Department of Surgery, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.
Division of Endocrine Surgery, University Surgical Cluster, National University Health System, National University Hospital, Singapore, Singapore.
World J Surg Oncol. 2022 Nov 11;20(1):360. doi: 10.1186/s12957-022-02817-6.
Pheochromocytomas (PCC) are rare and functional neuroendocrine tumors developing from adrenal chromaffin cells. Predicting malignant behavior especially in the absence of metastasis can be quite challenging even in the era of improved understanding of the molecular mechanisms involved in PCCs. Currently, two histopathological grading systems Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) score are used in clinical practice, but these are subject to significant interobserver variability. Some of the most useful clinical factors associated with malignancy are large size ([4-5 cm), and genetic features such as presence of SDHB germline mutations. Local invasion is uncommon in PCC and metastasis seen in 10 to 17% but higher in germline mutations and when this occurs management can be challenging. Here, we report on a case with challenges faced by the pathologist and clinicians alike in diagnosis and management of PCC recurrence.
嗜铬细胞瘤（PCC）是一种罕见的功能性神经内分泌肿瘤，起源于肾上腺嗜铬细胞。即使在分子机制理解有所提高的时代，预测恶性行为，特别是在没有转移的情况下，仍然具有挑战性。目前，两种组织病理学分级系统——肾上腺嗜铬细胞瘤的评分系统（PASS）和肾上腺嗜铬细胞瘤和副神经节瘤的分级系统（GAPP）评分在临床实践中使用，但这些系统存在显著的观察者间变异性。与恶性肿瘤相关的一些最有用的临床因素是肿瘤较大（[4-5 厘米）和遗传特征，如存在 SDHB 种系突变。PCC 局部侵犯并不常见，转移发生率为 10%至 17%，但在种系突变和发生转移时更为常见，管理具有挑战性。在这里，我们报告了一个病例，该病例在病理学家和临床医生的诊断和 PCC 复发管理中都面临挑战。
