Nasal Chondromesenchymal Hamartoma of Infancy Clinically Mimicking Meningoencephalocele

Abstract

We report a typical case of nasal chondromesenchymal hamartoma (NCMH) of infancy, which extended to the orbit and intracranium through the skull base. The nasal mass was incidentally found in a 5-month-old otherwise healthy boy who had recently developed ptosis of the left eye. On neuroimaging, there were defects in the left ethmoid bone of the anterior cranial fossa and medial orbital wall. A round heterogeneous mass was found in the left nasal cavity. Solid portions were strongly enhanced. The preoperative diagnosis was nasal glioma or encephalocele. After frontal craniotomy, the mass was separated from the cranial basal dura. Through the transnasal approach, the mass was dissected from the left orbital content and removed. NCMH is a rare benign lesion and histopathological analogue of the mesenchymal hamartoma of the chest wall, having unique pathology: islands of hyaline cartilage, somewhat calcified or ossified hyalinized collagenous trabeculae and abundant myxoid stroma. Its occurrence in the nasal cavity was identified in 1998. The recognition of this entity is necessary to avoid misdiagnosis of the frozen section and for appropriate surgery.

Nasal chondromesenchymal hamartoma: a rare nasal benign tumor

“…[3][4][5] In the literature, NCMHs are invariably diagnosed in infants or in early childhood. [1,2,[6][7][8][9][10][11][12][13][14][15] Patients with NCMHs commonly present with an intranasal mass that causes symptoms such as nasal obstruction, rhinorrhea and epistaxis. The symptoms and clinical presentation of the patient are mostly determined by the size and location of tumor.…”

“…Proptosis, enophthalmos or impairment of eye movement can be the presenting symptoms or findings in cases with orbital involvement. [2,6,[9][10][11][12][13][14][16][17][18][19] Intracranial extension of the tumor can result in neurologic manifestations. [2,17] Symptoms or signs such as difficulties in respiration or nourishment, epistaxis, rhinorrhea, middle ear effusion can be encountered due to tumor size and site.…”

Endoscopy in the Evaluation and Management of the Pediatric Airway

“…This rare nasal mass, a chondromesencymal hamartoma, is a benign congenital lesion that has known propensity to have intracranial extension. This can mimic the appearance of a meningoencephalocele (Kim 2004).…”

Nasal chondromesenchymal hamartoma in children with pleuropulmonary blastoma—A report from the International Pleuropulmonary Blastoma Registry registry

“…Twenty-six NCMH cases have previously been reported, predominantly in infants [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]. NCMH has a mixed morphologic composition of immature cartilage surrounded by spindle cell stroma with or without fibro-osseous and aneurysmal bone cystlike features [1].…”

“…NCMH has a mixed morphologic composition of immature cartilage surrounded by spindle cell stroma with or without fibro-osseous and aneurysmal bone cystlike features [1]. NCMH may be expansile, locally destructive and extend into the intracranial space [1,3,4,6], leading to uncertainty about a hamartomatous versus neoplastic designation. Nasal chondromesenchymal hamartoma Pleuropulmonary blastoma Cancer predisposition PPB NCMH DICER1…”