Pancreatic solid pseudopapillary neoplasm in male patients: systematic review with three new cases
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REVIEW ARTICLE
Pancreatic solid pseudopapillary neoplasm in male patients: systematic review with three new cases Anna Caterina Milanetto1 · Anna‑Lea Gais Zürcher1 · Lorenzo Macchi1 · Alina David1 · Claudio Pasquali1 Received: 27 March 2020 / Accepted: 20 October 2020 © The Author(s) 2020
Abstract Pancreatic solid pseudopapillary neoplasm (pSPN) is a rare exocrine neoplasm, which generally occurs in young women. This study analyses the clinical characteristics of pSPN in male patients through a systematic review of the literature, adding three new cases from our institution. We reviewed our experience in Pspns, and we performed a systematic review of pSPN of all articles published in English in PubMed and SCOPUS from 1980. Using the final included articles, we evaluated clinicpathological features, surgical treatment and prognosis of male patients affected by pSPN. From the literature review and our cases, we collected 246 male patients with a proven pSPN. Mean age was 34.3 (range 4–78) years, with 26.2% patients younger than 18 years. Patients were asymptomatic in 35.9% of cases, despite a mean tumour size of 6.3 cm. In 63.7% of cases, the pSPN was located in the body–tail region. Distant metastases were reported at diagnosis in only 10 (4.1%) patients. A correct pre-operative diagnosis (including cytopathology) was provided in 53.6% of patients, with only 40 fine-needle aspiration/biopsy performed. Standard pancreatic resections represented 90.4% of surgical procedures. Beta-catenin and progesterone receptors were positive at immunostaining in 100% and 77.8% of cases, respectively. Fourteen (7.2%) patients relapsed after a mean disease-free survival of 43.1 months. After a mean follow-up of 47 (range 4–180) months, 89.5% of patients were alive and disease-free. Although rare, when dealing with a solid-cystic pancreatic mass, even in asymptomatic male patients, a pSPN should be considered as a possible diagnosis. Keywords Solid pseudopapillary neoplasm · Male patient · Pancreatic tumour · Pancreatic surgery
Introduction In 1959, the pathologist Virginia Kneeland Frantz firstly described a solid pseudopapillary neoplasm (SPN) of the pancreas in a 2-year-old male patient [1]. It is a low-grade malignant tumour lacking a specific line of pancreatic epithelial differentiation [2], accounting for 1–3% of all pancreatic tumours [3]. Solid pseudopapillary neoplasm is known for its strong female preponderance (F:M = 10:1); in fact, it affects women less than 30 years old in 85% of cases [4], Electronic supplementary material The online version of this article (https://doi.org/10.1007/s13304-020-00905-4) contains supplementary material, which is available to authorized users. * Anna Caterina Milanetto [email protected] 1
Clinica Chirurgica 1, Pancreatic and Endocrine Digestive Surgical Unit, Department of Surgery, Oncology and Gastroenterology, Università degli Studi di Padova, via Giustiniani, 2 ‑ 35128 Padua, Italy
while men are rarely affected. About 20–25% of SPNs occur in patients younger than 18 yea
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