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Abstract：
BACKGROUND: The clinical course and surgical outcomes of undifferentiated sarcomatoid carcinoma of the pancreas (USCP) remain poorly characterized owing to its rarity. This study aimed to describe the histology, clinicopathologic features, perioperative outcomes, and overall survival (OS) of 23 resected USCP patients.
METHODS: We retrospectively described the histology, clinicopathologic features, perioperative outcomes and OS of patients who underwent pancreatectomy with a final diagnosis of USCP in a single institution.
RESULTS: A total of 23 patients were included in this study. Twelve patients were male, the median age at diagnosis was 61.5 ± 13.0 years (range: 35-89). Patients with USCP had no specific symptoms and characteristic imaging findings. The R0 resection was achieved in 21 cases. The En bloc resection and reconstruction of mesenteric-portal axis was undertaken in 9 patients. There were no deaths attributed to perioperative complications in this study. The intraoperative tumor-draining lymph nodes (TDLNs) dissection was undergone in 14 patients. The 1-, 3- and 5-year survival rates were 43.5%, 4.8% and 4.8% in the whole study, the median survival was 9.0 months. Only 1 patient had survived more than 5 years and was still alive at last follow-up. The presence of distant metastasis (p = 0.004) and the presence of pathologically confirmed mesenteric-portal axis invasion (p = 0.007) was independently associated with poor OS.
CONCLUSIONS: USCP was a rare subgroup of pancreatic malignancies with a bleak prognosis. To make a diagnose of USCP by imaging was quite difficult because of the absence of specific manifestations. Accurate diagnosis depended on pathological biopsy, and the IHC profile of USCP was mainly characterized by co-expression of epithelial and mesenchymal markers. A large proportion of patients have an early demise, especially for patients with distant metastasis and pathologically confirmed mesenteric-portal axis invasion. Long-term survival after radical resection of USCPs remains rare.
METHODS: We retrospectively described the histology, clinicopathologic features, perioperative outcomes and OS of patients who underwent pancreatectomy with a final diagnosis of USCP in a single institution.
RESULTS: A total of 23 patients were included in this study. Twelve patients were male, the median age at diagnosis was 61.5 ± 13.0 years (range: 35-89). Patients with USCP had no specific symptoms and characteristic imaging findings. The R0 resection was achieved in 21 cases. The En bloc resection and reconstruction of mesenteric-portal axis was undertaken in 9 patients. There were no deaths attributed to perioperative complications in this study. The intraoperative tumor-draining lymph nodes (TDLNs) dissection was undergone in 14 patients. The 1-, 3- and 5-year survival rates were 43.5%, 4.8% and 4.8% in the whole study, the median survival was 9.0 months. Only 1 patient had survived more than 5 years and was still alive at last follow-up. The presence of distant metastasis (p = 0.004) and the presence of pathologically confirmed mesenteric-portal axis invasion (p = 0.007) was independently associated with poor OS.
CONCLUSIONS: USCP was a rare subgroup of pancreatic malignancies with a bleak prognosis. To make a diagnose of USCP by imaging was quite difficult because of the absence of specific manifestations. Accurate diagnosis depended on pathological biopsy, and the IHC profile of USCP was mainly characterized by co-expression of epithelial and mesenchymal markers. A large proportion of patients have an early demise, especially for patients with distant metastasis and pathologically confirmed mesenteric-portal axis invasion. Long-term survival after radical resection of USCPs remains rare.
摘要：
背景：由于胰腺未分化肉瘤样癌（USCP）的罕见性，其临床病程和手术结果仍不明确。本研究旨在描述组织学，临床病理特征，围手术期结局,23例切除的USCP患者的总生存期（OS）。
方法：我们回顾性描述了组织学，临床病理特征，在单个机构中接受胰腺切除术并最终诊断为USCP的患者的围手术期结果和OS。
结果：本研究共纳入23例患者。12名患者为男性，诊断时的中位年龄为61.5±13.0岁(范围:35~89岁).USCP患者没有特异性症状和特征性影像学表现。21例获得R0切除。对9例患者进行了En整块切除和肠系膜门静脉轴重建。在这项研究中，没有因围手术期并发症而死亡。对14例患者进行了术中肿瘤引流淋巴结（TDLN）清扫。1-,3、5年生存率为43.5%,在整个研究中分别为4.8%和4.8%，中位生存期为9.0个月.只有1名患者存活超过5年，并且在最后一次随访时仍然活着。远处转移的存在（p=0.004）和经病理证实的肠系膜门静脉轴浸润的存在（p=0.007）与不良OS独立相关。
结论：USCP是一种罕见的胰腺恶性肿瘤亚组，预后不佳。由于缺乏特定的表现，通过成像诊断USCP非常困难。准确的诊断取决于病理活检，USCP的IHC谱主要表现为上皮和间充质标志物的共表达。很大一部分病人早逝,特别是对于有远处转移和病理证实的肠系膜-门轴侵犯的患者。USCPs根治性切除术后的长期生存仍然很少。
方法：我们回顾性描述了组织学，临床病理特征，在单个机构中接受胰腺切除术并最终诊断为USCP的患者的围手术期结果和OS。
结果：本研究共纳入23例患者。12名患者为男性，诊断时的中位年龄为61.5±13.0岁(范围:35~89岁).USCP患者没有特异性症状和特征性影像学表现。21例获得R0切除。对9例患者进行了En整块切除和肠系膜门静脉轴重建。在这项研究中，没有因围手术期并发症而死亡。对14例患者进行了术中肿瘤引流淋巴结（TDLN）清扫。1-,3、5年生存率为43.5%,在整个研究中分别为4.8%和4.8%，中位生存期为9.0个月.只有1名患者存活超过5年，并且在最后一次随访时仍然活着。远处转移的存在（p=0.004）和经病理证实的肠系膜门静脉轴浸润的存在（p=0.007）与不良OS独立相关。
结论：USCP是一种罕见的胰腺恶性肿瘤亚组，预后不佳。由于缺乏特定的表现，通过成像诊断USCP非常困难。准确的诊断取决于病理活检，USCP的IHC谱主要表现为上皮和间充质标志物的共表达。很大一部分病人早逝,特别是对于有远处转移和病理证实的肠系膜-门轴侵犯的患者。USCPs根治性切除术后的长期生存仍然很少。
