A Rare Aggressive Primary Hepatic Neoplasm: A Case Report of a Malignant Peripheral Nerve Sheath Tumor of the Liver and Literature Review

Primary malignant peripheral nerve sheath tumors (MPNST) of the liver are extremely rare. We report the case of a 77-year-old male with primary hepatic MPNST which demonstrated an aggressive clinical course with rapid growth after initial presentation and early recurrence with innumerable bilobar liver metastases, as well as abdominal soft tissue masses along the right paracolic gutter, within 3 months of treatment with complete radical surgical resection. The diagnosis of MPNST can often be challenging and relies not only on classic histomorphologic features, but also on immunohistochemical stains (such as S-100 positivity and complete loss of trimethylation of the lysine 27 residue of histone 3, H3K27me3). Surgery remains the mainstay of therapy for resectable tumors. Systemic doxorubicin-based therapy, such as doxorubicin plus olaratumab, is to be considered for unresectable or metastatic disease. Overall, primary hepatic MPNST have an aggressive clinical course with rapid growth, recurrence, metastasis and poor prognosis with little long-term follow-up data in the literature.