Background: High-grade neuroendocrine tumours (HGNETs) of the oesophagus are extremely rare and few cases have been reported worldwide. Here we aim to understand clinical profile, treatment strategy and outcomes for oesophageal HGNETs.
Methods: We report a single-centre retrospective review of 21 patients with oesophageal HGNETs treated between 20112016. Data on incidence, tumour location, stage at presentation, histopathology, treatment protocol and clinical outcomes was collected and analysed.
Results: Among >2000 cases discussed at upper GI multidisciplinary team meetings in Manchester between 20112016, 21 cases of oesophageal HGNET were identified. Median age at presentation was 70 years (range, 4785). Female:male ratio 57.1% (n=12) and 42.9% (n=9) respectively. Histology confirmed small-cell NET in the majority (76.2%, n=16), NOS 19% (n=4) and large cell 4.8% (n=1). At diagnosis (9/21, 42.9%) of patients had distant organ metastasis. The most common site of metastatic disease was the liver.
All but one patient were deemed suitable for active anticancer treatment. Twelve patients (57.1%) were considered candidates for radical treatment. Among them, 9 (75.0%) commenced neo-adjuvant chemotherapy with intent to proceed to chemoradiotherapy (CRT); only 7 (58.3%) received CRT, while 2 (16.7%) progressed during neo-adjuvant chemotherapy. Three patients (25.0%) underwent radical surgery with or without chemotherapy. Of 8 patients not amenable for radical treatment, all received platinum/etoposide doublet chemotherapy: 70% (n=7) received cisplatin and 30% (n=3) carboplatin. Median number of cycles was 4 (range, 1 6). Best response was stable disease in 2 patients (25%) and partial response in 2 patients (25%). Median survival 16.4 months for patients treated with surgery, 14.6 months for patients who followed the radical CRT pathway and 14.1 months for those treated with palliative chemotherapy (P>0.1).
Conclusion: HGNETs of the oesophagus are a rare aggressive malignancy generally resistant to approved treatments for typical oesophageal cancer. Response rate to platinum-based chemotherapy is much lower than with high-grade lung NETs, while long-term disease-free interval after radical surgical resection is a rare phenomenon. In view of their poor prognosis, patients with local or locoregional disease might be better treated conservatively with radical chemoradiotherapy, while newer therapeutic agents should be studied in patients with metastatic disease.
05 Dec 2016
UK and Ireland Neuroendocrine Tumour Society