Primary small cell neuroendocrine carcinoma of the petrous apex: a report of an atypical case

Abstract

Introduction: The head and neck region is a very uncommon location for small cell neuroendocrine carcinomas (SCNEC), which are notoriously aggressive and have a terrible prognosis. We describe the second case of a poorly differentiated SCNEC involving the petrous apex of the temporal bone in the literature and discuss its therapy.
Case Summary: A 42-year-old white male smoker, without personal or familial significant history, presented with right-sided otalgia. A transnasal endoscopic biopsy of the right apical petrous bone was performed and the histopathological analysis was consistent with poorly differentiated SCNEC. The patient received 4 cycles of systemic chemotherapy followed by 30 radiotherapy sessions. Brain magnetic resonance imaging, positron emission tomography scan, and whole-body computed tomography scan images confirmed the absence of metastatic focus.
Discussion: The literature lacks enough documentation of treatment regimens for head and neck SCNECs. The biggest difficulty in our management is that few studies advise combining radiotherapy and chemotherapy without surgery.