Diagnosis and treatment of a rare sinonasal neuroendocrine tumour: adding to the evidence

Abstract

Sinonasal neuroendocrine tumours (NETs) are rare, aggressive neoplasms with a high recurrence potential. There are no robust protocols for the management of these tumours. An 81-year-old man presented with an incidental sinonasal mass visualised on CT scan of head. Over the next few weeks he developed new onset, progressively worsening headache, right eye ptosis and restricted extraocular movements. Imaging confirmed a rapidly enlarging tumour involving the right nasal cavity and the orbit. Biopsy showed a large cell neuroendocrine carcinoma of the right ethmoidal sinus. The patient was treated with concurrent cisplatin chemotherapy and radiation. Repeat imaging showed decrease in tumour volume. The patient continues to do well and follows up regularly with our oncology service. Current protocols comprising chemotherapy after radiation are based on limited studies. A regimen involving concurrent chemoradiation also appears to aid in tumour volume reduction. Additional studies are required to formulate robust clinical protocols for management of sinonasal NETs.

Large Cell Neuroendocrine Carcinoma in the Sinonasal Cavity with Large Intracranial Extension Treated with Endonasal Endoscopic and Transcranial Combined Surgery: A Case Report

“…Including our case, 12 sinonasal LCNECs have been reported (Table 1). 4,[15][16][17][18][19][20] Eight cases were resected surgically. The endoscopic approach was used in two cases (Lahma et al 18) and our case), and the combined endoscopic and transcranial approaches were used only in our case according to our review of published papers.…”

Poorly differentiated orbital neuroendocrine carcinoma

“…While most well-differentiated low-grade NETs (G1 NETs) arise within the gastrointestinal (GI) system and can functionally secrete hormones, neuroendocrine carcinomas originate most frequently as small cell carcinoma of the lung with only <5% arising outside of the pulmonary system 2 3. Primary extrapulmonary NECs have most commonly been reported in the GI or head and neck regions with only exceedingly rare reports of orbital involvement either by distant metastasis or extension from the paranasal sinuses 4–8. The few reports of orbital neuroendocrine carcinomas have shown a wide range of presentations,5 6 8–10 making the initial diagnosis difficult and demanding robust considerations of differentials tailored on a case-by-case basis.…”

“…Primary extrapulmonary NECs have most commonly been reported in the GI or head and neck regions with only exceedingly rare reports of orbital involvement either by distant metastasis or extension from the paranasal sinuses 4–8. The few reports of orbital neuroendocrine carcinomas have shown a wide range of presentations,5 6 8–10 making the initial diagnosis difficult and demanding robust considerations of differentials tailored on a case-by-case basis. In this report, we describe a rare case of primarily orbital presentation of a poorly differentiated extrapulmonary NEC with prominent bilateral extraocular muscle involvement.…”