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Background: Giant intramuscular hemangioma (GIH) is a rare, progressively enlarging benign tumor, characterized by variable presentation and usually initially diagnosed in childhood. Large volume, rapid enlargement and particular radiologic imaging create suspicion of malignancy. Radiologic investigations and needle or small excisional biopsy are not always reliable for an accurate diagnosis; therefore, histology on a large surgical specimen is often requested. The timing and modality, of treatment of these tumors is a matter of debate. Patients and Methods: Data on 3 patients with GIH of the upper trunk and neck are reported. Associated vascular anomalies were found in all patients. All 3 patients had surgery because of the effect of the growth on their function, the severe symptoms and suspicion of malignancy. Results: A one-step excision of an enormous tumor was carried out in one patient, who died from severe postoperative complications. A second patient was successfully treated by a multistep surgical and multidisciplinary approach. An uneventful removal of part of the tumor was performed on the third patient, who is currently in follow-tip for completion of treatment. Conclusion: Surgery remains the most effective mode of treatment for GIH and often results in permanent cure. The authors suggest performing the surgical removal of these tumors at first diagnosis, when their smaller size requires less demanding procedures, presents lower rates of morbidity and offers a better chance of complete excision.

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