Diagnosis, treatment, and prognosis of adult pancreatoblastoma

IF 2.9 2区医学 Q2 ONCOLOGY Cancer Medicine Pub Date : 2024-08-20 DOI:10.1002/cam4.70132

Jiaqian Yuan, Yong Guo, Yan Li

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Abstract

Background

Pancreatoblastoma (PB) is one of the rare malignant tumors that typically occurs in children. Cases of PB in adults are highly unusual. This disease often presents with subtle symptoms and lacks characteristic clinical manifestations, leading to diagnostic challenges.

Objective

This study integrates the relevant literature on adult PB, conducting data analysis on clinical features, laboratory and imaging results, pathological characteristics, and treatments according to inclusion and exclusion criteria. Kaplan–Meier univariate analysis and Log-rank tests are employed to analyze survival data from adult PB follow-up, exploring factors influencing prognosis.

Results

A total of 65 articles were included, encompassing 103 cases of adult PB. The average age of PB patients was 41.78 years (range 19–81 years), and the male-to-female ratio was 1.06:1. Patients frequently presented with abdominal pain as the initial symptom. Laboratory results lacked specificity and imaging findings often presented as large, well-defined masses. PB exhibited distinctive pathological features, including squamous corpuscles (n = 76, 89.41%) and acinar differentiation (n = 34, 40%), with frequent positive expression of Trypsin, Chymotrypsin, and AACT (Alpha-1-Antichymotrypsin). APC (Adenomatous Polyposis Coli) gene mutation was the most common molecular alteration in adult PB. During the follow-up period, 43.59% of patients died (range 3 days to 348 months). The primary factors affecting prognosis were the presence of metastasis (χ² = 3.996, p = 0.046) and incomplete surgical resection (χ² = 5.586, p = 0.018), with mean survival times of 48 months and 27 months, respectively.

Conclusions

PB in adults is an invasive tumor. The key to distinguishing PB from other pancreatic tumors lies in recognizing its unique pathological feature, the squamous corpuscles. Timely and complete surgical resection is the preferred treatment following diagnosis. Patients with incomplete resection or the presence of lymph nodes or (and) distant metastases have a poor prognosis.

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成人胰母细胞瘤的诊断、治疗和预后。

背景：胰母细胞瘤（PB）是罕见的恶性肿瘤之一，通常发生在儿童身上。成人胰母细胞瘤病例非常罕见。这种疾病通常症状不明显，缺乏特征性临床表现，因此诊断困难：本研究整合了有关成人 PB 的相关文献，根据纳入和排除标准对临床特征、实验室和影像学结果、病理特征和治疗方法进行了数据分析。采用Kaplan-Meier单变量分析和Log-rank检验分析成人PB随访生存数据，探讨影响预后的因素：结果：共纳入 65 篇文章，涵盖 103 例成人 PB。PB患者的平均年龄为41.78岁（19-81岁），男女比例为1.06:1。患者常以腹痛为首发症状。实验室检查结果缺乏特异性，影像学检查结果通常表现为界限清楚的大肿块。PB表现出明显的病理特征，包括鳞状细胞（76例，89.41%）和针状分化（34例，40%），胰蛋白酶、糜蛋白酶和AACT（α-1-抗糜蛋白酶）常呈阳性表达。APC（腺瘤性息肉病大肠杆菌）基因突变是成人 PB 中最常见的分子改变。在随访期间，43.59%的患者死亡（3天至348个月）。影响预后的主要因素是出现转移（χ2 = 3.996，P = 0.046）和手术切除不彻底（χ2 = 5.586，P = 0.018），平均生存时间分别为48个月和27个月：结论：成人PB是一种侵袭性肿瘤。将胰腺癌与其他胰腺肿瘤区分开来的关键在于识别其独特的病理特征--鳞状上皮细胞。确诊后，及时、彻底的手术切除是首选治疗方法。切除不彻底或存在淋巴结或（和）远处转移的患者预后较差。

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来源期刊

Cancer Medicine ONCOLOGY-

CiteScore

5.50

自引率

2.50%

发文量

907

审稿时长

19 weeks

期刊介绍： Cancer Medicine is a peer-reviewed, open access, interdisciplinary journal providing rapid publication of research from global biomedical researchers across the cancer sciences. The journal will consider submissions from all oncologic specialties, including, but not limited to, the following areas: Clinical Cancer Research Translational research ∙ clinical trials ∙ chemotherapy ∙ radiation therapy ∙ surgical therapy ∙ clinical observations ∙ clinical guidelines ∙ genetic consultation ∙ ethical considerations Cancer Biology: Molecular biology ∙ cellular biology ∙ molecular genetics ∙ genomics ∙ immunology ∙ epigenetics ∙ metabolic studies ∙ proteomics ∙ cytopathology ∙ carcinogenesis ∙ drug discovery and delivery. Cancer Prevention: Behavioral science ∙ psychosocial studies ∙ screening ∙ nutrition ∙ epidemiology and prevention ∙ community outreach. Bioinformatics: Gene expressions profiles ∙ gene regulation networks ∙ genome bioinformatics ∙ pathwayanalysis ∙ prognostic biomarkers. Cancer Medicine publishes original research articles, systematic reviews, meta-analyses, and research methods papers, along with invited editorials and commentaries. Original research papers must report well-conducted research with conclusions supported by the data presented in the paper.