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A rare case of symptomatic solid pseudopapillary neoplasm in a 23 year female.

Published onJun 16, 2023
A rare case of symptomatic solid pseudopapillary neoplasm in a 23 year female.

Aim: Establish the diagnosis of solid pseudopapillary neoplasm (SPN) in our resource limited country and compare the outcome of using advanced surgery (anterior RAMPS) with literature. 

Methods: A 23-year-old Zambian female presented with a 3-month history of dull abdominal pain, loss of appetite, and weight loss. Physical examination was performed. Laboratory workup included complete blood count, liver function test and enzymes, serum creatinine, blood urea, and electrolytes. Imaging studies included abdominal ultrasound, computerized tomography (CT) scan and ultrasound guided fine needle aspiration (for histology and immunohistochemistry).  

Results: Laboratory work up revealed leukopenia (white cell count= 3.7×10*9/L) while other parameters were normal. Abdominal ultrasound showed a left upper quadrant mass, confirmed on CT scan as a mass with cystic and solid areas. Histology showed cells arranged in a papillary architecture and immunohistochemistry was positive for beta catenin, CD 10, CD 56, SOX 11 and AE1-AE3 confirmed the diagnosis of solid pseudo papillary neoplasm. An anterior radical antegrade modular pancreato-splenectomy was performed, and the tumour of size 11×8×7cm (fig 1.1) located on the body and tail of the pancreas was resected and staged as T3N0M0. Postoperative phase was complicated by a grade B pancreatic fistula that was successfully managed and follow up at 33 days post-operative showed no evidence of the tumour on ultrasound. Patient was asymptomatic and well. 

Discussion: SPN clinical presentation is vague and non-specific. It is rare in Zambia, but our patient fits the most described presentation in the literature.  CT scan, histology and immunohistochemistry are most useful in diagnosis. Overall SPN cases have low malignancy transformation potential, however surgical intervention is warranted in cases impacted by clinical presentation as our patient.

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