A systematic review of penile peripheral nerve-sheath tumors: From diagnosis to management

Penile peripheral nerve sheath tumors (PNSTs) — including schwannomas, neurofibromas, and malignant peripheral nerve sheath tumors (MPNSTs) — are exceedingly rare and often clinically underrecognized. This systematic review analyzes 71 published cases to characterize their demographic profiles, clinical presentations, imaging findings, histopathological features, and treatment strategies. Benign tumors, particularly schwannomas, typically present in adults as painless, slow-growing, well-circumscribed masses. In contrast, neurofibromas and MPNSTs are more frequently observed in children and are often associated with neurofibromatosis type 1. Imaging modalities such as ultrasound and magnetic resonance imaging are useful for localization but lack diagnostic specificity. Histopathological analysis remains the gold standard for definitive diagnosis. Surgical excision is the primary treatment approach, with an emphasis on preserving neurovascular function when feasible. In cases of MPNSTs and certain high-risk or recurrent tumors, adjuvant therapies — such as radiotherapy, chemotherapy, and Gamma Knife radiosurgery — have been utilized, yielding variable clinical outcomes. Additionally, targeted therapies are emerging as promising options for unresectable or syndromic tumors. Heightened clinical awareness and a multidisciplinary approach are critical for optimizing outcomes and minimizing diagnostic delays. This review highlights key diagnostic clues and treatment principles to guide clinicians in managing these rare lesions.