目的:胰腺颗粒细胞瘤(GCT)大多是良性的,非常罕见,没有独特的识别放射学特征。在对GCT患者进行病例讨论后,我们对现有文献进行了全面回顾,以确定与GCT相关的常见诊断特征.
方法:根据我们机构确定的病例报告,由两名作者按照系统评价和荟萃分析方案(PRISMA)指南的首选报告项目进行系统评价.数据库MEDLINE,EMBASE,Scopus,科学世界,和灰色文献在2021年8月进行了搜索。纳入标准为组织病理学诊断的胰腺颗粒细胞瘤。
结果:一名37岁男性出现1个月腹痛,MRI显示主胰管扩张,远端实质萎缩,但没有局灶性病变.在6个月时重复MRI再次证明了类似的发现,随后的内窥镜超声怀疑主管IPMN。经过多学科小组讨论,进行了保留脾脏的远端胰腺切除术.组织病理学显示颗粒细胞瘤,S100细胞呈弥漫性阳性,无恶性转化。在文献中确定了11例病例报告,并根据S-100蛋白的免疫组织化学阳性染色在组织组织病理学上证实了诊断。8例患者出现胃肠道症状,腹痛是主要主诉(50%)。10例患者行门静脉造影CT检查,全部行内镜检查。在EUS的五项研究中,影像学发现相似,这表明低回声病变具有均匀的外观。在非对比CT上,GCT是等增强的,门静脉造影显示增强减弱,后期逐渐增强。根据影像学和活检描述了6例胰腺癌的术前诊断。导致手术切除的进展。9例患者接受了手术治疗,随访(6-52个月)未发现并发症。
结论:目前提出的管理途径包括EUS活检和CT,和手术切除建议由于恶性肿瘤的风险。使用EUS-FNA和使用S-100免疫组织化学的显微镜评估改进的样品收集可以改善术前诊断。局限性包括报道文献中的罕见数字和短期随访,无法评估GCT的自然史和恶性肿瘤风险。其他病例将扩大胰腺GCT的当前数据集,以便将来可以避免手术切除。
OBJECTIVE: Granular cell tumours (GCTs) of the pancreas are mostly benign and exceptionally rare, with no unique identifying radiological features. Following a case discussion of a patient with GCT, a comprehensive review of available literature was conducted to identify the common diagnostic features associated with GCT.
METHODS: Following a case report identified in our institution, a systematic review was conducted by two authors in accordance with Preferred Reporting Items for Systematic review and Meta-Analysis protocols (PRISMA) guidelines. Databases MEDLINE, EMBASE, Scopus, World of Science, and grey literature were searched on August 2021. Inclusion criteria were histopathology diagnosed granular cell tumour of the pancreas.
RESULTS: A 37-year-old male presented with 1 month of abdominal pain and an MRI demonstrating a dilated main pancreatic duct, distal parenchymal atrophy, but no focal lesion. Repeat MRI at 6 months re-demonstrated similar findings and subsequent endoscopic ultrasound was suspicious for main duct IPMN. Following multidisciplinary team discussion, a spleen-preserving distal pancreatectomy was performed. Histopathology demonstrated granular cell tumour with cells diffusely positive for S100 and no malignant transformation. 11 case reports were identified in the literature with diagnosis confirmed on tissue histopathology based on positive immunohistochemical staining for S-100 protein. Eight patients presented with gastrointestinal symptoms with abdominal pain the main presenting complaint (50%). 10 patients underwent CT with portal venous contrast and all underwent endoscopic examination. Imaging findings were similar in five studies for EUS which demonstrated a hypoechoic lesion with homogenous appearance. On non-contrast CT GCT was iso-enhancing, and with portal venous contrast demonstrated hypo-enhancement that gradually enhanced on late phases. Pre-operative diagnosis of pancreatic carcinoma was described in six cases based on imaging and biopsy, resulting in progression to surgical resection. Nine patients were managed surgically and no complications identified on follow-up (6-52 months).
CONCLUSIONS: The currently proposed management pathway includes EUS with biopsy and CT, and surgical resection recommended due to malignancy risk. Improved sample collection with EUS-FNA and microscopic assessment utilising S-100 immunohistochemistry may improve pre-operative diagnosis. Limitations include rare numbers in reported literature and short follow-up not allowing an assessment of GCT\'s natural history and malignancy risk. Additional cases would expand the current dataset of GCTs of the pancreas, so that surgical resection may be avoided in the future.