Zhao Xiaofeng, Bocker Edmonston Tina, Miick Ronald, Joneja Upasana
Department of Pathology, Cooper University Hospital, Camden, NJ 08103, United States.
World J Gastrointest Oncol. 2024 Dec 15;16(12):4738-4745. doi: 10.4251/wjgo.v16.i12.4738.
Pancreatic mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs) are rare malignancies affecting the pancreas. The World Health Organization defines MiNENs as neoplasms composed of morphologically recognizable neuroendocrine and non-neuroendocrine components, each constituting 30% or more of the tumor volume. Adenocarcinoma-neuroendocrine carcinoma is the most frequent MiNEN combination. A well-differentiated neuroendocrine tumor (NET) component is rarely reported in MiNENs.
Here we report a rare case with intermingled components of ductal adenocarcinoma and grade 1 well-differentiated NET in the pancreas. The two tumors show distinct histology and significant differentiation discrepancy (poorly differentiated high grade adenocarcinoma and well-differentiated low grade NET), and also present as metastases in separate lymph nodes. Next generation sequencing of the two components demonstrates and mutations in the ductal adenocarcinoma, but no genetic alterations in the NET, suggesting divergent origins for these two components. Although tumors like this meet the diagnostic criteria for MiNEN, clinicians often find the diagnosis and staging confusing and impractical for clinical management.
Mixed NET/non-NET tumors with distinct histology and molecular profiles might be better classified as collision tumors rather than MiNENs.
胰腺混合性神经内分泌-非神经内分泌肿瘤(MiNENs)是一种累及胰腺的罕见恶性肿瘤。世界卫生组织将MiNENs定义为由形态学上可识别的神经内分泌和非神经内分泌成分组成的肿瘤,每种成分占肿瘤体积的30%或更多。腺癌-神经内分泌癌是最常见的MiNEN组合。在MiNENs中很少报道高分化神经内分泌肿瘤(NET)成分。
在此,我们报告一例罕见病例,胰腺中存在导管腺癌和1级高分化NET的混合成分。这两种肿瘤显示出不同的组织学特征和显著的分化差异(低分化高级别腺癌和高分化低级别NET),并且在不同的淋巴结中出现转移。对这两种成分进行二代测序显示,导管腺癌中有 和 突变,但NET中未发现基因改变,提示这两种成分起源不同。尽管这样的肿瘤符合MiNEN的诊断标准,但临床医生常常发现其诊断和分期对于临床管理来说既混乱又不实用。
具有不同组织学和分子特征的混合性NET/非NET肿瘤可能更好地归类为碰撞瘤而非MiNENs。