Aggressive pediatric solid pseudopapillary neoplasms of the pancreas presented as acute abdomen: A case report and review of literature

Table 1 Pediatric SPN with invasive malignant features

Ref.	Age (year-old)	Sex	Symptoms	Tumor size (cm)	Location	Liver metastasis	local pancreatic invasion	Histological features	IHC	Treatment	Prognosis alive
Nunes et al[5], 2026	11	Female	Acute abdominal pain, fever, nausea, and vomiting	6.8	Tail	No	Yes	Extensive necrosis; cholesterol clefts	Ki-67 < 2%, β-catenin (+), CD56(+), CD10(+), Cyclin D1(+)	SPDP	No recurrence (follow-up duration unspecified); yes
Gehlot and Sethna[6], 2025	Early adolescence	Female	Abdominal pain for 4 months, weight loss, occasional fever	12.1	Tail	VIII, IVa	No	Characteristic pseudopapillary structures; eosinophilic cytoplasm; extensive hemorrhagic necrosis; cholesterol clefts	Ki-67 < 2%, Syn(-), CgA(-)	Resection of pancreatic and liver tumors	No recurrence at 4-month follow-up; yes
Hu et al[23], 2024	17	Female	Trauma-induced abdominal pain	11	Anterior pancreas	Multiple	No	NA	β-catenin(+), Vimentin(+), CD10(+), Ki-67 approximately 5%, Syn (partial+), CgA (-)	Initial pancreatic tumor resection + partial hepatectomy; recurrence at 3 months, RFA	No recurrence at 43-month follow-up; yes
Sumida et al[24], 2007	14	Female	Abdominal pain for 4 days	8.5	Tail	Multiple	No	NA	PR(+)	Initial DPS; liver metastasis enlarged at 3 months, LT	No recurrence at 24-month follow-up; yes
Hassan et al[13], 2005	15	Female	Abdominal pain	14	Head	II, IV, V, VI, VII	No	Gross: Cystic-solid components with partial hemorrhage; microscopic: Characteristic pseudopapillary structures, focal necrosis and hemorrhage, cholesterol deposition	Vimentin(+), ACT(+), AAT(+), NSE(+), PR(+), Ki-67 < 10%, CgA(-)	Initial PD + PVR + RH; liver metastasis enlarged at 12 months, liver metastasectomy	No recurrence at 6-month follow-up; yes

SPDP: Spleen-preserving distal pancreatectomy; RFA: Radiofrequency ablation; DPS: Distal pancreatectomy with splenectomy; LT: Liver transplantation; PD: Pancreatoduodenectomy; PVR: Portal vein reconstruction; RH: Right Hepatectomy; CgA: Chromogranin A; NA: Not available; PR: Progesterone receptor; IHC: Immunohistochemistry; ACT: Alpha-1-antichymotrypsin; AAT: Alpha-1-antitrypsin; NSE: Neuron-specific enolase; Syn: Synaptophysin.