Solid pseudopapillary epithelial neoplasm of the pancreas with liver metastasis

A girl in early adolescence presented with complaints of abdominal pain lasting for 4 months, along with a palpable lump in the epigastric region. A CT scan revealed a large solid-cystic mass lesion measuring 9.5×10.7×12.1 cm, involving the body and tail of the pancreas. Additionally, well-defined hypodense lesions in the liver, localised to segments VIII and IVa, were observed, with the aetiology being indeterminate. An endoscopic ultrasound-guided biopsy of the pancreatic mass confirmed a diagnosis of solid pseudopapillary epithelial neoplasm (SPEN). Ultrasonography (USG) of the liver suggested liver metastasis. Surgical excision of the pancreatic mass and complete excision of the liver metastases were performed with the aid of intraoperative USG. No other sites of metastasis were identified. Liver surface masses, measuring 4×3 cm and 5×3 cm in segments VIII and IVa, respectively, were excised, along with a 13×10×6 cm mass in the pancreatic body and tail. Microscopic examination revealed tumour cells arranged in pseudopapillary and solid patterns. The cells were round to oval with eosinophilic cytoplasm, and areas of necrosis, hyalinisation, cholesterol clefts and calcification were identified, consistent with SPEN. The liver lesions were confirmed as metastases of SPEN. All surgical margins were clear, and the three lymph nodes examined were free of tumour involvement. Immunohistochemistry was negative for synaptophysin and chromogranin, supporting the diagnosis of SPEN. Less than 2% of the tumour cells were positive for Ki-67. A final diagnosis of SPEN of the pancreas with liver metastases was established. The patient is currently on routine follow-up and remains disease-free 4 months postsurgery.