Case report: A rare solid mesenchymal tumor of the paranasal sinuses in a patient with chronic sinusitis and prior radiation therapy

Introduction:

A 38 year old female patient presented with the clinical picture of a chronic sinusitis. In the patient history was a radiation therapy of a Hemangioma of the left medial canthus during the infancy to be mentioned. The patient was finally diagnosed with a rare tumor of the right frontal sinus, which is presented in this article.

Case Presentation:

The patient was admitted to us after prior sinus surgery with persistent symptoms of chronic sinusitis and an unclear histological image. The clinical examination showed normal postoperative findings and did not detect any abnormalities. The CT imaging revealed a tumor with smooth rounded borders and no bone erosion in the right frontal sinus. Endonasal endoskopic revision surgery of the right frontal sinus in the sense of a Draf IIb operation was performed, the tumor was completely excised.

A solid mesenchymal tumor, partially with a chondroid extracellular matrix, was histologically confirmed. The tumor was negative for Pan-Cytokeratin, CK18, S100, Desmin, CD31, CD34, nuclear negative for Beta-Catenin. The tumor cells were strongly positive for smooth muscle actin, about 1% of the cells were tested positiv for the proliferation marker Ki67. A translocation in the EGFR1-Gen was not detected. After further histological testing a chondromyxoid fibroma was finally diagnosed.

Discussion:

Chondromyxoid fibroma is a rare benign primary tumor, that affects mostly the long bones. Because of its complexity and rarity it remains a challenge for the clinical and histological diagnosis.

Publication History

Publication Date:
18 April 2018 (online)