Cytological Diagnosis of Pancreatic Solid-Pseudopapillary Neoplasm: A Single-Institution Community Practice Experience

Abstract

Introduction. Pancreatic solid-pseudopapillary neoplasm (SPN) is a rare tumor that typically occurs in young females. Although a cytological diagnosis may be easily made in this age group when there are typical features, atypical clinical presentations and unusual cytological features may make this a challenging diagnosis. We present our single-institution experience in a cohort of these tumors, outlining both typical and atypical features. Awareness of unusual clinical and cytological features can help to avoid pitfalls during diagnosis. Methods. We performed a review of all cases of pancreatic SPNs diagnosed over a 15-year period (January 2007 to December 2021). Detailed cytological, clinical, and follow-up histological features were presented and analyzed. Results. Twenty-two cases of SPN were diagnosed at our institution during this 15-year period. Patients ranged from 12 to 73 years of age (mean 33 y, median 26 y) and included 19 females and 3 males. Seventeen patients had cytological material, and fourteen were diagnosed by EUS-FNA. Typical cytological features included papillary clusters with central capillaries, myxoid stroma, monomorphism, cercariform cells, and hyaline globules. Atypical or unusual cytological features that were seen in a few cases were multinucleated giant cells, clear cells, and/or foamy macrophages. A few cases showed features that were similar to pancreatic neuroendocrine tumors (PanNETs). Tumor cells were always positive for β-catenin, CD10, CD56, cyclin-D1, progesterone receptor (PR), and vimentin by immunohistochemistry. They were always negative for chromogranin. Pancytokeratin and synaptophysin stains were positive in 9% and 46% of cases evaluated, respectively. All cases had histological confirmation on resection. The median follow-up duration was 69 months (a range of 2–177 months), with only three cases lost to follow-up. No recurrence or metastasis was identified. Conclusions. We present our experience with cytological diagnoses of SPN in a well-characterized cohort of 22 patients with histological correlation and follow-up data. These tumors occur over a wide range and show varied cytological features. SPNs can be confidently diagnosed on limited cytological material, with limited panel immunohistochemistry aiding diagnosis in atypical cases. Recognizing the associated degenerative changes is crucial in avoiding a misdiagnosis.

Role of endoscopic ultrasound-guided fine needle aspiration biopsies in diagnosing pancreatic neoplasms in the paediatric population: experience from a tertiary center and review of the literature

“… 24 – 25 There is however an exception, where a few large pleomorphic atypical multinucleated giant cells could be intermingled with more typical ones, which was not found in our cases. 25 …”

“…[24][25] There is however an exception, where a few large pleomorphic atypical multinucleated giant cells could be intermingled with more typical ones, which was not found in our cases. 25 Main cytopathologic differential diagnosis of SPN in children depends on the age, comprising neuroendocrine neoplasms in patients older than 10 years and a pancreatoblastoma in younger patients. 3 In comparison to neuroendocrine neoplasms, SPNs have hyaline-mucinous stroma, cytoplasmic vacuoles, nuclei with grooves and negative reactions to chromogranin and cytokeratin.…”

“…In the cytopathological specimens, SPN comprises pseudopapillary clusters, acinar groups and single cells, delicate capillaries and globules of amorphous myxoid material which was well presented in case 1, but not in case 2 which was mainly necrotic. [24][25] Histomorphologically and cytomorphologically, the tumour is most commonly composed of unimorphic cells, with sparse to moderately abundant cytoplasm, which may be pale or oncocytic, with vacuoles and round to oval nuclei, with grooves and a fine chromatin structure, which also presented in our cases. [24][25] There is however an exception, where a few large pleomorphic atypical multinucleated giant cells could be intermingled with more typical ones, which was not found in our cases.…”

“…[24][25] Histomorphologically and cytomorphologically, the tumour is most commonly composed of unimorphic cells, with sparse to moderately abundant cytoplasm, which may be pale or oncocytic, with vacuoles and round to oval nuclei, with grooves and a fine chromatin structure, which also presented in our cases. [24][25] There is however an exception, where a few large pleomorphic atypical multinucleated giant cells could be intermingled with more typical ones, which was not found in our cases. 25 Main cytopathologic differential diagnosis of SPN in children depends on the age, comprising neuroendocrine neoplasms in patients older than 10 years and a pancreatoblastoma in younger patients.…”

Case report of peritoneal carcinomatosis in the clinical course of pancreatic solid pseudopapillary neoplasm (Frantz's tumor) in a patient with a history of blunt abdominal trauma

“…The histological characteristics of Frantz's tumor include the findings of solid areas of loose tissue forming pseudo-papillae with cystic areas containing hemorrhagic foci. Immunohistochemical findings of markers such as beta-catenin, CD10, CD56, cyclin-1 and progesterone receptors are common [ 10 ].…”