Nasal Chondromesenchymal Hamartoma with Skull Base and Orbital Involvement: Case Presentation

Abstract

Nasal chondromesenchymal hamartoma (NCMH) is a rare benign tumor of the sinonasal tract in children with possible orbit and skull base involvement. We present the 57th published observation of this kind of tumor. A 25-month-old female patient presented with recurrent mass lesion of the sinonasal tract. According to her history, she had feeding difficulties and nasal obstruction since birth. She underwent partial resection at eight months of age via transfacial approach in the local hospital. Due to progression of tumor remnants, a second surgery was performed using an endoscopic endonasal approach resulting in subtotal resection. At 12 months of follow-up, a good postoperative result was observed with no signs of tumor progression despite incomplete resection. Histological and immunohistochemical examination of the biopsy specimens is presented. Comparison of specimens obtained from each of the two surgeries showed a difference in histological patterns. Endoscopic endonasal approach is the mainstay of surgical management. In case of incomplete resection, careful follow-up MRI studies should be recommended.

Seltene Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis

“…Nasennebenhöhlen, Nasenhaupthöhle und Orbita können betroffen sein. Ausdehnungen entlang der Schädelbasis und nach intrakraniell sind möglich [209,210].…”

“…The tumor is rare and mostly occurs in children, rarely also in adolescents and adults with a slight predilection of the male gender. In the current literature currently 60 cases have been published [209].…”

“…Paranasal sinuses, nasal cavity, and orbita may be affected. Extension along the skull base and in intracranial direction are possible [209,210].…”

Morphological diagnosis of chondroid tumors of skull bones: аctual situation

“…При малом объеме биоптата, содержащего только хрящевой компонент, дифференциальную диагностику с хондромой позволяет провести возраст пациента. Существуют данные о том, что хондромезенхимальная гамартома может входить в спектр поражений с наличием мутации гена DICER [11,12].…”

Multidisciplinary approach to children with sinonasal tumors: A review

“…Complete tumor resection will ensure a favourable outcome. Nasal chondromesenchymal hamartoma is a rare sinonasal benign tumor in children that may present with orbital and skull base involvement . Endonasal endoscopic resection is also the mainstay of treatment for this tumor.…”