Solid-pseudopapillary Neoplasms of the Pancreas is still an Enigma: a Clinicopathological Review

Abstract

The solid-pseudopapillary neoplasm of the pancreas is a rare but enigmatic entity occurring mainly in young women. Since the first description by V. Frantz in 1959 the terminology of this tumor has continuously changed but it has remained simply descriptive, because the exact histogenesis is still obscure. Although in majority of cases the survival is excellent, nevertheless, the expected prognosis is not exactly predictable. In this review the authors aim to summarize its clinico-pathological features, the expected biological behavior, the molecular alterations, the immune phenotype and discuss the putative histogenesis. From diagnostic point of view, the salient histological characteristic findings are analyzed that would help to differentiate it from other, look-alike pancreatic tumors, and suggestions are made about the desirable content of the histological report.

Surgical Management of Solid Pseudopapillary Neoplasms of Pancreas: A Single-Center Experience of 60 Patients

“…Due to the rarity of SPNs, most of the current data are from case reports and retrospective case series. The biological behavior and prognosis of SPNs are still difficult to predict, and pancreatic SPN is still an enigma [14,15]. Surgery remains the mainstay of therapy [6,7,16], but selection of surgical approaches is still under debate.…”

Extrapancreatic primary solid pseudopapillary tumor in the gastric antrum: Case report

“…Histologic positivity for vimentin, nuclear beta‐catenin, or cyclin‐D1, and a lack of neuroendocrine markers such as chromogranin‐A usually prompt an accurate diagnosis of SPT 44 . Most SPTs are positive for CD10 with a perinuclear dot pattern, and CD99 is also a reliable positive marker 44 . Neuroendocrine tumors can be confused with SPT 45 because they both express synaptophysin and CD56.…”

“…They can also have intracytoplasmic hyaline globules such as SPTs (5% in neuroendocrine tumors 45 ). Nuclear beta‐catenin is classically positive in SPT 46,47 but negative in neuroendocrine tumors 44 . The absence of nuclear expression of beta‐catenin, negativity for CD99, TFE3, and the absence of arguments for a neuroendocrine tumor 44 led to the diagnosis of undifferentiated carcinoma initially.…”

“…Nuclear beta‐catenin is classically positive in SPT 46,47 but negative in neuroendocrine tumors 44 . The absence of nuclear expression of beta‐catenin, negativity for CD99, TFE3, and the absence of arguments for a neuroendocrine tumor 44 led to the diagnosis of undifferentiated carcinoma initially. Only the final histological examination after gastrectomy made the diagnosis of EPSPT possible.…”

“…Only the final histological examination after gastrectomy made the diagnosis of EPSPT possible. Notably, 1.4% of SPTs are negative for vimentin, 3 2.1% do not have nuclear labeling for beta‐catenin, 3 4.3% are CD10‐negative, 3 6% are negative for TFE3, 44 and 18.5% are negative for synaptophysin 3 …”