Malignant Peripheral Nerve Sheath Tumor of the Pancreas: A Case Report and Updated Review of Related Literature

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas with poor prognosis due to their high recurrence rates. The prevalence of MPNST in the general population is 0.001%, with tumors arising from the retroperitoneum accounting for only 1% of all MPNSTs. In this report, we present a case of a 59-year-old male with pancreatic MPNST. To the authors’ knowledge, this is the first documented case of pancreatic MPNST in the Philippines. 

The patient initially presented with a 3-month history of abdominal pain, weight loss, and anorexia. On abdominal computed tomography (CT) scan, a large cystic mass involving the pancreatic head and body, with an enhancing peripheral solid component in the superior region was seen. The patient underwent distal pancreatectomy, en bloc splenectomy and excision of duodenal cyst. Post-operative histopathology and immunohistochemistry staining were consistent with pancreatic MPNST with tumor very near the margin of resection adjacent to the portal vein. Adjuvant systemic chemotherapy and radiotherapy were not performed due to lack of evidence of benefit over risk for this population. Disease recurrence (nodal-peritoneal metastases) was noted six months post-operatively and he was given palliative chemotherapy with single-agent doxorubicin. However, disease progression was noted after five cycles of chemotherapy. Second-line regimen was planned but the patient died of a pulmonary embolism prior to the initiation of chemotherapy.

Due to the rarity and highly aggressive nature of MPNSTs, furthering knowledge on these tumors is important, particularly in their inclusion among the differential diagnoses for pancreatic tumors. Prompt diagnosis and histopathologic confirmation by a pathologist specializing in sarcomas are crucial in the treatment planning and prognostication of these tumors. Lastly, further studies are needed to establish more effective treatments in unresectable or metastatic disease.