Rare aggressive solid pseudopapillary neoplasm of the ovary with metastatic disease following surgical resection

Extra-pancreatic solid pseudopapillary neoplasms (SPNs) are rare tumours with an overall favourable prognosis and low malignant potential. SPNs with metastatic spread, distant lymph node metastasis and extrapancreatic origin are exceedingly rare. Significant controversy regarding the treatment and the management of metastatic disease exists and, currently, there are no standardised guidelines or treatment recommendations for the use of adjuvant therapy. In this case report, the authors present a patient with widely metastatic SPN of likely ovarian origin with the invasion of the inguinal lymph nodes and multiple abdominal metastatic deposits. Using the currently available literature, the authors discuss treatment options for metastatic SPN of the ovary and highlight the need for continued research in this important field.