Esthesioneuroblastoma metastatic to the trachea

Abstract

Esthesioneuroblastoma is a rare tumour, for which a multimodal approach, including a combination of surgery and radiation, appears to provide the best disease-free and overall survival. Well-known for its tendency for local recurrence and distant spreading by both lymphatic and haematogenous routes, the most common sites of metastases are lungs and bones, followed by liver, spleen, scalp, breast, adrenals and ovary. One single case of metastasis to the trachea has been reported in the literature. The case is reported here of a patient who developed metastatic esthesioneuroblastoma to the trachea 18 months after primary surgery and radiation therapy. The patient was treated by two subsequent N-YAG laser endoscopic resections and chemotherapy.

Keywords: Esthesioneuroblastoma; Malignant tumours; Trachea; Tracheal metastasis.

Fig. 1a

Endoscopic imaging of endotracheal metastasis at first presentation. Two mucous vegetations are evident, causing a 95% reduction of tracheal lumen.

Fig. 1b

Endoscopic imaging after N-Yag laser resection and double stent placement. Patency of tracheal lumen is achieved.

Fig. 2

Post-treatment CT-scan: significant tissue thickening between trachea and oesophagus is evident. Metal stent allows optimal patency of trachea.

Fig. 3

E.E. specimen: in typical neuroendocrine-type esthesioneuroblastoma, low-power microscopy reveals rather monitonous-looking cells arranged in lobules on delicate fibrovascular stroma. Some esthesioneuroblastomas contain true Homer-Wright rosettes and axons may be demonstrated with special stains.