Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2011  |  Volume : 16  |  Issue : 2  |  Page : 78--79

Unresectable gastrointestinal stromal tumors


Prema Menon1, Deepak Bansal2, KLN Rao1,  
1 Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012, India
2 Department of Pediatric, Hematology/Oncology unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012, India

Correspondence Address:
Prema Menon
Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
India




How to cite this article:
Menon P, Bansal D, Rao K. Unresectable gastrointestinal stromal tumors.J Indian Assoc Pediatr Surg 2011;16:78-79


How to cite this URL:
Menon P, Bansal D, Rao K. Unresectable gastrointestinal stromal tumors. J Indian Assoc Pediatr Surg [serial online] 2011 [cited 2021 Nov 27 ];16:78-79
Available from: https://www.jiaps.com/text.asp?2011/16/2/78/78139


Full Text

Sir,

We read with interest the recent article on "Gastrointestinal stromal tumors" published in the October issue of this Journal. [1] Dr. Bhatnagar was able to resect the tumor completely in her patient. We would like to supplement the information regarding the usefulness of immunotherapy through 'imatinib mesylate' in advanced and unresectable stromal tumors in the pediatric age group.

A 12-year-old boy was admitted in October 2006 with us for abdominal pain of 10 months and swelling in the right side of abdomen of three months duration, associated with occasional nonbilious vomiting. The abdominal examination revealed a 10 Χ 10 cm size, hard, immobile, nontender lump in the right lower quadrant of the abdomen. At laparotomy, it was a large retroperitoneal mass infiltrating the duodenum, terminal ileum and cecum. The mid-ureter and the iliac vessels were engulfed by the tumor. No distinct dissection planes could be found to resect the tumor completely. Only debulking of the tumor was surgically feasible with ileocecal resection. There was gross residual tumor left at the end of surgery. The mid-ureter had to be resected with an end-to-end anastomosis and stenting.

The histopathology revealed malignant gastrointestinal stromal tumor (GIST) and the C-kit was focally positive. Three months later, the ureteral stent was removed cystoscopically. The child was initiated on imatinib mesylate therapy with a dose of 400 mg once daily and was periodically followed up. An ultrasonography and computed tomogram (CT) scan of abdomen were performed in August 2010 (four years after surgery) and no residual tumor could be detected. The left over tumor after debulking, four years earlier, has completely disappeared.

There is no consensus on the duration of immunotherapy with imatinib. In our patient, we plan to continue the drug for at least five years (Professor Jean-Yves Blay, France 2009, personal communication) and continue life-long follow up (Professor Martin Benesch, Austria, 2009, personal communication). In a large (n>700) randomized, double-blind, placebo-controlled, multinational trial [ACOSOG (American College of Surgeons Oncology Group) Z9001], patients who received one year of adjuvant treatment with oral imatinib, 400 mg/day, after surgical resection of GIST had significantly longer recurrence-free survival than placebo recipients. [2] In an open-label national multicenter phase 3 study in France, patients with GIST, free of progression after three years of imatinib were randomly assigned to continue or interrupt imatinib. Imatinib interruption after three years in responders resulted in a high risk of rapid progression in patients with advanced GIST. Discontinuation of imatinib is not recommended outside clinical trials unless patients experience significant toxic effects. [3] The optimum duration of adjuvant treatment, safety and efficacy is currently under investigation with two ongoing randomized control trials and two nonrandomized studies. [4]

Thus in the index child, the grossly incompletely resected gastrointestinal stromal tumor (malignant) has completely regressed with immunotherapy using imatinib. In such advanced tumors also, the therapy was useful.

References

1Bhatnagar SN. Gastrointestinal stromal tumors: Role of surgery and immunotherapy. J Indian Assoc Pediatr Surg 2010;15:148-50.
2Dematteo RP, Ballman KV, Antonescu CR, Maki RG, Pisters PW, Demetri GD, et al. American College of Surgeons Oncology Group (ACOSOG) Intergroup Adjuvant GIST Study Team: Adjuvant imatinib mesylate after resection of localised, primary gastrointestinal stromal tumour: A randomised, double-blind, placebo-controlled trial. Lancet 2009;373:1097-104.
3Le Cesne A, Ray-Coquard I, Bui BN, Adenis A, Rios M, Bertucci F, et al. Discontinuation of imatinib in patients with advanced gastrointestinal stromal tumours after 3 years of treatment: An open-label multicentre randomised phase 3 trial. Lancet Oncol 2010;11:942-9.
4Essat M, Cooper K. Imatinib as adjuvant therapy for gastrointestinal stromal tumours - A systematic review. Int J Cancer 2011;128:2202-14.