Department of Visceral, Thoracic and Vascular Surgery, Philipps University Marburg, Germany.
Pancreas. 2011 Mar;40(2):213-21. doi: 10.1097/MPA.0b013e3182015f5d.
Pancreatic endocrine tumors (PETs) are characterized by the presence of hormone syndromes. Reports focusing on calcitonin-secreting PET (CTsPETs) are very rare. This study aimed to define a CTsPET-associated syndrome in regard to chemical, anatomical, and developmental aspects.
A computerized MEDLINE search was conducted under the search items: "pancreatic endocrine tumor," "calcitonin," "neuroendocrine pancreatic tumor," and "pancreas." Results of clinical, histopathological, immunohistochemical, and biochemical assessments of all patients identified with CTsPET were registered and statistically analyzed.
Thirty-seven patients with CTsPET were identified. Mean serum calcitonin was elevated to the 89.2-fold of the upper reference value. Main symptoms were watery diarrhea (51.4%) and abdominal pain (35.1%). Most patients (59.5%) presented with metastatic spread at the time of diagnosis. Of all patients, 66.7% were alive after a mean follow-up of 28.9 months. Survival was higher in patients who underwent more aggressive surgical therapies independent from tumor sizes and in those with no metastases at the time of diagnosis.
High calcitonin levels should always raise suspicion of medullary thyroid carcinomas. However, when thyroid examination remains without pathological findings, a CTsPET should be excluded. An aggressive surgical approach even in cases with large primary tumor sizes may lead to a longer survival.
胰腺内分泌肿瘤（PET）的特征是存在激素综合征。关于分泌降钙素的 PET（CTsPET）的报道非常罕见。本研究旨在从化学、解剖和发育方面定义 CTsPET 相关综合征。
在以下搜索项下进行了计算机 MEDLINE 搜索：“胰腺内分泌肿瘤”、“降钙素”、“神经内分泌胰腺肿瘤”和“胰腺”。对所有确诊为 CTsPET 的患者的临床、组织病理学、免疫组织化学和生化评估结果进行了登记和统计分析。
共发现 37 例 CTsPET 患者。平均血清降钙素升高至正常值的 89.2 倍。主要症状为水样腹泻（51.4%）和腹痛（35.1%）。大多数患者（59.5%）在诊断时已发生转移扩散。所有患者在平均 28.9 个月的随访后，66.7%仍存活。独立于肿瘤大小，接受更积极手术治疗的患者和诊断时无转移的患者的生存率更高。
高降钙素水平应始终怀疑为甲状腺髓样癌。然而，当甲状腺检查无病理发现时，应排除 CTsPET。即使在原发性肿瘤较大的情况下，积极的手术方法也可能导致更长的生存时间。
