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Neuroradiology Case of the Week

Case 446

October 2009

Shawn Rosen, Balasubramanya Kolar, MD, and P-L Westesson, MD, PhD, DDS

Clinical Presentation: Patient is a 43-year old male, two years status post-resection of a bi-frontal esthesioneuroblastoma with adjuvant radiotherapy and chemotherapy, now presenting with increasing unsteadiness and nausea. One year ago, he underwent radiotherapy for a lumbosacral leptomeningeal mass.

Imaging Findings: See below.

Diagnosis: Recurrent esthesioneuroblastoma

Discussion: Esthesioneuroblastoma is a rare malignancy arising from the olfactory epithelium. First reported by Berger in 1924, 945 cases have been reported with half in the last decade [1]. The first staging system was proposed by Kadish in 1976 trifurcates disease into three stages: A – confined to the nasal cavity; B – confined to the nasal cavity and one or more paranasal sinuses; and C – extending beyond that in B, including involvement of the orbit, base of skull or intracranial cavity, cervical lymph nodes, or distant metastatic sites. This staging system has been found to be of prognostic significance, with reoccurrence rates of 20%, 56%, and 78% for patients with stage A, B, and C disease, respectively [1]. While the condition can respond favorably to therapy, at least one recent case report described a patient with subdural metastases who deteriorated within two years of the metastases’ identification [1].
     One distinguishing feature of esthesioneuroblastomas are their occasional focal calcifications, which makes CT particularly useful in the presence of a superior nasal mass. Although esthesioneuroblastomas are initially unilateral within the upper nasal cavity, they subsequently expand the nasal cavity and ethmoid air cells bilaterally, before penetrating the orbit and cribriform plate. There is a broad base of bony destruction when the tumor extends into the anterior cranial fossa [2].
     Imaging can be especially important in making the diagnosis as the histopathologic diagnosis can be difficult, mistaking the tumor for other anterior cranial fossa tumors including lymphoma, meningioma, and squamous cell carcinoma [2].

References:

1. Capelle L, Krawitz H. Esthesioneuroblastoma: A case report of diffuse subdural recurrence and review of recently published studies. J Med Imaging Radiat Oncol. 2008 Feb;52(1):85-90. PMID: 18373833 [PubMed]
2. Connor SE, Umaria N, Chavda SV. Imaging of giant tumours involving the anterior skull base. Br J Radiol. 2001 Jul;74(883):662-7. PMID: 11509406 [PubMed]