Jakub Fichtl, Tomáš Skalický, Josef Vodička, Vladislav Třeška, Radek Tupý, Ondřej Hes
Klin Onkol. 2018 Fall;31(5):376-379. doi: 10.14735/amko2018376.
Benign cystic tumors represent only 2% of all pancreatic tumors (pancreatic cancer - PC). In contrast to malignant cystic tumors, these tumors occur typically in young women. A solid pseudopapillary tumor is a relatively rare affliction representing less than 4% of cystic PC. Although the tumor is considered benign, metastasis, especially to the spleen, has been reported in approximately 0.5-4% patients. Despite R0 resection, vascular and perineural invasion is monitored in 20% of cases. Invasion is the cause of tumor relapse in up to one third of affected patients. Characteristic features of the disease are latent clinical indicators such as signs of pain and malfunction of intestinal passage. The diagnostics is based on MR, sometimes in combination with positron emission tomography. Medical treatment is specifically surgical.
Authors present a case of a 20-year-old female patient who was examined due to pain in the epigastrium, further exasperated by a voluminous expansion of the abdominal cavity. An initial ultra-sonographic examination was conducted to examine for possible nodular focal nodular hyperplasia of the liver; however, an MRI scan revealed the likelihood of a malignant tumor in the subhepatic region. During laparotomy, a tumor protruding from the head of the pancreas was discovered and removed. Histological examination showed it was a solid pseudopapillary pancreatic tumor. After a month of good post-operative progress, the patient was re-operated because of the presence of pancreatic fistula. Complete healing of the fistula was achieved after total parenteral nutrition and administration of sandostatin. At her last examination, the patient was without any problems.
Solid pseudopapillary pancreatic tumors are rare, mainly benign lesions. It is essential to consider them in the differential diagnostics of afflictions of the subhepatic region, especially in young women. The only generally accepted cure nowadays is surgical resection. It is necessary to monitor patients consistently considering the rather high frequency of relapse of disease despite R0 resections. In the case of surgical removal, the 5-year survival rate is near 97%. Key words: solid pseudopapillary tumor of pancreas - diagnostics - therapy The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE recommendation for biomedical papers. Submitted: 17. 4. 2018 Accepted: 13. 8. 2018.
良性囊性肿瘤仅占所有胰腺肿瘤(胰腺癌 - PC)的2%。与恶性囊性肿瘤不同,这些肿瘤通常发生在年轻女性中。实性假乳头状肿瘤是一种相对罕见的疾病,占囊性PC的比例不到4%。尽管该肿瘤被认为是良性的,但据报道约0.5 - 4%的患者会发生转移,尤其是转移至脾脏。尽管进行了R0切除,但仍有20%的病例监测到血管和神经周围侵犯。侵犯是多达三分之一受影响患者肿瘤复发的原因。该疾病的特征性表现为潜在的临床指标,如疼痛和肠道功能紊乱的迹象。诊断基于磁共振成像(MR),有时结合正电子发射断层扫描。医学治疗主要是手术治疗。
作者介绍了一名20岁女性患者的病例,该患者因上腹部疼痛就诊,腹腔大量扩张使疼痛加剧。最初进行了超声检查以排查肝脏可能的结节性局灶性结节性增生;然而,磁共振成像扫描显示肝下区域可能存在恶性肿瘤。在剖腹手术中,发现并切除了一个从胰头突出的肿瘤。组织学检查显示为实性假乳头状胰腺肿瘤。术后一个月恢复良好,但患者因出现胰瘘而再次手术。经全胃肠外营养和使用善龙后,瘘管完全愈合。在最后一次检查时,患者没有任何问题。
实性假乳头状胰腺肿瘤罕见,主要为良性病变。在肝下区域疾病的鉴别诊断中,尤其是在年轻女性中,必须考虑到这种肿瘤。目前唯一普遍接受的治疗方法是手术切除。鉴于尽管进行了R0切除但疾病复发频率较高,有必要持续监测患者。手术切除的情况下,5年生存率接近97%。关键词:胰腺实性假乳头状肿瘤 - 诊断 - 治疗 作者声明他们在研究中使用的药物、产品或服务方面没有潜在的利益冲突。编辑委员会声明该手稿符合国际医学杂志编辑委员会(ICMJE)对生物医学论文的建议。提交日期:2018年4月17日 接受日期:2018年8月13日