Inflammatory myofibroblastic tumor of the paranasal sinuses - A case report

A 4-year-old patient was presented with a left sided nose blockade, rhonchopathy and lymphadenopathy on the left side. A polyposis was seen in the left nasal cavity. Magnetic resonance imaging of the skull showed an expansively growing process of the ethmoid bone with lateral displacement of the medial wall of the maxillary sinus with an intact orbital cavity and skull base. Endoscopic endonasal sinus surgery was performed with subtotal resection of the tumor to obtain a reliable histologic finding. Histological examination revealed a spindle-cell ALK1-positive lesion consistent with an inflammatory myofibroblastic tumor. The endoscopic endonasal tumor resection was performed as a sinus revision surgery on the left side. The tumor was completely resected.

The clinical and magnetic resonance imaging controls showed no evidence of a tumor recurrence over a time period of more than 2 years.

Inflammatory myofibroblastic tumors are soft tissue tumors that can occur in almost all areas of the body, but particularly in the abdomen. The majority of these tumors (57 % ) occurs in children. Histomorphologically they are characterized as spindle cell tumors with varying degrees of pronounced, predominantly plasmacellular inflammatory infiltrates. Despite the generally benign entity - malignant processes are the exception - these tumors should be resected radically. Relapses are also described after R0 resections. Furthermore, in tumors with a size larger than 3cm there is a risk of malignant transformations, so that appropriate follow-up should be performed in the affected patients. It is assumed that inflammation-mediated fibroblast proliferation causes the development of the tumor. However, the exact etiology remains unclear.

Poster-PDF A-1042.pdf

Conflict of interest

Der Erstautor gibt keinen Interessenskonflikt an.

Publication History

Article published online:
13 May 2021