Uccella Silvia, Blank Annika, Maragliano Roberta, Sessa Fausto, Perren Aurel, La Rosa Stefano
Department of Medicine and Surgery, Unit of Pathology, University of Insubria, 21100, Varese, Italy.
Institute of Pathology, University of Bern, Bern, Switzerland.
Endocr Pathol. 2017 Dec;28(4):351-361. doi: 10.1007/s12022-017-9505-4.
Increased levels of circulating calcitonin are a clue in the diagnosis of medullary thyroid carcinoma. However, hypercalcitoninemia can also be related to other pathological conditions, including pancreatic neuroendocrine neoplasms (PanNENs). Ectopic hormonal production is not unusual in both functioning and non-functioning PanNENs; however, little is known about the frequency of calcitonin expression in these neoplasms. The aims of this study were to assess the frequency of calcitonin immunoreactivity in PanNENs, independently from serum calcitonin levels, and to evaluate the clinicopathological and prognostic features of calcitonin-immunoreactive (Cal-IR) PanNENs, including a comparison with cases already reported in the literature. We screened 229 PanNENs for the immunohistochemical expression of calcitonin, including both functioning and non-functioning neoplasms, as well as both well-differentiated and poorly differentiated PanNENs. Both the clinicopathological data and the follow-up information were available and were compared with the immunohistochemical results. In addition, we reviewed the features of the calcitonin-producing PanNENs previously reported in the literature. Calcitonin was expressed in 25 of our 229 PanNENs (10.9%). Examples of well- and poorly differentiated, as well as both functioning and non-functioning PanNENs, were found to be calcitonin immunoreactive. Cal-IR PanNENs did not show any significant difference with the whole series of neoplasms included in the study, when the clinicopathological parameters were considered, except for a younger age at diagnosis and for a larger size of the tumor in non-functioning Cal-IR PanNENs. Taken together, our results show that calcitonin immunoreactivity is not an exceptional event in PanNENs. Furthermore, calcitonin expression does not identify a separate clinical entity, in contrast to other PanNENs with ectopic hormone production, such as adrenocorticotropic hormone (ACTH)-producing PanNENs, which show a distinctively more aggressive behavior.
循环降钙素水平升高是甲状腺髓样癌诊断的一个线索。然而，高降钙素血症也可能与其他病理状况有关，包括胰腺神经内分泌肿瘤（PanNENs）。在功能性和非功能性PanNENs中，异位激素产生并不罕见；然而，关于这些肿瘤中降钙素表达的频率知之甚少。本研究的目的是评估PanNENs中降钙素免疫反应性的频率，独立于血清降钙素水平，并评估降钙素免疫反应性（Cal-IR）PanNENs的临床病理和预后特征，包括与文献中已报道的病例进行比较。我们筛选了229例PanNENs进行降钙素的免疫组化表达检测，包括功能性和非功能性肿瘤，以及高分化和低分化的PanNENs。临床病理数据和随访信息均可用，并与免疫组化结果进行比较。此外，我们回顾了文献中先前报道的产生降钙素的PanNENs的特征。在我们的229例PanNENs中，有25例（10.9%）表达降钙素。发现高分化和低分化、功能性和非功能性PanNENs的病例均有降钙素免疫反应性。当考虑临床病理参数时，Cal-IR PanNENs与本研究中纳入的整个肿瘤系列相比，没有显示出任何显著差异，除了非功能性Cal-IR PanNENs诊断时年龄较小和肿瘤较大。综上所述，我们的结果表明，降钙素免疫反应性在PanNENs中并非罕见事件。此外，与其他产生异位激素的PanNENs（如产生促肾上腺皮质激素（ACTH）的PanNENs，其行为明显更具侵袭性）不同，降钙素表达并不能识别一个单独的临床实体。
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