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以吞咽困难和多发性肝肿瘤为表现的小儿产甲胎蛋白胃癌

Pediatric Alpha-Fetoprotein-Producing Gastric Cancer Presenting With Dysphagia and Multiple Liver Tumors.

作者信息

Hirano Takayuki, Hoshi Reina, Yamaoka Bin, Ono Kako, Watanabe Yosuke, Goto Shumpei, Hosokawa Takashi, Kanezawa Koji, Shimozawa Katsuyoshi, Arakawa Ayumu, Uehara Shuichiro

机构信息

Department of Pediatric Surgery, Nihon University School of Medicine, Tokyo, Japan.

Department of Pediatrics and Child Health, Nihon University School of Medicine, Tokyo, Japan.

出版信息

Cancer Rep (Hoboken). 2025 Sep;8(9):e70348. doi: 10.1002/cnr2.70348.

Abstract

BACKGROUND

Alpha-fetoprotein (AFP)-producing gastric cancer (AFPGC) is resistant to chemotherapy and is associated with poor prognosis. Pediatric gastric cancer has an incidence of 0.02% among gastric cancer patients, with a median survival of 5 months.

CASE

A 13-year-old boy presented with progressive dysphagia for 2 months, accompanied by loss of appetite and significant weight loss. Liver dysfunction and multiple lesions were observed. Laboratory investigations revealed elevated liver enzymes and tumor markers, including AFP, at 24 502 ng/mL. Computed tomography (CT) showed thickening of the gastric cardia and multiple liver lesions. Upper gastrointestinal endoscopy revealed a type 2 tumor in the cardia. Histopathology confirmed adenocarcinoma, and immunohistochemical staining was positive for AFP, establishing a diagnosis of AFPGC with liver metastases (Stage IV). Given the unresectable and HER2-negative nature of the cancer, chemotherapy with TS-1 and cisplatin was initiated, resulting in a temporary reduction in AFP levels and tumor size. However, disease progression was noted after 3 months, requiring a switch in treatment to ramucirumab, paclitaxel, bleomycin, etoposide, cisplatin, or a study drug. Despite these efforts, the patient succumbed to the disease 16 months after initial treatment.

CONCLUSION

AFPGC in children is extremely rare, with few reported cases. The 16-month survival observed in this case exceeds previously reported durations (8 and 4 months). Systematic evaluation of persistent gastrointestinal symptoms enabled earlier diagnosis. Standard adult gastric cancer treatment protocols appeared more effective than AFP-tumor-specific regimens, suggesting they may be optimal for pediatric AFPGC as well. Early diagnosis through detailed history-taking and prompt endoscopic examinations in children with gastrointestinal symptoms may lead to significantly prolonged survival and improved management in this rare malignancy.

摘要

背景

产生甲胎蛋白(AFP)的胃癌(AFPGC)对化疗耐药,且预后不良。儿童胃癌在胃癌患者中的发病率为0.02%,中位生存期为5个月。

病例

一名13岁男孩出现进行性吞咽困难2个月,伴有食欲减退和显著体重减轻。观察到肝功能障碍和多处病变。实验室检查显示肝酶和肿瘤标志物升高,包括AFP,达24502 ng/mL。计算机断层扫描(CT)显示贲门增厚和多处肝脏病变。上消化道内镜检查显示贲门处有2型肿瘤。组织病理学确诊为腺癌,免疫组化染色AFP呈阳性,确诊为伴有肝转移(IV期)的AFPGC。鉴于该癌症无法切除且人表皮生长因子受体2(HER2)阴性,开始使用替吉奥(TS-1)和顺铂进行化疗,导致AFP水平和肿瘤大小暂时降低。然而,3个月后疾病进展,需要更换治疗方案为雷莫西尤单抗、紫杉醇、博来霉素、依托泊苷、顺铂或一种研究药物。尽管采取了这些措施,患者在初始治疗16个月后死于该疾病。

结论

儿童AFPGC极为罕见,报道的病例很少。该病例观察到的16个月生存期超过了先前报道的时长(8个月和4个月)。对持续的胃肠道症状进行系统评估有助于早期诊断。标准的成人胃癌治疗方案似乎比针对AFP肿瘤的特定方案更有效,这表明它们可能对儿童AFPGC也是最佳的。通过对有胃肠道症状的儿童进行详细的病史询问和及时的内镜检查进行早期诊断,可能会显著延长这种罕见恶性肿瘤的生存期并改善治疗效果。

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