Lomal Linda University Children's Hospital Redlands, CA
Award: Presidential Poster Award
Kalyan Ray Parashette, MD, MPH, FACG Lomal Linda University Children's Hospital, Redlands, CA
Introduction: Solid pseudopapillary tumor (SPT) is uncommon neoplasm of pancreas that mainly occurs in young females without notable symptoms. It is very rare in children.
Case Description/Methods: A 11 year old previously healthy girl presented with acute epigastric abdominal pain and non-bilious emesis for 4 days. She also had 2 pound weight loss, tactile fever, and cough. Family history was significant for colon cancer in paternal grand father and leukemia in maternal grand mother. Exam showed fullness in epigastric area. She had normal CBC, CMP, amylase, & lipase. She was positive for COVID 19. CT abdomen (CTA) showed 8.0 x 10.3 x 7.7 cm heterogeneously hypodense mass in pancreatic body. CEA, CA-125, and CA 19-9 were normal.
She underwent distal pancreatectomy and splenectomy. Gross pathology showed 7.9 x 7.2 x 6 cm focally lobulated, semi-firm mass with a peripheral fibrous capsule. The cut surface showed intervening fibrous septations, and 80% brown-red and soft (likely necrotic) and 20% pink-tan and papillary to granular (likely viable). Biopsy showed large solid-pseudopapillary tumor of the pancreas with multiple small nodules of soft tissue outside main tumor capsule (Figure 1). The diagnosis was supported by pattern of immunostaining (positive diffuse staining for CD10, with nuclear staining for progesterone receptors, and diffuse strong nuclear staining for beta-catenin with negative staining for keratins or neuroendocrine markers). There was concern for skip lesion due to multifocal soft tissue invasion adjacent to pancreas and small area of tumor adjacent to spleen.
About 2 months later, she presented with bowel obstruction requiring exploratory laparotomy and lysis of adhesions and serosal repairs. She has remained asymptomatic at 10 month follow up. Repeat CTA at 4 and 9 months showed no evidence of recurrence of mass.
Discussion: Solid pseudopapillary tumor (SPT) of the pancreas is extremely rare in children. It is usually localized in the head or tail of the pancreas. In our case, SPT was in the body of pancreas. SPT is mostly diagnosed incidentally on radiological exam. Our patient presented with acute epigastric pain and vomiting. Surgical resection is the only curative therapy with excellent prognosis. SPT has low-grade malignancy that can be locally aggressive, but incidence of metastasis is low. Close follow-up is recommended to diagnose local recurrence. Our patient didn’t have recurrent of SPT on follow up imaging.
Figure: Figure 1. Uniform tumor cells compartmentalized by delicate capillary scaffolding. Cells have uniform nuclei with only small nucleoli and scant mitotic activity. The cytoplasm is moderately abundant and ranges from clear and vacuolated to pink and granular. Reverse palisading present around some small vessels. Some areas show focal necrosis with cyst formation and degenerative changes results in pseudopapillary features.
Disclosures:
Kalyan Ray Parashette indicated no relevant financial relationships.
Kalyan Ray Parashette, MD, MPH, FACG. P1185 - Solid Pseudopapillary Tumor of the Pancreas: A Rare Cause of Acute Abdomen in Teenage Girl, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.
