Wang Xinbo, Zhu Daojun, Bao Wei, Li Min, Wang Sizhen, Shen Rongxi
Research Institute of General Surgery, Jinling Hospital, Nanjing University Medical School, Nanjing, China.
Department of Clinical Pathology, Jinling Hospital, Nanjing University Medical School, Nanjing, China.
Front Oncol. 2021 Oct 18;11:729151. doi: 10.3389/fonc.2021.729151. eCollection 2021.
Solid pseudopapillary neoplasm (SPN) of the pancreas shows an indolent clinical behavior in cases undergoing surgical resection. The efficacy of combination therapy in the metastatic extrapancreatic SPN treatment remains largely unknown and a clinical challenge.
We report a case of a metastatic pancreatic SPN in a 45-year-old woman who presented with an aggressive peritoneal dissemination and hepatic metastases and still showed an indolent clinical course with combination therapy with repeated surgery and targeted therapy. Although the follow-up effect remains to be seen, this is the first report of practical experience of the targeted agents sunitinib and everolimus in metastatic SPN tumors based on the mutation status of (c.379G>A; p.G127R) and (c.98C>G; p.S33C). To our knowledge, the variant identified in this case has not been previously reported in SPN.
Evidence on variant genetics indicates that future molecular studies may not only help to explain the mechanism of SPN occurrence and development but are also more likely to direct to future precision treatments.
胰腺实性假乳头状瘤（SPN）在接受手术切除的病例中表现出惰性的临床行为。联合治疗在转移性胰腺外SPN治疗中的疗效在很大程度上仍不清楚，是一项临床挑战。
我们报告了一例45岁女性的转移性胰腺SPN病例，该患者表现为侵袭性腹膜播散和肝转移，经重复手术和靶向治疗的联合治疗仍表现出惰性的临床病程。尽管随访效果还有待观察，但这是基于（c.379G>A；p.G127R）和（c.98C>G；p.S33C）的突变状态，关于靶向药物舒尼替尼和依维莫司在转移性SPN肿瘤中的实际应用经验的首例报告。据我们所知，该病例中鉴定出的 变异此前在SPN中尚未有报道。
变异遗传学证据表明，未来的分子研究不仅可能有助于解释SPN发生发展的机制，而且更有可能导向未来的精准治疗。
