Solid bifocal pseudopapillary neoplasm of the pancreas: A case report

Abstract

Introduction This neoplasm of the pancreas is an uncommon entity, with a frequency of 0.3–2.7% of all pancreatic tumors and even more so the finding of a synchronous lesion of the same histological lineage. For this reason, we report the atypical presentation of a SPNPs through a clinical case, review of the literature and a classification proposal, from the quantitative point of view. Case presentation 21-year-old patient, with incidental finding of two pancreatic tumors. Surgery included a pyloric preserving pancreatoduodenectomy with pancreatojejunostomy, distal pancreatectomy and central pancreas was preserved. The patient presents low output pancreatic fistula and nosocomial infection, treated with antibiotic therapy, being discharged 29 days after the intervention. Pathological and immunohistochemical analysis consistent with two SPNP. Discussion Its diagnosis is confirmed with the histological study and two synchronic SPNP are a rare entity and for this, or multiple lesions, an attempt should be made of a conservative resection of the parenchyma to minimize pancreatic insufficiency in a frequently young population, and always look for R0 resection, due to its uncertain behavior. Conclusion Bifocal SPNP is rare and for this it is utility classify this entity -from the quantitative point of view- into unifocal, bifocal and multifocal for future medical research.

Current highlights on solid pseudopapillary neoplasm of the pancreas

“…Nevertheless, no certain risk factors nor relationships with functional endocrine syndromes have been identified. SPN shows a bimodal incidence in women with two peaks at 28 years and 64 years and a unimodal behavior in men at 64 years[ 8 ]. Furthermore, recent studies described larger masses and more aggressive disease in men and post-menopausal women, suggesting an estrogen dependent behavior of these tumors[ 9 , 10 ].…”

“…The tail of the pancreas seems to be the most frequent site of presentation although bifocal lesions have been sometimes reported. Based on these findings, Flores et al [ 8 ] proposed to classify SPN as follows: Unifocal SPN, referred to single lesions, bifocal SPN when there are two lesions and multifocal SPN when they are three[ 8 ].…”

“…Histogenesis remains unclear and, although no specific immunochemistry pattern has been identified, most lesions show loss of positivity for E-cadherin and positivity for β-catenin, vimentin, alpha-1-antitrypsin, alpha-1-antitrypsin, CD10, CD117 and progesterone receptors. These characteristics may be added to the clinical, imaging and histological findings to provide diagnosis[ 8 ].…”

“…Curative resection, as conservative as possible, with both open or laparoscopic approach, is the best treatment option[ 17 - 19 ] providing long term overall and disease-free survival even in node positive patients[ 20 ]. Surgical planning is crucial and the classification proposed by Flores et al [ 8 ] could be useful in this matter[ 8 ].…”

Two isolated solid pseudopapillary neoplasms in the pancreas in an asymptomatic young woman

“…SPN is a rare entity, accounting for 1%–2% of pancreatic tumors 1 . In addition, the “bifocal” presentation of SPN in the pancreas is an atypical finding only one case report has explicitly reported 2 . EUS played a key role in pre‐surgical assessment, including careful screening of the pancreas for additional indeterminant lesions, detailed characteristics of each abnormality, and accurate evaluation of surrounding structures including outward extension, pancreatic duct, or vascular involvement.…”