Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA.
Am J Surg Pathol. 2011 Oct;35(10):1498-504. doi: 10.1097/PAS.0b013e3182281767.
Paragangliomas are rare neuroendocrine neoplasms arising in extra-adrenal chromaffin cells of the autonomic nervous system. In rare instances, paragangliomas present around and involve the pancreas, thereby mimicking one of the more common primary pancreatic lesions. These neoplasms present considerable diagnostic difficulty not only for the clinician and radiologist but also for the pathologist. We have collected a series of 9 peripancreatic paragangliomas clinically simulating a primary pancreatic lesion. The paragangliomas were diagnosed in 4 men and 5 women with an age range of 37 to 78 years (mean, 50 y). Patients presented clinically either with diffuse epigastric and abdominal pain (7 of 9, 78%) or with an incidental mass (2 of 9, 22%) discovered on routine radiographic imaging. All patients were found to have mass lesions suspicious for a primary pancreatic neoplasm on radiographic examination. The lesions were predominantly located in the body of the pancreas (5 of 9, 56%) and ranged in size from 5.5 to 17.0 cm (mean, 10.0 cm). Five of 9 (56%) neoplasms also demonstrated cystic change. Fine-needle aspiration (FNA) was performed on 6 cases; however, the diagnostic accuracy was low, with 3 of 6 (50%) neoplasms misdiagnosed as pancreatic neuroendocrine tumor (PanNET) (n=1), spindle cell neoplasm (n=1), or pseudocyst (n=1). In addition, 2 of 8 (25%) surgically resected tumors were misdiagnosed by the referring pathologist as a PanNET. Immunohistochemistry was performed on all cases, confirming the characteristic 2-cell populations: chief cells (synaptophysin positive and chromogranin A positive) and sustentacular cells (S-100 protein positive). Follow-up information was available for all patients and ranged from 2 months to 11.6 years (mean, 2.7 y). Three of 9 (33%) patients developed metastatic disease, and 2 of these 3 died of their disease at 2.8 and 4.6 years after diagnosis. In summary, in unsuspected cases, interpretation of FNA and surgical pathology resections can be diagnostically challenging. Awareness and proper recognition of this entity, including differential diagnosis, are imperative in establishing the correct diagnosis. Further, close follow-up of these cases should be considered because of the significant risk of metastatic disease.
副神经节瘤是一种罕见的神经内分泌肿瘤，起源于自主神经系统的肾上腺外嗜铬细胞。在极少数情况下，副神经节瘤出现在胰腺周围并累及胰腺，从而模拟更常见的原发性胰腺病变之一。这些肿瘤不仅给临床医生和放射科医生，也给病理学家带来了相当大的诊断困难。我们收集了一系列 9 例临床模拟原发性胰腺病变的胰周副神经节瘤。这些副神经节瘤发生于 4 名男性和 5 名女性，年龄 37 至 78 岁（平均 50 岁）。患者的临床表现为弥漫性上腹痛和腹部疼痛（9 例中的 7 例，78%）或在常规影像学检查中偶然发现肿块（9 例中的 2 例，22%）。所有患者在影像学检查中均发现胰腺占位性病变，疑似原发性胰腺肿瘤。病变主要位于胰腺体部（9 例中的 5 例，56%），大小为 5.5 至 17.0 厘米（平均 10.0 厘米）。9 例中有 5 例（56%）肿瘤还表现出囊性变。对 6 例进行了细针穿刺抽吸（FNA），但诊断准确率较低，6 例中有 3 例（50%）误诊为胰腺神经内分泌肿瘤（PanNET）（n=1）、梭形细胞肿瘤（n=1）或假性囊肿（n=1）。此外，2 例由转诊病理学家诊断为 PanNET 的手术切除肿瘤也被误诊。对所有病例进行了免疫组织化学检查，证实了其特有的 2 种细胞群：主细胞（突触素阳性和嗜铬粒蛋白 A 阳性）和支持细胞（S-100 蛋白阳性）。所有患者均获得随访信息，随访时间为 2 个月至 11.6 年（平均 2.7 年）。9 例中有 3 例（33%）发生转移性疾病，其中 2 例在诊断后 2.8 年和 4.6 年死于该疾病。总之，在未被怀疑的情况下，细针穿刺抽吸和外科病理切除的解读可能具有诊断挑战性。认识到这一实体的存在，并包括鉴别诊断，对于确立正确的诊断至关重要。此外，由于存在显著的转移疾病风险，应考虑对这些病例进行密切随访。
