Abstract
BackgroundNasal chondromesenchymal hamartoma (NCMH) is a very rare, benign tumour of the sinonasal tract usually presenting in infants. We present a systematic review of NCMH cases alongside a case report of an adult with asymptomatic NCMH.MethodsA systematic review was conducted in accordance with PRISMA guidelines. A PubMed, EMBASE and manual search through references of relevant publications was used to identify all published case-reports of NCMH. Data was collected from each case-report on: patient demographics, laterality, size and location of NCMH, presentation, co-morbidities, investigations, treatment and follow-up.ResultsThe systematic review identified 48 patients (including ours): 33 male, 15 female. Mean age was 9.6 years (range: 1 day–69 years) with the majority aged 1 year or younger at presentation (n = 18). Presentations included: nasal congestion (n = 17), nasal mass (n = 15) and eye signs (n = 12). NCMH also involved the paranasal sinuses (n = 26), orbit (n = 16) and skull-base (n = 14). All patients underwent operative resection of NCMH. A small 2014 case-series found DICER1 mutations in 6 NCMH patients, establishing a link to the DICER1 tumour spectrum.ConclusionsNCMH is a rare cause of nasal masses in young children and adults. In light of the newly established link between NCMH and DICER1 mutations surgeons should be vigilant for associated DICER1 tumours, as NCMH may be the ‘herald tumour’ of this disease spectrum.
Highlights
Nasal chondromesenchymal hamartoma (NCMH) is a very rare, benign tumour of the sinonasal tract usually presenting in infants
We present an unusual case of NCMH in an adult without nasal obstructive symptoms due to the anatomical location of the NCMH attached to the nasal septum
A systematic review of the literature has highlighted that presentation is mostly related to tumour location, with nasal mass, nasal obstruction and ophthalmic signs being the most common forms of presentation
Summary
Nasal chondromesenchymal hamartoma (NCMH) is a very rare, benign tumour of the sinonasal tract usually presenting in infants. Nasal chondromesenchymal hamartoma (NCMH) is a very rare, benign tumour of the sinonasal tract. NCMH patients present with symptoms that are dependent on the location of the tumour in the nasal cavity or paranasal sinuses and their compression of local structures. These symptoms range from nasal obstruction to visual impairment and facial and dental. Case report A 49-year-old man was referred to our outpatient clinic presenting with a small mass in his right nasal cavity. The patient did not complain of any symptoms but sought consultation as his wife was concerned by the cosmetic appearance of the mass
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