A 16-year-old girl presented in the emergency department with acute on-set generalized non-radiating abdominal pain, relieved by lateral decubitus, accompanied with nausea, bilious vomits and anorexia. She was on oral contraceptive and had no relevant medical or family history. Besides epigastric pain her physical examination was unremarkable. Laboratory studies only revealed slightly elevation of amilase (167 UI/L; range 0 – 100 U/L). Abdominal ultrasound was performed showing a 20 x 10 mm non-vascularized lesion, with hydric central part, in the pancreatic head. For further characterization, the patient underwent an endoscopic ultrasound (EUS) which demonstrated a 29 mm well-delimitated cephalic lesion, with solid and cystic areas but no calcifications. There was no vascular involvement (Figure 1).
Figure 1: Linear EUS-FNA showing a mixed (solid and cystic) lesion in the cephalic region of the pancreas
Fine-needle aspiration (FNA) was performed with a 22-gauge needle through the duodenal bulb, in a single pass (because of narrow sedation window). The abdominal magnetic resonance imaging (MRI) findings were consistent to those of the EUS, both suggesting solid pseudopappilary tumor of the pancreatic cephalic region. The cytological examination was suggestive of papillary neoplasia (Figure 2).
Figure 2: Cytological analysis showing papillary clusters of epithelial cells. A – Giemsa stain. B – Papanicolaou stain.
The patient underwent surgery for enucleation of the tumor. Intraoperative ultrasound and exploration confirmed no vascular invasion or metastasis. Histopathological analysis revealed a 1.7 cm solid pseudopapillary pancreatic tumor, pT1N0, with necrohemorrhagic central area surrounded by fibrous tissue with many histiocytes. The cells stained positive for beta-catenin, vimentin and CD10, and negative for chromogranin A.
No further treatment was offered to the patient and she was maintained under surveillance.
Solid pseudopappilary neoplasms (SPN) are uncommon lesions accounting for 0.7%-2.7% of all pancreatic tumors (1,2). They affect predominantly women in their 2nd to 3rd decades of life (3). Most cases are found incidentally with the widespread use of imaging techniques but the most frequent symptom is abdominal pain, followed by nausea, vomiting and weight loss (4).
Laboratory findings including tumor markers are often unremarkable. The diagnosis is usually made on cross-section imaging in the presence of a well-defined mass with central areas of calcifications, hemorrhage, necrosis or cystic degeneration (5). EUS features include encapsulated mass with solid or mixed (solid and cystic) content, and calcifications in up to 20% of cases. Performing EUS-FNA allows for preoperative histological diagnosis in 75% of cases, especially from solid material (6); the characteristic cytological findings are branching pappilae with fibroid stroma staining positive for vimentin, CD10 and beta-catenin (1).
SPN usually have an indolent course but given the malignant potential, and when symptomatic, patients should be treated with surgical resection regardless of the disease stage (3). The surgery-related mortality is low (<2%) and even in the presence of metastatic disease the prognosis of SPN is favorable, with 5-year survival rate of 95% after tumor resection (1). Despite the absence of specific guidelines, surveillance should be offered for at least 5 years since the mean time for postoperative recurrence is 4 years.
1. Jana T., Shroff J., Bhutani M. Pancreatic cystic neoplasms: Review of current knowledge, diagnostic challenges, and management options. J Carcinog. 2015; 14: 3. doi: 10.4103/1477-3163.153285.
2. Ren Z., Zhang P., Zhang X., Liu B. Solid pseudopapillary neoplasms of the pancreas: clinicopathologic features and surgical treatment of 19 cases. Int J Clin Exp Pathol 2014;7(10):6889-6897.
3. Khalid A, Brugge WR. ACG practice guidelines for the diagnosis and management of neoplastic pancreatic cysts. Am J Gastroenterol 2007; 102:2339.
4. Romics L Jr, Oláh A, Belágyi T, Hajdú N, Gyurus P, Ruszinkó V. Solid pseudopapillary neoplasm of the pancreas–proposed algorithms for diagnosis and surgical treatment. Langenbecks Arch Surg 2010; 395:747.
5. Dan D., Rambally R., Cawich S., Maharaj R., Naraynsingh V. Solid Pseudopapillary Neoplasms of the Pancreas: A Report of Two Cases. Hindawi Publishing Corporation – Case Reports in Medicine. Volume 2014, Article ID 356379, 5 pages. doi: 10.1155/2014/356379.
6. Fasanella KE, McGrath K. Cystic lesions and intraductal neoplasms of the pancreas. Best Pract Res Clin Gastroenterol. 2009;23(1):35.
Sara Pires1, Pedro Pinto-Marques2, Hélder Coelho3, Maria José Brito3, António Folgado4
1 Gastroenterology department, Hospital Espírito Santo, Évora, Portugal
2 Gastroenterology department, Hospital Garcia de Orta, Lisbon, Portugal
3 Pathology department, Hospital Garcia de Orta, Lisbon, Portugal
4 General Surgery department, Hospital Garcia de Orta, Lisbon, Portugal