Clinic case:

We present an extraordinary case report from a male diagnosed with Inflammatory Myofibroblastic Tumors (IMT) after suddenly presenting with a haemorrhagic shock due to hematuria. He underwent a partial cystectomy to achieve the complete resection and confirm the pathologic diagnosis. After the complete resection, he is free of relapse.

Relevance:

Our case report shows an extraordinary aggressiveness and a life-threatening disease course. The final pathologic exam confirmed the diagnosis and reported the pathologic correlation with the radiological findings of such an infrequent entity.

Clinical implications:

Although infrequent, IMT might present aggressively. After the initial clinical and radiological suspicion, finding must be confirmed by the complete resection and the pathologic exam.

Conclusion:

The early diagnosis enables the complete resection of the tumor, which is the most determinant prognostic factor. Selected patients with unresectable lesions might also benefit from adjuvant treatment with ALK-inhibitors.