Current highlights on solid pseudopapillary neoplasm of the pancreas

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 most commonly due to mass effect. Diagnosis is made by computed tomography scan or magnetic resonance imaging and histological characterization is obtained by endoscopic ultrasound-guided fine needle biopsy. Microscopically, these lesions are composed by both solid and pseudopapillary structures with necrotic and hemorrhagic areas. Occasionally, the biological behavior is aggressive with tumor recurrence and distant metastasis. Usually, curative R0 surgical resection is the best option able to provide long term survival even in advanced disease. Unresectable disease is the main predictor of poor prognosis. Chemotherapy and radiotherapy regimens are not well standardized. However, they could be effective in reducing tumor size as neoadjuvant treatment or disease control in palliative setting. Although complete surgical resection provides a cure rate of > 95%, considering young age of the patients and morbidity associated to pancreatic surgery, further studies are needed to better investigate risk factors and responsiveness to hormones in order to allow early diagnosis and follow up strategies that could avoid unnecessary surgery in less aggressive disease.