Pleomorphic solid pseudopapillary neoplasm of the pancreas: degenerative change rather than high-grade malignant potential

Abstract

Solid pseudopapillary neoplasms (SPNs) are rare tumors of the pancreas characterized by poorly cohesive uniform cells with solid and pseudopapillary growth patterns. Nuclear pleomorphism is not a well-recognized feature of SPNs and may complicate differentiation from other pancreatic neoplasms. We compared histologic, immunohistochemical, and clinical features of 18 pleomorphic SPNs with 121 conventional SPNs. The prevalence of pleomorphic SPN was 12.9% (18/139). Pleomorphic SPNs arose in older patients (median, 45 years versus 32 years; P < .001), but no differences were found in sex, tumor location, recurrence, and metastasis when compared with conventional SPNs. Except for pleomorphic nuclei, other cytologic and histologic features of pleomorphic SPNs, such as growth pattern, tumor size, infiltrative pattern, tumor extension, mitosis, and Ki-67 labeling index, were not different from those of conventional SPNs. Pleomorphic SPNs showed a significantly higher p53 protein expression (64.7% [11/17 cases]) than that of conventional SPNs (1.8% [2/112 cases], P < .001). However, immunoreactivity for β-catenin and E-cadherin was not different between pleomorphic and conventional SPNs. A TP53 gene mutation was observed in 2 of 3 p53-immunoreactive pleomorphic SPNs. In summary, nuclear pleomorphism occurs in a subset of SPNs. They are more often p53 immunoreactive than SPNs without pleomorphism, and some harbor TP53 gene mutations. However, pleomorphic SPNs do not appear to be more aggressive than conventional SPNs. Low mitotic rate and Ki-67 labeling index may suggest nuclear pleomorphisms as degenerative changes. Recognition of typical poorly cohesive tumor cells and immunohistochemical features could establish the correct diagnosis of SPNs.