Current highlights on solid pseudopapillary neoplasm of the pancreas

Abstract

Solid pseudopapillary neoplasm of the pancreas is a low-grade malignant tumor that predominantly affects young women in their third and fourth decade.Etiology and risk factors are unknown. Clinical symptoms are aspecific and mostcommonly due to mass effect. Diagnosis is made by computed tomography scanor magnetic resonance imaging and histological characterization is obtained byendoscopic ultrasound-guided fine needle biopsy. Microscopically, these lesionsare composed by both solid and pseudopapillary structures with necrotic andhemorrhagic areas. Occasionally, the biological behavior is aggressive with tumorrecurrence and distant metastasis. Usually, curative R0 surgical resection is thebest option able to provide long term survival even in advanced disease.Unresectable disease is the main predictor of poor prognosis. Chemotherapy andradiotherapy regimens are not well standardized. However, they could beeffective in reducing tumor size as neoadjuvant treatment or disease control inpalliative setting. Although complete surgical resection provides a cure rate of >95%, considering young age of the patients and morbidity associated to pancreaticsurgery, further studies are needed to better investigate risk factors and responsivenessto hormones in order to allow early diagnosis and follow up strategiesthat could avoid unnecessary surgery in less aggressive disease