Presenter Status

Fellow

Abstract Type

Case report

Primary Mentor

Jason Brown, DO

Start Date

4-5-2022 11:30 AM

End Date

4-5-2022 1:30 PM

Presentation Type

Poster Presentation

Description

Background: Nasal chondromesenchymal hamartoma (NCMH) is a rare benign tumor of the sinonasal cavity. It commonly presents in infancy, however there are reported cases of adult tumors. As of August 2021, there have been 56 reported cases in the literature. This is the first description of a solely endoscopically resected NCMH in a neonate in addition to a review of the literature.

Case Presentation: A 5-day old male patient born at term presented to our institution for stridor and cyanosis since birth. The patient was reported to have desaturations while feeding at the outside hospital. On bedside examination, the patient was found to have a large obstructive lesion in his right nasal cavity. Magnetic resonance imaging of the face and sinuses showed a heterogenous mass in the right nasal cavity measuring 2.6x1.5x1.9 cm. The mass was displacing the nasal septum to the left, however there was no frank invasion into surrounding structures. A subsequent computed tomography showed internal calcifications within the mass. The cribriform plate and orbital wall were intact. The patient underwent complete endoscopic surgical excision of the mass. Pathological examination showed chondromesenchymal hamartoma.

Discussion: Nasal chondromesenchymal hamartoma is a benign lesion of the sinonasal cavity that was first described by McDermott et al. in 1998. The lesion is rare with only 56 reported cases in the literature. Although it more commonly presents in infants, there are reported cases in adult patients. The average age of presentation is 9.6 years with a male to female ratio of 2.2:1. The neoplasm commonly arises from the nasal septum or the nasal vestibule, however, there are reported cases arising from various parts of the nasal cavity. The lesion is usually not invasive however it can cause mass effect and destruction of surrounding structures. In the present case, the tumor emanated from the medial wall of the right middle turbinate. Patients typically present with nasal obstruction however they may also present with ocular or neurologic symptoms. Histologically, NCMH shows hyaline cartilage and mesenchymal cells in a myxoid background. Malignant transformation of the lesion is unlikely with only one reported case in the literature. Depending on the location of the mass, complete surgical excision is necessary to avoid recurrence. In our case, complete surgical resection was performed through an endoscopic endonasal approach in a neonate. To our knowledge, this has not been reported before.

MeSH Keywords

chondromesenchymal hamartoma; pediatric; nasal mass

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May 4th, 11:30 AM May 4th, 1:30 PM

Nasal Chondromesenchymal Hamartoma: A Case Report and Review of the Literature

Background: Nasal chondromesenchymal hamartoma (NCMH) is a rare benign tumor of the sinonasal cavity. It commonly presents in infancy, however there are reported cases of adult tumors. As of August 2021, there have been 56 reported cases in the literature. This is the first description of a solely endoscopically resected NCMH in a neonate in addition to a review of the literature.

Case Presentation: A 5-day old male patient born at term presented to our institution for stridor and cyanosis since birth. The patient was reported to have desaturations while feeding at the outside hospital. On bedside examination, the patient was found to have a large obstructive lesion in his right nasal cavity. Magnetic resonance imaging of the face and sinuses showed a heterogenous mass in the right nasal cavity measuring 2.6x1.5x1.9 cm. The mass was displacing the nasal septum to the left, however there was no frank invasion into surrounding structures. A subsequent computed tomography showed internal calcifications within the mass. The cribriform plate and orbital wall were intact. The patient underwent complete endoscopic surgical excision of the mass. Pathological examination showed chondromesenchymal hamartoma.

Discussion: Nasal chondromesenchymal hamartoma is a benign lesion of the sinonasal cavity that was first described by McDermott et al. in 1998. The lesion is rare with only 56 reported cases in the literature. Although it more commonly presents in infants, there are reported cases in adult patients. The average age of presentation is 9.6 years with a male to female ratio of 2.2:1. The neoplasm commonly arises from the nasal septum or the nasal vestibule, however, there are reported cases arising from various parts of the nasal cavity. The lesion is usually not invasive however it can cause mass effect and destruction of surrounding structures. In the present case, the tumor emanated from the medial wall of the right middle turbinate. Patients typically present with nasal obstruction however they may also present with ocular or neurologic symptoms. Histologically, NCMH shows hyaline cartilage and mesenchymal cells in a myxoid background. Malignant transformation of the lesion is unlikely with only one reported case in the literature. Depending on the location of the mass, complete surgical excision is necessary to avoid recurrence. In our case, complete surgical resection was performed through an endoscopic endonasal approach in a neonate. To our knowledge, this has not been reported before.

 

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