Context.— Solid pseudopapillary neoplasm of the pancreas is a low-grade malignant tumor generally associated with a good prognosis. Solid pseudopapillary neoplasms show peculiar morphologic feature...

/pdf/pancreatic-solid-pseudopapillary-neoplasm-key-pathologic-and-15weiufjv9.pdf

Pancreatic Solid Pseudopapillary Neoplasm: Key Pathologic and Genetic Features.

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.

/pdf/solid-pseudopapillary-neoplasms-of-the-pancreas-is-still-an-18lvh7kfgu.pdf

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

The diagnosis of solid pseudopapillary neoplasms (SPNs) is challenging because some SPNs share many similar morphological and immunohistochemical features with other pancreatic neoplasms. In this study, we investigated potential diagnostic markers of SPN. Based on the SPN-specific upregulated genes from a previous DNA microarray and proteome study, we selected six immunohistochemical markers [beta-catenin, androgen receptor (AR), lymphoid enhancer-binding factor 1 (LEF1), transcription factor for immunoglobulin heavy-chain enhancer 3 (TFE3), fused in sarcoma (FUS), and WNT inhibitory factor 1 (WIF-1)]. We also evaluated the Ki-67 proliferative index to investigate its associations with prognosis. To validate these markers, we studied 91 SPNs as well as 51 pancreatic ductal carcinomas (PDC) and 48 neuroendocrine tumors (NET) as controls. We found frequent and diffuse nuclear expressions of β-catenin (98.9%), AR (81.3%), LEF1 (93.4%), TFE3 (74.7%), FUS (84.6%), and cytoplasmic expression of WIF-1 (96.7%) in SPNs. In contrast, PDCs and NETs showed no expression. (P < 0.001). When beta-catenin, LEF1, and TFE3 staining were combined, the sensitivity and specificity were 100% and 91.9%, respectively. Four (4.4%) SPNs showed distant metastasis and these tumors were associated with a relatively high Ki-67 proliferative index (≥ 5%; P = 0.013). We identified LEF1, TFE3, and AR as putative diagnostic markers of SPN, auxiliary to β-catenin. Incorporated into an immunohistochemical panel, these markers could be beneficial to distinguish SPN from PDC and NET. In addition, we suggest that the Ki-67 proliferative index can be a predictive marker of metastasis in SPNs.

/pdf/lef1-tfe3-and-ar-are-putative-diagnostic-markers-of-solid-51e7kivubh.pdf

LEF1, TFE3, and AR are putative diagnostic markers of solid pseudopapillary neoplasms

Background Solid pseudopapillary tumor of the pancreas (SPTP) has been reported as a rare disease with low malignant potential. The aim of this study was to summarize experiences of the diagnosis and treatment for the patients reported in the Chinese population. Method 2450 SPTP cases reported in English and Chinese literature before Jan 2020 were for our review and analysis retrospectively. Result There are 389 male cases and 2061 female cases, and the ratio of male/female was 1 : 5.3. The average age was 29.3 years. The main clinical symptoms were upper abdominal pain and bloating discomfort in 51.6% of the cases and epigastric mass. 38.6% of the tumor was located at the head of the pancreas and 55.4% at the body and tail of the pancreas. The most frequent operative styles were tumor enucleation (38.4%). Pathology showed that the average diameter of the tumor was 8.2 cm and 12.3% of SPTP was malignant. 98.3% of cases had favorable survival. Conclusions SPTP is a rare indolent tumor occurring mainly in young women, and the main clinical performances are abdominal mass and abdominal pain; most tumors are distributed at the head and the tail of the pancreas; the prognosis after complete resection is excellent.

/pdf/a-review-of-clinicopathological-characteristics-and-4p8l45zbl9.pdf

A Review of Clinicopathological Characteristics and Treatment of Solid Pseudopapillary Tumor of the Pancreas with 2450 Cases in Chinese Population.

Solid pseudopapillary tumor is a rare low grade malignant pancreatic tumor that occurs mainly in women (85-90%). The wide variability of histological features of SPNs reflects on the imaging findings, from completely solid tumors to almost completely cystic masses. Smaller masses are usually completely or mostly solid, while larger lesions typically present as large well-encapsulated masses with varying solid and cystic components caused by hemorrhagic degeneration. When lesions are entirely or partially cystic, especially for larger lesions, MRI can be more useful in depicting features that are helpful for the diagnosis, such as intra-tumoral hemorrhage and the peripheral enhancement pattern.

Solid Pseudopapillary Neoplasms

α-Methylacyl-CoA racemase (P504S) is a useful marker for the differential diagnosis of solid pseudopapillary neoplasm of the pancreas

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α-Methylacyl-CoA racemase (P504S) is a useful marker for the differential diagnosis of solid pseudopapillary neoplasm of the pancreas