Das Satya, Shi Chanjuan, Du Liping, Idrees Kamran, Berlin Jordan
Division of Hematology Oncology, Department of Medicine, Vanderbilt University Medical Center, 2220 Pierce Avenue, 777 Preston Research Building, Nashville, TN, 37232, USA.
Department of Pathology, Vanderbilt University Medical Center, Nashville, TN, USA.
J Gastrointest Cancer. 2019 Dec;50(4):709-715. doi: 10.1007/s12029-018-0131-2.
Adenocarcinoma ex-goblet cell carcinoids (AGCCs) are rare appendiceal tumors with mixed neuroendocrine and glandular features. They tend to behave more aggressively than typical carcinoid tumors, affect younger patients, and have a greater predilection for spreading to the peritoneum. Outcomes of AGCC patients treated with chemotherapy, extrapolated from colon cancer regimens, in the adjuvant or metastatic setting have not been explicitly reported. We sought to explore outcomes of AGCC patients with either local disease treated with adjuvant FOLFOX or metastatic disease treated with FOLFOX/FOLFIRI post-cytoreductive debulking (or CRS plus HIPEC in the peritoneal-limited setting).
We performed a single-institution retrospective analysis of 23 pathologically identified AGCC patients from Vanderbilt University Medical Center treated with chemotherapy in either the adjuvant or metastatic settings. Each patient's tumor was categorized as group B or group C based on the criteria from Tang et al. Median progression-free survival (PFS) or disease-free survival (DFS) (in the curative setting) and overall survival (OS) were determined for each patient and specified patient subgroup.
AGCC patients who were treated with FOLFOX chemotherapy in the adjuvant setting or FOLFOX/FOLFIRI in the metastatic setting experienced prolonged PFS, DFS, and OS. Five patients with peritoneal-limited disease treated with CRS plus HIPEC have not yet reached median PFS or OS. While small sample size, patient selection, and retrospective nature limit the generalizability of findings from our analysis, the efficacy signals we observed suggest prospective evaluation with chemotherapy and CRS plus HIPEC is warranted in AGCC patients.
杯状细胞类癌腺癌（AGCC）是一种罕见的阑尾肿瘤，具有神经内分泌和腺性混合特征。它们的行为往往比典型类癌更具侵袭性，影响年轻患者，并且更易扩散至腹膜。从结肠癌治疗方案推断而来的AGCC患者在辅助或转移情况下接受化疗的结果尚未得到明确报道。我们试图探讨接受辅助性FOLFOX治疗局部疾病或接受细胞减灭性肿瘤切除术（或在腹膜局限情况下接受CRS加HIPEC）后用FOLFOX/FOLFIRI治疗转移性疾病的AGCC患者的预后。
我们对范德堡大学医学中心23例经病理确诊、在辅助或转移情况下接受化疗的AGCC患者进行了单机构回顾性分析。根据Tang等人的标准，将每位患者的肿瘤分为B组或C组。确定每位患者及特定患者亚组的中位无进展生存期（PFS）或无病生存期（DFS）（在治愈情况下）以及总生存期（OS）。
在辅助情况下接受FOLFOX化疗或在转移情况下接受FOLFOX/FOLFIRI治疗的AGCC患者的PFS、DFS和OS均延长。5例接受CRS加HIPEC治疗的腹膜局限疾病患者尚未达到中位PFS或OS。虽然样本量小、患者选择和回顾性性质限制了我们分析结果的普遍性，但我们观察到的疗效信号表明，对AGCC患者进行化疗以及CRS加HIPEC的前瞻性评估是有必要的。
