Abstract
Objective: 1) Present the unique radiology and pathology of a coarsely calcified pediatric esthesioneuroblastoma (ONB) that was incorrectly suggested preoperatively to be a fibro-osseous tumor. 2) Review the clinical presentation, radiographical and pathological findings, treatment, and prognosis of ONBs.
Method: The PubMed database was searched from 1958 to 2011, limited to the English language, using keywords esthesioneuroblastoma, radiology, and calcification.
Results: Esthesioneuroblastomas present with nasal obstruction, pain, hyposmia and epistaxis. Imaging shows a destructive, soft tissue mass without calcifications. Treatment includes surgery, radiation and chemotherapy. Ten year survival is significantly decreased in Hyams high-grade pathological tumors. We present a 12 year-old female with Kadish C and Dulguerov-Calcaterra T3N0M0 esthesioneuroblastoma that on CT and MRI revealed a coarsely calcified 3.8 × 3.5 × 4.1 cm mass involving the right nasal vault and paranasal sinuses. Due to the extensive calcifications, it was incorrectly suggested preoperatively to be a fibro-osseous lesion. She underwent endoscopic-assisted resection with postoperative radiation and is disease-free 9 months post-treatment.
Conclusion: Our case highlights a unique radiographic presentation of esthesioneuroblastoma and emphasizes that this uncommon neuroectodermal malignant tumor always be in the differential for unilateral nasal cavity lesions. Optimal treatment requires a multidisciplinary approach and long-term clinical surveillance is essential.
Get full access to this article
View all access options for this article.