[Solid pseudopapillary neoplasm. Report of three cases and review of the literature].

Javier Iván Baltazar-Ramos, Gabriela Martínez-Reyes, Miguel Ángel Pérez-Corro, Edmundo Denis-Rodríguez, Guadalupe Melo-Santiesteban
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Abstract

Background: Solid pseudopapillary neoplasm, or Frantz-Gruber tumor, is a diagnostic challenge for the surgical pathologist. It is classified by the WHO as a malignant epithelial tumor of the pancreas, its prevalence is low, it occurs in only 1 to 2% of all malignant tumors of the pancreas, it usually affects mainly young women, its origin is still unclear, it is generally It presents as a solitary, encapsulated lesion, without invasion of peripancreatic tissues with rare cases of metastasis, which is why it is considered a low-grade malignant tumor by the WHO. The objective of this article is to present three clinical cases and to evaluate the epidemiology, clinical manifestations, morphology and immunohistochemical expression of the tumor in a review of the bibliography, as well as to compare it with the cases already reported on the subject.

Clinical case: Three cases of Frantz tumor were diagnosed by the pathology department of a tertiary hospital are presented, which correspond to two women aged 17 and 34, as well as a 52-year-old man whose presentation by age and sex is rare.

Conclusions: After the bibliographical review and the analysis of the cases presented, we verified the difficulty to make a correct diagnosis, since its presence is rare in the daily practice of the surgical pathologist. The morphological patterns of the solid pseudopapillary tumor are varied and can often be reminiscent of neuroendocrine tumors of the pancreas, whose presentation rate is higher.

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实性假乳头状肿瘤。三例报告及文献复习]。
背景:实性假乳头状肿瘤,或弗朗茨-格鲁伯肿瘤,是外科病理学家诊断的一个挑战。WHO将其归类为胰腺恶性上皮性肿瘤,其患病率较低,仅占胰腺所有恶性肿瘤的1%至2%,通常主要影响年轻女性,其起源尚不清楚,通常表现为孤立的、包封的病变,不侵犯胰腺周围组织,罕见的转移病例,这就是WHO认为其为低级别恶性肿瘤的原因。本文的目的是提出三个临床病例,并在回顾文献中评价肿瘤的流行病学,临床表现,形态学和免疫组织化学表达,并与已报道的病例进行比较。临床病例:本文报道某三级医院病理科诊断的3例Frantz肿瘤,分别为2例17岁和34岁的女性,1例52岁的男性,年龄和性别比较少见。结论:经过文献回顾和病例分析,我们证实了正确诊断的困难,因为它在外科病理学家的日常实践中很少出现。实性假乳头状瘤形态多样,常与胰腺神经内分泌肿瘤相似,表现率较高。
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