Background: Solid pseudopapillary tumors (SPT) of the pancreas are rare, low malignancy and predominantly affect young women, but it may be locally aggressive. Pancreatic resection is the main treatment for SPTs. However, low malignancy SPT may give insidious extrapancreatic invasions.
Case report: A 20-year-old woman was admitted with non-specific abdominal pain and diarrhea. A 9-cm SPT of the pancreas was discovered with extra-pancreatic invasion of the left adrenal gland and the spleen, in close contact with the body-tail of the pancreas, proximal portion of the jejunum, splenic flexure of the colon and the gastric fundus, with no signs of infiltration. For the young patient, a pancreas-preserving tumor excision was performed, with en-bloc resection of the spleen and adrenal gland, lymphadenectomy of the splenic vessels (13 lymph-nodes) and pre-pancreatic lymph node dissection, with no need for distal pancreatectomy. The duration of the surgery was 145 min, with no transfusion. The woman's postoperative course was complicated by a splenic lodge abscess treated via CT-guided percutaneous drainage, and a left pleural effusion treated medically, with a hospital stay of 16 days. Histology confirmed the diagnosis of a 9- cm low-grade SPT of the pancreas. In a close follow-up, the patient was asymptomatic 21 months later, with no tumor recurrence and good health.
Conclusions: Low-grade SPT of the pancreas with extra-pancreatic invasion of loco-regional organs can be treated by a pancreas-preserving approach to avoid a pancreatectomy. Moreover, still few cases of extra-pancreatic SPT are reported in the literature and there is an urgent need for more relevant evidence.
Key words: Extrapancreatic, Frantz's tumor, Pancreas preserving, Pancreas, Pancreatic neoplasm, Solid pseudopapillary tumor, Solid pancreatic tumor.
I tumori solidi pseudopapillari (SPT) del pancreas sono rari, si presentano come una voluminosa massa addominale, a bassa malignità e colpiscono prevalentemente le giovani donne. Tuttavia, possono essere localmente aggressivi. Gli SPT rappresentano meno del 2% delle neoplasie pancreatiche esocrine. Le caratteristiche cliniche e patologiche dell’ SPT sono diverse dal cancro del pancreas; la patogenesi del tumore non è chiara e la derivazione cellulare è ancora da determinare. La presentazione clinica del tumore è aspecifica. L’intervento chirurgico è il trattamento principale per gli SPT pancreatici: pancreatectomia distale, pancreatectomia distale con conservazione della milza (Warshaw/Kimura) e duodenocefalopancreatectomia (Whipple o Longmire) seguite dalla resezione locale ed enucleazione sono le procedure più comuni. Ci proponiamo di illustrare un raro caso di SPT del pancreas con localizzazione extra-pancreatica ed invasione di organi loco-regionale in una giovane donna di 20 anni, trattata con una chirurgia pancreas-preserving evitando la pancreasectomia, in combinazione con uno stretto follow-up. Inoltre, il nostro scopo è di proporre una revisione della letteratura, includendo tutti i casi disponibili in letteratura di SPT extra-pancreatico istologicamente confermato.