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Extrapulmonary high-grade neuroendocrine cancers (NEC) are treated like small-cell lung cancer, with case series indicating similar responses to systemic therapy. Clinical trials have not addressed optimal treatment for this aggressive disease.
Investigators from Japan now report results of the phase 3, randomized TOPIC-NEC trial comparing chemotherapy with etoposide plus cisplatin (EP) versus irinotecan plus cisplatin (IP) in patients with advanced high-grade NEC of the gastrointestinal tract. Of 170 patients, 69% were men, the most common primary sites were hepatobiliary (41%) and stomach (32%), 94% had metastatic or recurrent disease, 46% had large-cell cancers and 39% had small-cell cancers. Only 2.9% of patients had grade 3 neuroendocri…