Gardner's syndrome in a 40-year-old woman: successful treatment of locally aggressive desmoid tumors with cytotoxic chemotherapy

Prabhat K Bhama¹ , Rashmi Chugh² , Laurence H Baker² and Gerard M Doherty¹

¹Department of Surgery, Division of Endocrine Surgery, University of Michigan, Ann Arbor, Michigan, USA

²Department of Internal Medicine, Division of Hematology-Oncology, University of Michigan, Ann Arbor, Michigan, USA

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World Journal of Surgical Oncology 2006, 4:96doi:10.1186/1477-7819-4-96


Published:	17 December 2006

Abstract

Background

Desmoid tumors that present as a part of Gardener's syndrome can present very difficult management problems.

Case presetation

We report a case of intra-abdominal desmoid tumor causing distal small bowel obstruction that complicated the management of a more proximal enterocutaneous fistula from the jejunum. After failure of more conventional management options including imatinib, the patient's disease responded to doxorubicin and ifosfamide. The response resolved the bowel obstruction and allowed small intestinal resection to resolve the enterocutaneous fistula.

Conclusion

Systemic cytotoxic therapy with doxorubicin and ifosfamide can be useful for patients with complications from intra-abdominal desmoid tumor.