Department of Medical Oncology, University of Turin, San Luigi Gonzaga Hospital, Orbassano, Turin, Italy.
Department of Medical Oncology, University of Turin, Ordine Mauriziano Hospital, Turin, Italy.
Endokrynol Pol. 2019;70(4):380-383. doi: 10.5603/EP.a2018.0076. Epub 2018 Oct 25.
Neuroendocrine neoplasms (NENs) are a rare and heterogeneous group of tumours, with a variety of primary origins and variable aggressiveness. NENs with an atypical primary origin, such as breast and retroperitoneal NENs, are extremely rare. As a consequence, an established diagnostic and therapeutic strategy in this particular subgroup is lacking. The combination of capecitabine and temozolomide, called CAPTEM regimen, has produced promising response rates in patients with grade 1 or 2 neuroendocrine tumours of multiple origins.
The first is a case of a 68-year-old woman with a metastatic primary breast neuroendocrine tumour, treated with cisplatin plus etoposide as first line, followed by CAV scheme (cyclophosphamide, doxorubicin, and vincristine), and subsequently treated, in third line with the CAPTEM regimen, obtaining radiological response and good tolerance. The second is the case of a 66-year-old woman affected by a metastatic primitive retroperitoneal NET G2. The patient progressed after a somatostatin analogue-based first line, whereas the CAPTEM regimen led to a partial and durable response with a favourable safety profile.
CAPTEM chemotherapy has been shown to be an active and safe therapeutic option in advanced, metastatic G1/2 atypical primary NENs.
神经内分泌肿瘤（NENs）是一组罕见且异质性的肿瘤，具有多种原发起源和不同的侵袭性。具有非典型原发起源的 NENs，如乳腺和腹膜后 NENs，极为罕见。因此，针对这一特定亚组，尚未建立明确的诊断和治疗策略。卡培他滨和替莫唑胺的联合治疗（称为 CAPTEM 方案）在多种起源的 1 级或 2 级神经内分泌肿瘤患者中产生了有前景的反应率。
第一个病例是一名 68 岁女性，患有转移性原发性乳腺神经内分泌肿瘤，一线治疗采用顺铂加依托泊苷，随后采用 CAV 方案（环磷酰胺、多柔比星和长春新碱），随后三线治疗采用 CAPTEM 方案，获得了影像学缓解和良好的耐受性。第二个病例是一名 66 岁女性，患有转移性原始腹膜后 NET G2。患者在基于生长抑素类似物的一线治疗后进展，而 CAPTEM 方案导致部分缓解且持久，具有良好的安全性。
CAPTEM 化疗已被证明是晚期、转移性 G1/2 非典型原发性 NEN 的有效且安全的治疗选择。
