WOFAPS 2025 8th World Congress of Pediatric Surgery

Arije Zouaoui, Senda Houidi, Yosra Kerkeni, Skander Smaoui, Riadh Jouini

Pediatric Surgery Department, Children's Hospital Bechir Hamza, Tunis, Tunisia

Introduction:
Solid pseudopapillary neoplasm (SPN) of the pancreas, also known as Frantz tumor, is a rare pancreatic tumor of low malignant potential. It primarily affects young women around 25 years of age, with pediatric cases being exceptional. Radiologic imaging is essential for diagnosis. Surgical resection remains the cornerstone of the treatment and directly determines prognosis.

Case Presentation:
We report the case of a 9-year-old girl presenting to the emergency department with worsening paroxysmal abdominal pain evolving over one month, associated with food vomiting, without fever or other symptoms. Clinical examination revealed overweight status, significant abdominal tenderness in the epigastric and right hypochondriac regions, without palpable mass or jaundice. Laboratory investigations showed normal findings except for elevated serum amylase (8× normal) and transaminases (3× normal). Abdominal ultrasound followed by CT scan revealed a well-defined, 15 cm iso-dense pancreatic head mass with a discontinuous fibrous capsule and necrotic-hemorrhagic areas. The lesion showed heterogeneous contrast enhancement, without upstream Wirsung duct dilation or bile duct invasion. There was mass effect on the stomach and superior mesenteric vein, which remained patent. No lymphadenopathy or distant metastasis were detected. SPN was suspected. Surgical management involved a cephalic duodenopancreatectomy with uneventful postoperative recovery after a 2-years follow-up. Histopathological examination confirmed the diagnosis.

Conclusion:
Solid pseudopapillary neoplasm of the pancreas is a rare pediatric tumor with distinct clinicopathological features. Its excellent prognosis depends entirely on complete surgical excision. Early diagnosis and referral to specialized centers are essential to optimize outcomes.