, et al. High prognostic value of p16/INK4a alterations in gastrointestinal stomal tumors. J Clin Oncol. 2003;21:1688–1697
  5. Agaimy A, Pelz AF, Corless CL, et al. Epithelioid gastric stromal tumours of the antrum in young females with the Carney triad: report of three new cases with mutational analysis and comparative genomic hybridisation. Oncol Rep. 2007;18:9–15
  6. Fletcher CDM, Berman JJ, Corless C, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol. 2002;33:459–465
  7. Agaimy A, Wünsch PH, Sobin LH, Lasota J, Miettinen M. Occurrence of other malignancies in patients with gastrointestinal stromal tumors. Semin Diagn Pathol. 2006;23:120–129
  8. Tarn C, Merkel E, Canutescu A, et al. Analysis of KIT mutations in sporadic and familial gastrointestinal stromal tumors: therapeutic implications through protein modeling. Clin Cancer Res. 2005;11:3668–3677
  9. Heinrich MC, Rubin BP, Longley BJ, Fletcher JA. Biology and genetic aspects of gastrointestinal stromal tumors: KIT activation and cytogenetic alterations. Hum Pathol. 2002;33:484–495
  10. Lasota J, Jasinski M, Sarlomo-Rikala M, Miettinen M. Mutations in exon 11 of c-kit occur preferentially in malignant versus benign gastrointestinal stromal tumors and do not occur in leiomyomas or leiomyosarcomas. Am J Pathol. 1999;154:53–60
  11. Lasota J, Miettinen M. KIT and PDGFRA mutations in gastrointestinal stromal tumors (GISTs). Semin Diagn Pathol. 2006;23:91–102
  12. Corless CL, McGreevey L, Haley A, Town A, Heinrich MC. KIT mutations are common in incidental gastrointestinal stromal tumors one centimeter or less in size. Am J Pathol. 2002;160:1567–1572
  13. Agaimy A, Wünsch PH, Hofstaedter F, et al. Minute gastric sclerosing stromal tumors (GIST tumorlets) are common in adults and frequently show c-KIT mutations. Am J Surg Pathol. 2007;31:113–120
  14. Kawanowa K, Sakuma Y, Sakurai S, et al. High incidence of microscopic gastrointestinal stromal tumors in the stomach. Hum Pathol. 2006;37:1527–1535
  15. Agaimy A, Wünsch PH, Dirnhofer S, Bihl MP, Terracciano LM, Tornillo L. Microscopic gastrointestinal stromal tumors in esophageal and intestinal surgical resection specimens. A clinicopathologic, immunohistochemical, and molecular study of 19 lesions. Am J Surg Pathol. 2007;(in press)
  16. Wardelmann E, Losen I, Hans V, et al. Deletion of Trp-557 and Lys-558 in the juxtamembrane domain of the c-kit protooncogene is associated with metastatic behavior of gastrointestinal stromal tumors. Int J Cancer. 2003;106:887–895
  17. Martin J, Poveda A, Llombart-Bosch A, et al. Deletions affecting codons 557-558 of the c-KIT gene indicate a poor prognosis in patients with completely resected gastrointestinal stromal tumors: a study by the Spanish Group for Sarcoma Research (GEIS). J Clin Oncol. 2005;23:6190–6198
  18. Gunawan B, Heydebreck A, Sander B, et al. An oncogenetic tree model in gastrointestinal stromal tumours (GISTs) identifies different pathways of cytogenetic evolution with prognostic implication. J Pathol. 2007;211:463–470
  19. Wozniak A, Sciot R, Guillou L, et al. Array CGH analysis in primary gastrointestinal stromal tumors: cytogenetic profile correlates with anatomic site and tumor aggressiveness, irrespective of mutational status. Genes Chromosomes Cancer. 2007;46:261–276
  20. Schneider-Stock R, Boltze C, Lasota J, et al. Loss of p16 protein defines high-risk patients with gastrointestinal stromal tumors: a tissue microarray study. Clin Cancer Res. 2005;11:1–8
  21. Günther T, Schneider-Stock R, Häckel C, et al. Telomerase activity and expression of hTRT and hTR in gastrointestinal stromal tumors in comparison with extragastrointestinal sarcomas. Clin Cancer Res. 2000;6:1811–1818

PII: S0046-8177(08)00004-X

doi: 10.1016/j.humpath.2007.12.016

Human Pathology
Volume 39, Issue 8 , Pages 1252-1257 , August 2008

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