Aggressive Behavior and Intracranial Extension in Nasal Chondromesenchymal Hamartoma: Two Pediatric Cases and Literature Review

Abstract

Introduction/Background: Nasal chondromesenchymal hamartoma (NCMH) is a rare benign mesenchymal tumor that predominantly affects infants and young children. Although traditionally considered non-aggressive, selected cases demonstrate locally invasive behavior, including skull base erosion and intracranial extension.

Case Presentation: We report two pediatric cases of NCMH with aggressive clinical evolution. The first case involved a newborn presenting with respiratory distress due to a nasal mass. Despite initial surgical resection, early recurrence occurred within five months, with extension to the skull base requiring further partial resection. The second case involved a 9-year-old girl presenting with progressive visual loss, headache, strabismus, and cerebrospinal fluid rhinorrhea. Imaging revealed a large lesion involving the anterior skull base, suprasellar region, clivus, and paranasal sinuses. Following subtotal endoscopic endonasal resection, the patient developed multiple recurrences requiring repeated surgical interventions and evolved with sixth cranial nerve palsy.

Conclusions: NCMH may exhibit unexpectedly aggressive behavior despite its benign histopathology. These cases highlight the importance of achieving maximal safe resection, close long-term imaging surveillance, and a multidisciplinary management strategy, particularly in cases of incomplete resection.