Department of Pathology, Josephine Nefkens Institute, Erasmus MC-University Medical Center Rotterdam, Room Ae304, P.O. Box 2040, 3000, CA, Rotterdam, The Netherlands.
Endocr Pathol. 2012 Mar;23(1):43-54. doi: 10.1007/s12022-012-9194-y.
Pheochromocytomas (PCCs) are slow-growing neuroendocrine tumors arising from adrenal chromaffin cells. Tumors arising from extra-adrenal chromaffin cells are called paragangliomas. Metastases can occur up to approximately 60% or even more in specific subgroups of patients. There are still no well-established and clinically accepted "metastatic" markers available to determine whether a primary tumor is or will become malignant. Surgical resection is the most common treatment for non-metastatic PCCs, but no standard treatment/regimen is available for metastatic PCC. To investigate what kind of therapies are suitable for the treatment of metastatic PCC, animal models or cell lines are very useful. Over the last two decades, various mouse and rat models have been created presenting with PCC, which include models presenting tumors that are to a certain degree biochemically and/or molecularly similar to human PCC, and develop metastases. To be able to investigate which chemotherapeutic options could be useful for the treatment of metastatic PCC, cell lines such as mouse pheochromocytoma (MPC) and mouse tumor tissue (MTT) cells have been recently introduced and they both showed metastatic behavior. It appears these MPC and MTT cells are biochemically and molecularly similar to some human PCCs, are easily visualized by different imaging techniques, and respond to different therapies. These studies also indicate that some mouse models and both mouse PCC cell lines are suitable for testing new therapies for metastatic PCC.
嗜铬细胞瘤（PCCs）是起源于肾上腺嗜铬细胞的生长缓慢的神经内分泌肿瘤。起源于肾上腺外嗜铬细胞的肿瘤称为副神经节瘤。在特定的患者亚组中，转移的发生率高达约 60%甚至更高。目前仍然没有经过充分验证且临床可接受的“转移性”标志物来确定原发性肿瘤是否或将会发生恶性转化。手术切除是非转移性 PCC 的最常见治疗方法，但对于转移性 PCC 尚无标准的治疗方案。为了研究哪些治疗方法适用于转移性 PCC 的治疗，动物模型或细胞系非常有用。在过去的二十年中，已经创建了各种呈现 PCC 的小鼠和大鼠模型，其中包括在一定程度上具有与人类 PCC 相似的生化和/或分子特征并发生转移的肿瘤模型。为了能够研究哪些化疗方案可能对转移性 PCC 的治疗有用，最近引入了一些细胞系，如小鼠嗜铬细胞瘤（MPC）和小鼠肿瘤组织（MTT）细胞，它们都表现出转移性行为。似乎这些 MPC 和 MTT 细胞在生化和分子上与一些人类 PCC 相似，可通过不同的成像技术轻松可视化，并对不同的治疗方法有反应。这些研究还表明，一些小鼠模型和两种小鼠 PCC 细胞系都适合用于测试转移性 PCC 的新治疗方法。
