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首页> 外文期刊>Updates in Surgery >Solid pseudopapillary tumor of the pancreas in children: report of a case and review of the literature
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Solid pseudopapillary tumor of the pancreas in children: report of a case and review of the literature

机译：儿童胰腺实体假乳头状瘤：一例报告并文献复习

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摘要

Solid pseudopapillary tumor (SPT) of the pancreas is an infrequent neoplasm of low malignant potential, first described by Frantz in 1933 and representing less than 3% of all exocrine tumors. SPT is rare in children, accounting for 6% of all cases and shows different clinical features compared with adults. Here, a pediatric case is reported and a review of the Literature is provided. A 15-year-old girl with a 12 × 14 × 10 cm solid mass growing from the tail and the body of the pancreas, involving spleen, left adrenal gland and kidney, stomach and some bowel loops, was referred for surgical treatment. A distal pancreasectomy with splenectomy was performed. Histopathological examination revealed that the tumor was a 14-cm well-circumscribed solid mass, with pseudopapillary cell architecture, showing strong cellular immunoreactivity for alpha-1 antitrypsin, vimentin, neurone-specific enolase, progesterone receptors and in part to CD10 and CAM 5.2, but not to sinaptofisin and chromogranin. A 24-month post-surgical follow-up after successful surgical resection showed no evidence of recurrent disease. SPT shows different clinical features in childhood. High survival rates can be achieved in most cases, warranting aggressive treatments even in metastatic disease.
机译：胰腺实体假乳头状瘤（SPT）是恶性可能性低的罕见肿瘤，Frantz于1933年首次对其进行了描述，占所有外分泌肿瘤的不到3％。 SPT在儿童中很少见，占所有病例的6％，并且与成人相比表现出不同的临床特征。在此，报告了一个儿科病例并提供了文献复习。一名15岁女孩从胰腺尾部和身体长出12×14×10 cm实心肿块，涉及脾脏，左肾上腺和肾脏，胃和一些肠loop，被推荐进行外科治疗。进行了远端胰腺切除术和脾切除术。组织病理学检查显示，肿瘤是一个14厘米长的界限清楚的实体团，具有假乳头状细胞结构，显示出对α-1抗胰蛋白酶，波形蛋白，神经元特异性烯醇化酶，孕激素受体和部分CD10和CAM 5.2的强烈细胞免疫反应性，但不限于芥子黄素和嗜铬粒蛋白。手术切除成功后，进行了24个月的术后随访，没有发现复发的迹象。 SPT在儿童期表现出不同的临床特征。在大多数情况下，可以达到较高的生存率，即使在转移性疾病中也需要积极治疗。

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  • 来源
    《Updates in Surgery》 |2010年第1期|69-72|共4页
  • 作者

    Antonio Crucitti; Ugo Grossi; Piero Giustacchini; Pasquina M. C. Tomaiuolo; Rocco Bellantone;

  • 作者单位

    Dipartimento di Scienze Chirurgiche Policlinico “A. Gemelli” Catholic University of the Sacred Heart Largo A. Gemelli 8 00168 Rome Italy;

    Dipartimento di Scienze Chirurgiche Policlinico “A. Gemelli” Catholic University of the Sacred Heart Largo A. Gemelli 8 00168 Rome Italy;

    Dipartimento di Scienze Chirurgiche Policlinico “A. Gemelli” Catholic University of the Sacred Heart Largo A. Gemelli 8 00168 Rome Italy;

    Dipartimento di Scienze Chirurgiche Policlinico “A. Gemelli” Catholic University of the Sacred Heart Largo A. Gemelli 8 00168 Rome Italy;

    Dipartimento di Scienze Chirurgiche Policlinico “A. Gemelli” Catholic University of the Sacred Heart Largo A. Gemelli 8 00168 Rome Italy;

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  • 原文格式 PDF
  • 正文语种 eng
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  • 关键词

    Solid pseudopapillary tumor (SPT); Frantz tumor; Childhood;

    机译：实体假乳头状瘤（SPT）;Franz肿瘤;儿童期;

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