Lymph node metastasis in gastrointestinal stromal tumours (GIST) occurs preferentially in young patients ≤40 years: an overview based on our case material and the literature

  • Abbas Agaimy
  • Peter H. Wünsch
Current Concepts in Clinical Surgery



Data on the clinicopathological and biological profiles of gastrointestinal stromal tumours (GISTs) at higher risk for nodal metastasis are lacking.

Materials and methods

We reviewed our own case material and the relevant English literature.


Regional node metastasis was detected in two of 210 GISTs in our case material (1%) corresponding to 6% of 33 patients with nodal dissection (mean, 11 nodes) and 25% of those ≤40 years of age. The two patients had a mean age of 32 years, contrasting with a mean age of 65 years for the whole cohort. One of them had incomplete Carney triad with pulmonary chondromas. The literature disclosed a comparable frequency of lymphatic spread among paediatric patients and young adults (≈20%), irrespective of the features of the Carney triad. Although a wider age range was reported in isolated reports, a majority (66%) of patients with lymph node metastasis were women ≤40 years with gastric epithelioid/mixed type ulcerated endoluminal GIST presenting with GI bleeding.


Contrasting GISTs in elderly patients, lymphatic spread represents a common route of initial spread in patients ≤40 with gastric GISTs, suggesting the need for node sampling in this particular subset of GIST patients. The prognostic significance of nodal metastasis in GIST patients remains to be further clarified.


