Author(s): Timothy Josef L. Lao, MD and Michael N. Sabalza, MD, FAFN
This is a case of a 47-year-old female presenting with typical Cushingoid appearance and CSF rhinorrhea. MRI revealed a 4.4 cm x 2.9 cm x 4.5 cm enhancing intranasal mass with evidence of erosion of the left cribriform extending to the left anterior cranial fossa. Dexamethasone suppression test yielded elevated cortisol level. Endoscopic nasal biopsy done showed a round cell tumor positive for ACTH, synaptophysin, chromogranin A, and S-100. Patient subsequently underwent endoscopic endonasal excision of left intranasal mass with creation of peri cranial flap for repair of CSF leak. This report is presented to discuss a rare case of ACTH secreting esthesioneurblastoma including its diagnostic challenges and surgical options for repair of anterior cranial fossa defect to address CSF leak particularly by means of a vascularized peri cranial flap.
Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare neoplasm arising from undifferentiated tumors of neuroectodermal origin derived from the olfactory epithelium.1 Since first described by Berger and Luc in 1924, approximately 1000 cases have been reported2 with an estimated incidence of 4 cases per 10 million individuals accounting for approximately 5% of all sinonasal tumors.3 Of these cases, around 9 have been reported to be ACTH producing tumors.4-11
Presented is a local case of this rare tumor presenting with ectopic ACTH production causing Cushing’s syndrome.
Key words: Esthesioneuroblastoma, olfactory neuroblastoma, Cushing’s syndrome, ectopic ACTH
production, CSF leak