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symptomatic Solid Pseudopapillary Neoplasm of the PancreasERGA C. GLEESON,* THOMAS C. SMYRK,‡ and SURESH T. CHARI*

Division of Gastroenterology and Hepatology, ‡Department of Pathology, Mayo Clinic College of Medicine, Rochester, Minnesota

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26-year-old woman with an uneventful medical history wasnoted incidentally to have right upper-quadrant fullness on

hysical examination at her annual gynecologic evaluation. An ab-ominal ultrasound revealed a pancreatic head mass measuring 10.7

7.4 � 9.4 cm. Computerized tomography showed a well-demar-ated, inhomogeneous, low-density mass, in parts containing areas ofery low density and areas displaying well-enhanced solid componentsFigure A). There was no clinical evidence of pancreatic endocrine orxocrine insufficiency or abnormal liver function tests.

A pylorus-preserving pancreaticoduodenectomy was performed inonjunction with a cholecystectomy. Macroscopically, a well-encapsu-ated solid and cystic pseudopapillary pancreatic tumor with evidencef cystic hemorrhage measuring 10.5 � 9.0 � 8.5 cm in the pancreaticead was established (Figure B). Cholelithiasis with 33 mixed stoneslso was established. Microscopically the tumor infiltrated the adja-ent pancreatic parenchyma, without evidence of perineural or vascu-ar invasion. Lymph node examination was negative for malignancy.he immunohistochemical analysis for �-catenin, chromogranin, and

ynaptophysin showed that the tumor cells showed nuclear expres-ion for �-catenin consistent with the diagnosis (Figure C). The pa-ient will continue with postoperative surveillance to evaluate for localecurrence or distant metastases.

Solid pseudopapillary neoplasm, a rare pancreatic neoplasm ofncertain histogenesis with malignant potential, initially was identi-

ed by Franz in 1959, and typically is found in young women (mean

ge, 30 y). Abdominal discomfort is the most common presentingymptom with a median tumor size of 7 cm (range, 4–16 cm).1

esected neoplasms usually are well circumscribed, surrounded by abrous pseudocapsule, and may be almost entirely solid, varying tolmost completely cystic with evidence of hemorrhagic degeneration.olid pseudopapillary neoplasms are distinct genetically from pancre-tic adenocarcinoma and frequently display mutations within the-catenin gene.2 Progesterone receptor positivity has been shown inreater than 90% of resected specimens. Biologically the neoplasmsisplay low-grade malignant potential with metastatic disease identi-ed in 15%, which is related to vascular and perineural invasion.3 The

iver, peritoneum, and lymph nodes are the most common sites foretastatic disease. The role of neoadjuvant or adjuvant chemotherapy

nd radiotherapy is yet to be established.

References. Tipton SG, Smyrk TC, Sarr MG, et al. Malignant potential of solid pseu-

dopapillary neoplasm of the pancreas. Br J Surg 2006;93:733–737.. Salvia R, Bassi C, Festa L, et al. Clinical and biological behavior of

pancreatic solid pseudopapillary tumors: report on 31 consecutivepatients. J Surg Oncol 2007;95:304–310.

. Nishihara K, Nagoshi M, Tsuneyoshi M, et al. Papillary cystictumors of the pancreas. Assessment of their malignant potential.Cancer 1993;71:82–92.

© 2008 by the AGA Institute1542-3565/08/$34.00

doi:10.1016/j.cgh.2007.10.014

CLINICAL GASTROENTEROLOGY AND HEPATOLOGY 2008;6:xxii