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Review
. 2021 Nov 19;29(3):151-162.
doi: 10.1159/000519933. eCollection 2022 May.

Solid Pseudopapillary Neoplasm of the Pancreas: Unfolding an Intriguing Condition

Manuel António Alves Cruz  1 , Pedro Moutinho-Ribeiro  1   2 , Pedro Costa-Moreira  1   2 , Guilherme Macedo  1   2
Affiliations
Review

Solid Pseudopapillary Neoplasm of the Pancreas: Unfolding an Intriguing Condition

Manuel António Alves Cruz et al. GE Port J Gastroenterol. .

Abstract
in English, Portuguese

Pancreatic cancer is one of the most lethal malignant neoplasms, with a 1-year survival rate after diagnosis of 24%, and a 5-year survival rate of only 9%. While this illustrates the behavior of its main histologic type - ductal adenocarcinoma, there are other histologic subtypes of pancreatic cancer that can harbor excellent prognosis. Solid pseudopapillary neoplasm, described as a rare low-grade malignant neoplasm by the World Health Organization, is the best example of that, having an overall 5-year survival rate of about 97%. Not only the prognosis, but everything about this entity is unique: its histogenesis, epidemiology, presentation, imaging characteristics, cytology features, immunohistochemical profile, and treatment. This explains the urge to improve our understanding about this entity and thus our ability to accurately recognize and manage it. Having this in mind, this article aims to summarize the most relevant topics regarding this entity.

O cancro do pâncreas é uma das neoplasias malignas mais letais, com uma taxa de sobrevida 1 ano após o diagnóstico de 24% e 5 anos após o diagnóstico de apenas 9%. Estes dados espelham, contudo, o comportamento do subtipo histológico mais prevalente − o adenocarcinoma ductal. Porém, nem todas as neoplasias malignas do pâncreas são adenocarcinomas e nem todas estão a associadas a um prognóstico tão reservado. A Neoplasia Pseudopapilar Sólida do Pâncreas é o exemplo disso: descrita pela Organização Mundial da Saúde como uma neoplasia maligna de baixo grau, é um tumor raro associado a um excelente prognóstico, com uma taxa de sobrevida aos 5 anos de 97%. Mais que uma neoplasia com um prognostico peculiarmente favorável, é uma neoplasia única em todas as suas componentes: histogénese, epidemiologia, apresentação clínica, características imagiológicas, citológicas e imunohistoquímicas e no tratamento. Estas particularidades devem estar presentes e consolidadas no raciocínio clínico de qualquer médico, para que estas neoplasias sejam devidamente reconhecidas e tratadas. Neste sentido, foi realizado este artigo de revisão que visa sumariar os mais relevantes tópicos relacionados com esta entidade clínica.

Keywords: Neoplasm; Pancreas; Solid pseudopapillary neoplasm.

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Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

Fig. 1
Fig. 1
Contrast-enhanced coronal (a) and axial (b) CT showing a large mass in the tail of the pancreas, with central hypodensity and heterogenous peripherical enhancement, associated with a giant hypervascular hepatic mass (a).
Fig. 2
Fig. 2
a Endoscopic ultrasonography showing a large and well-demarcated lesion, located on the body and tail of the pancreas, with mixed echogenicity revealing solid and cystic components. b Fine-needle aspiration with a 19-gauge needle was performed for cytology and biochemical analysis.
Fig. 3
Fig. 3
T2-weighted (a) and T1-weighted (b) magnetic resonance images showing an encapsulated lesion in the body/tail of the pancreas, heterogeneously slightly hyperintense on the T2-weighted image and with low signal intensity on the T1-weighted image. Areas of hemorrhagic degeneration (low signal on the T2- and strong signal on the T1-weighted image) can also be seen.
Fig. 4
Fig. 4
Histopathology images from surgical specimen showing: a pseudopapillary pattern (hematoxylin and eosin [HE], original magnification, ×13) (a); solid tumor tissue featuring cells with clear cytoplasm (HE, original magnification, ×30) (b); nuclei with grooved membranes (HE, original magnification, ×35) (c); and hyaline globules between tumor cells (HE, original magnification, ×27) (d).
See this image and copyright information in PMC

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