Alvarado-Cabrero I, García-Robles B, Medrano-Guzmán R, Hernández-Hoyos S, Alderete-Vázquez G

Language: Spanish
References: 24
Page: 135-140
PDF size: 174.41 Kb.

ABSTRACT

Background: Gastrointestinal stromal tumors (GISTs) are uncommon mesenchymal tumors of the gastrointestinal (GI) tract that occur predominantly in adults. GISTs in pediatric patients are rare and not well characterized. We reviewed the presentation, diagnostic work-up, pathological specimens, and outcomes of two children with GIST that originated from the stomach. Literature pertaining to pediatric GISTs was also reviewed.
Clinical cases: Both patients presented with upper GI bleeding from a gastric tumor. The first patient was a 10-year-old male who underwent partial gastrectomy but had recurrence 26 years later requiring surgical exploration due to extensive infiltration into the surrounding organs; the tumor was not resected. The patient is currently being treated with imatinib mesylate. The second patient was a 12-year-old female who had a pedunculated mass originating from the stomach and requiring resection. She subsequently had a local recurrence 2 years later requiring partial gastrectomy. Adjuvant imatinib mesylate was recommended because of the large size of the tumor (8 cm).
Conclusions: Pediatric GISTs represent a distinct subset of sarcomas with a strong predominance for females and gastric location, with 56 cases reported in the English language literature.

REFERENCES

1. Appelman HD. Smooth muscle tumor of the gastrointestinal tract. What we know now that Stout did not know. Am J Surg Pathol 1986;10:83-99.

2. Mazur MT, Clark HB. Gastric stromal tumors: reappraisal of histogenesis. Am J Surg Pathol 1983;7:507-519.

3. Fletcher CD, Berman JJ, Corless C, Gorstein F, Lasota J, Longley BJ, et al. Diagnosis of gastrointestinal stromal tumors: a consensus approach. Hum Pathol 2002;33:459-465.

4. Haider N, Kader M, McDermot M, Devaney D, Corbally MT, Fitzgerald RJ. Gastric stromal tumors in children. Pediatr Blood Cancer 2004;42:186-189.

5. Oguzkurt P, Akcoren Z, Senocak ME, Caglar M, Buyukpamukcu N. A huge gastric stromal tumor in a 13 year old girl. Turk J Pediatr 2002;44:65-68.

6. Egloff A, Lee EY, Dillon JE. Gastrointestinal stromal tumor (GIST) of stomach in a pediatric patient. Pediatr Radiol 2005;35:728-729.

7. Durham MM, Gow KW, Shehata BM, Katzenstein HM, Lorenzo RL, Ricketts RR. Gastrointestinal stromal tumors arising from the stomach: a report of three children. J Peditr Surg 2004;39:1495-1499.

8. Appelman HD, Helwig EB. Sarcomas of the stomach. Am J Clin Pathol 1977;67:2-10.

9. Nowain A, Bhakta H, Pais S, Kanel G, Verma S. Gastrointestinal stromal tumors: clinical profile, pathogenesis, treatment strategies and prognosis. J Gastroenterol Hepatol 2005;20:818-824.

10. Hirota S, Isozaki K, Moriyama Y, Hashimoto K, Nishida T, Ishiguro S, et al. Gain of function mutations of c-Kit in human gastrointestinal stromal tumors. Science 1998;279:577-580.

11. Alvarado-Cabrero I, Vázquez G, Sierra-Santiesteban I, Hernández-Hernández DM, Zavala-Pompa A. Clinicopathologic study of 275 cases of gastrointestinal stromal tumors: the experience at 3 large medical centers in Mexico. Ann Diagn Pathol 2007;11:39-45.

12. Miettinen M, Lasota J. Gastrointestinal stromal tumors. Definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch 2001;438:1-12.

13. Prakash S, Sarran L, Socci N, DeMatteo RP, Eisenstat J, Greco AM, et al. Gastrointestinal stromal tumors in children and young adults. J Pediatr Hematol Oncol 2005;27:179-189.

14. 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 followup and review of the literature. Am J Surg Pathol 2005;29:1373-1381.

15. Price VE, Zielenska M, Chilton-MacNeill S, Smith CHR, Pappo AS. Clinical and molecular characteristics of pediatric gastrointestinal stromal tumors. Pediatr Blood Cancer 2005;45:20-24.

16. Hayashi Y, Okazaki T, Yamataka A, Yanai T, Yamashiro Y, Tsurumaru M, et al. Gastrointestinal stromal tumor in a child and review of the literature. Pediatr Surg Int 2005;21:914-917.

17. Sullivan MJ, McCabe A, Gillet P, Penman ID, MacKinlay GA, Pritchard J. Multiple gastric stromal tumors in a child without syndromic association lacks common Kit or PDGFRá mutations. Pediatr Dev Pathol 2005;8:685-689.

18. Kuroiwa M, Hiwatari M, Hirato J, Suzuki N, Tsuchida Y, Shimada A, et al. Advanced stage gastrointestinal stromal tumor treated with Imatinib in a 12 year old girl with a unique mutation of PDGFRA. J Pediatr Surg 2005;40:1798-1801.

19. Kerr JZ, Hicks MJ, Nuchtern JG, Saldívar V, Heim-Hall J, Shah S, et al. Gastrointestinal autonomic nerve tumors in the pediatric population. A report of four cases and a review of the literature. Cancer 1999;85:220-230.

20. Cipriano MS, Jenkins JJ, Pappo AS, Rao BN, Daw NJ. Pediatric gastrointestinal stromal tumors and leiomyosarcoma. The St. Jude Childrens Research Hospital experience and a review of the literature. Cancer 2004;101:39-50.

21. Heinrich M, Corless CL. Gastric GI stromal tumors (GISTs): the role of surgery in the era of targeted therapy. J Surg Oncol 2005;90:195-207.

22. Papillon E, Rolachon A, Calender A, Chabre O, Barnoud R, Fournet J. A malignant gastrointestinal stromal tumor in a patient with multiple endocrine neoplasia type I. Eur J Gastroenterol Hepatol 2001;13:207-211.

23. Ishida T, Wada I, Horiuchi H. Multiple small intestinal stromal tumors with skenoid fibers in association with neurofibromatosis type I (von Recklinhausens disease). Pathol Int 1996;46:689-695.

24. De Matteo RP, Lewis JJ, Leug D, Mudan SS, Woodruff JM, Brennan MF. Two hundred gastrointestinal stromal tumors. Recurrence patterns and prognostic factors for survival. Ann Surg 2000;231:51-58.