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Solid Pseudopapillary Tumor : Clinical Features

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Pancreas_SolidPseudoPapillaryTumor47_Gross.jpg

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Epidemiology: Solid pseudopapillary tumor (SPT) of pancreas accounts for 1% to 2% of all pancreatic neoplasms; however, it makes up about 30% of all pancreatic tumors in patients below the age of 40. Almost 90% of cases arise in adolescent girls and young women (mean age: 28 years). It is rare in men (Female : Male ratio = 10:1) and in both sexes after age 50. About 20% of cases occur in the pediatric age group.

Clinical Features: The patients present with abdominal pain (65% to 80% of cases) or a large abdominal mass (35% of cases). The large size of the mass may cause abdominal discomfort, nausea, vomitting, early satiety, and weight loss. In rare instances, intratumoral hemorrhage or rupture, either spontaneously or following trauma, can cause acute abdomen.

There are no tumor markers or functional endocrine syndromes associated with this neoplasm. About 15% of cases are discovered incidentally on physical examination or on imaging studies performed after trauma or some other reason. Distant metastases are encountered at presentation in 5% to 10% of cases.

The photograph shows a distal pancreatectomy and splenectomy specimen with a large solid mass diagnosed as solid pseudopapillary tumor of pancreas. See cut surface of the tumor in the next slide. Courtesy of: Dr. Jean-Christophe Fournet, Paris, France; humpath.com; Used with permission

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