Poorly Differentiated Neuroendocrine Carcinomas of the Pancreas A Clinicopathologic Analysis of 44 Cases
Poorly Differentiated Neuroendocrine Carcinomas of the Pancreas A Clinicopathologic Analysis of 44 Cases
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DOI：
10.1097/pas.0000000000000169
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发表时间：
2014-04-01
影响因子：
5.6
通讯作者：
Klimstra, David S.
中科院分区：
文献类型：
--
作者：
Basturk, Olca;Tang, Laura;Klimstra, David S.
背景：在胰腺中，低分化的神经内分泌癌包括小细胞癌和大细胞神经内分泌癌，很少见；关于其病理和临床特征的资料非常有限。设计：采用2010年世界卫生组织提出的胃肠胰系统分类和分级（有丝分裂率/Ki67指数）标准，对107例最初诊断为低分化神经内分泌癌的胰腺切除术患者进行重新评估。对神经内分泌和腺泡分化标志物进行免疫组化标记。63例重新分类，多为分化良好的神经内分泌肿瘤（NET）或腺泡细胞癌，并予以排除。进一步评估其余44例低分化神经内分泌癌的临床病理特征和生存期。结果：患者平均年龄59岁（21 ~ 82岁），男女比例为1.4。27个肿瘤位于胰腺头部，3个位于身体，11个位于尾部。中位肿瘤大小为4cm（范围2 ~ 18cm）。27例为大细胞神经内分泌癌，17例为小细胞癌（平均有丝分裂率分别为37/10和51/10 HPF，平均Ki67指数分别为66%和75%）。8个肿瘤有合并成分，大多数是腺癌。此外，2例肿瘤具有高分化NET成分。88%的患者在发病时患有淋巴结或远处转移性疾病，另外7%的患者随后发生转移。43例患者可获得随访信息；33例死于疾病，中位生存期为11个月（范围0 - 104个月）；8例患者存活，中位随访时间为19.5个月（0 ~ 71个月）。2年和5年生存率分别为22.5%和16.1%。结论：胰腺低分化神经内分泌癌是一种高侵袭性肿瘤，易转移，生存率低。大多数患者在不到一年的时间内死亡。大多数（61%）为大细胞神经内分泌癌。高分化NET和腺泡细胞癌常被误诊为低分化神经内分泌癌，强调需要明确遵循诊断标准以确保准确诊断。
Background: In the pancreas, poorly differentiated neuroendocrine carcinomas include small cell carcinoma and large cell neuroendocrine carcinoma and are rare; data regarding their pathologic and clinical features are very limited. Design: A total of 107 pancreatic resections originally diagnosed as poorly differentiated neuroendocrine carcinomas were reassessed using the classification and grading (mitotic rate/Ki67 index) criteria put forth by the World Health Organization in 2010 for the gastroenteropancreatic system. Immunohistochemical labeling for neuroendocrine and acinar differentiation markers was performed. Sixty-three cases were reclassified, mostly as well-differentiated neuroendocrine tumor (NET) or acinar cell carcinoma, and eliminated. The clinicopathologic features and survival of the remaining 44 poorly differentiated neuroendocrine carcinomas were further assessed. Results: The mean patient age was 59 years (range, 21 to 82 y), and the male/female ratio was 1.4. Twenty-seven tumors were located in the head of the pancreas, 3 in the body, and 11 in the tail. The median tumor size was 4 cm (range, 2 to 18 cm). Twenty-seven tumors were large cell neuroendocrine carcinomas, and 17 were small cell carcinomas (mean mitotic rate, 37/10 and 51/10 HPF; mean Ki67 index, 66% and 75%, respectively). Eight tumors had combined components, mostly adenocarcinomas. In addition, 2 tumors had components of well-differentiated NET. Eighty-eight percent of the patients had nodal or distant metastatic disease at presentation, and an additional 7% developed metastases subsequently. Follow-up information was available for 43 patients; 33 died of disease, with a median survival of 11 months (range, 0 to 104 mo); 8 were alive with disease, with a median follow-up of 19.5 months (range, 0 to 71 mo). The 2- and 5-year survival rates were 22.5% and 16.1%, respectively. Conclusions: Poorly differentiated neuroendocrine carcinoma of the pancreas is a highly aggressive neoplasm, with frequent metastases and poor survival. Most patients die within less than a year. Most (61%) are large cell neuroendocrine carcinomas. Well-differentiated NET and acinar cell carcinoma are often misdiagnosed as poorly differentiated neuroendocrine carcinoma, emphasizing that diagnostic criteria need to be clearly followed to ensure accurate diagnosis.