GIST Paediatric Young adults Lymph nodes Metastasis 


  1. 1.
    Miettinen M, Lasota J (2006) Gastrointestinal stromal tumors: pathology and prognosis at different sites. Semin Diagn Pathol 23:70–83. doi: 10.1053/j.semdp.2006.09.001 PubMedCrossRefGoogle Scholar
  2. 2.
    Lasota J, Miettinen M (2006) KIT and PDGFRA mutations in gastrointestinal stromal tumors (GISTs). Semin Diagn Pathol 23:91–102. doi: 10.1053/j.semdp.2006.08.006 PubMedCrossRefGoogle Scholar
  3. 3.
    Fletcher CDM, Berman JJ, Corless C et al (2002) Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol 33:459–465. doi: 10.1053/hupa.2002.123545 PubMedCrossRefGoogle Scholar
  4. 4.
    Casali PG, Jost L, Reichardt P, Schlemmer M, Blay JY, ESMO Guidelines Working Group (2008) Gastrointestinal stromal tumors: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol 19(Suppl 2):ii35–ii38. doi: 10.1093/annonc/mdn080 PubMedCrossRefGoogle Scholar
  5. 5.
    DeMatteo RP, Lewis JJ, Leung D, Mudan SS, Woodruff JM, Brennan MF (2000) Two hundred gastrointestinal stromal tumors. Recurrence patterns and prognostic factors for survival.. Ann Surg 231:51–58. doi: 10.1097/00000658-200001000-00008 PubMedCrossRefGoogle Scholar
  6. 6.
    Pierie JP, Choudry U, Muzikansky A, Yeap BY, Souba WW, Ott MJ (2001) The effect of surgery and grade on outcome of gastrointestinal stromal tumors. Arch Surg 136:383–389. doi: 10.1001/archsurg.136.4.383 PubMedCrossRefGoogle Scholar
  7. 7.
    Aparicio T, Boige V, Sabourin JC et al (2004) Prognostic factors after surgery of primary resectable gastrointestinal stromal tumours. Eur J Surg Oncol 30:1098–1103. doi: 10.1016/j.ejso.2004.06.016 PubMedCrossRefGoogle Scholar
  8. 8.
    Tashiro T, Hasegawa T, Omatsu M, Sekine S, Shimoda T, Katai H (2005) Gastrointestinal stromal tumour of the stomach showing lymph node metastases. Histopathology 47:438–439. doi: 10.1111/j.1365-2559.2005.02133.x PubMedCrossRefGoogle Scholar
  9. 9.
    Sato T, Kanda T, Nishikura K et al (2007) Two cases of gastrointestinal stromal tumor of the stomach with lymph node metastasis. Hepatogastroenterology 54:1057–1060PubMedGoogle Scholar
  10. 10.
    El Demellawy D, Shokry P, Ing A, Khalifa M (2008) Polypoid gastrointestinal stromal tumor of small bowel metastasizing to mesenteric lymph nodes: a case report. Pathol Res Pract 204:197–201PubMedGoogle Scholar
  11. 11.
    Canda AE, Ozsoy Y, Ak Nalbant O, Sagol O (2008) Gastrointestinal stromal tumor of the stomach with lymph node metastasis. World J Surg Oncol 6:97. doi: 10.1186/1477-7819-6-97 PubMedCrossRefGoogle Scholar
  12. 12.
    Valadão M, de Mello EL, Lourenço L, Vilhena B, Romano S, Castro Ldos S (2008) What is the prognostic significance of metastatic lymph nodes in GIST? Hepatogastroenterology 55:471–474PubMedGoogle Scholar
  13. 13.
    Agaimy A, Pelz AF, Corless CL et al (2007) Epithelioid gastric stromal tumours of the antrum in young females with the Carney triad: a report of three new cases with mutational analysis and comparative genomic hybridization. Oncol Rep 18:9–15PubMedGoogle Scholar
  14. 14.
    Persson S, Kindblom LG, Angervall L, Tisell LE (1992) Metastasizing gastric epithelioid leiomyosarcomas (leiomyoblastomas) in young individuals with long-term survival. Cancer 70:721–732. doi: 10.1002/1097-0142(19920815)70:4<721::AID-CNCR2820700402>3.0.CO;2-G PubMedCrossRefGoogle Scholar
  15. 15.
    Appleman HD, Helwig EB (1976) Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastoma). Cancer 38:708–728. doi: 10.1002/1097-0142(197608)38:2<708::AID-CNCR2820380215>3.0.CO;2-6 PubMedCrossRefGoogle Scholar
  16. 16.
    Carney JA (1999) Gastric stromal sarcoma, pulmonary chondroma and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component and possible familial occurrence. Mayo Clin Proc 74:543–552PubMedCrossRefGoogle Scholar
  17. 17.
    Prakash S, Sarran L, Socci N, DeMatteo RP, Eisenstat J, Greco AM, Maki RG, Wexler LH, LaQuaglia MP, Besmer P, Antonescu CR (2005) 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 27:179–187. doi: 10.1097/01.mph.0000157790.81329.47 PubMedCrossRefGoogle Scholar
  18. 18.
    Miettinen M, Lasota J, Sobin LH (2005) 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 29:1373–1381. doi: 10.1097/01.pas.0000172190.79552.8b PubMedCrossRefGoogle Scholar
  19. 19.
    Price VE, Zielenska M, Chilton-MacNeill S, Smith CR, Pappo AS (2005) Clinical and molecular characteristics of pediatric gastrointestinal stromal tumors (GISTs). Pediatr Blood Cancer 45:20–24. doi: 10.1002/pbc.20377 PubMedCrossRefGoogle Scholar
  20. 20.
    Richter KK, Schmid C, Thompson-Fawcett M, Settmacher U, Altendorf-Hofmann A (2008) Long-term follow-up in 54 surgically treated patients with gastrointestinal stromal tumours. Langenbecks Arch Surg 393:949–955PubMedCrossRefGoogle Scholar
  21. 21.
    Miettinen M, Sobin LH, Lasota J (2005) Gastrointestinal stromal tumors of the stomach. A clinicopathologic, immunohistochemical and molecular genetic study of 1765 cases with long-term follow-up.. Am J Surg Pathol 29:52–68. doi: 10.1097/ PubMedCrossRefGoogle Scholar
  22. 22.
    Antonescu CR, Viale A, Sarran L et al (2004) Gene expression in gastrointestinal stromal tumors is distinguished by KIT genotype and anatomic site. Clin Cancer Res 10:3282–3290. doi: 10.1158/1078-0432.CCR-03-0715 PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  1. 1.Institut für PathologieUniversitätsklinikum ErlangenErlangenGermany
  2. 2.Institut für PathologieKlinikum NürnbergNurembergGermany

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