Nasal Chondromesenchymal Hamartoma (NCMH): a systematic review of the literature with a new case report

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.

Imaging Review of New and Emerging Sinonasal Tumors and Tumor-like Entities from the Fourth Edition of theWorld Health Organization Classification of Head and Neck Tumors

“…There is a link to germline or somatic DICER1 gene mutations, which are also associated with pleuropulmonary blastoma tumor predisposition disorder. 57 Histology demonstrates nodules of hyaline cartilage within the stromal components of spindle cells. 58,59 The tumor is benign but locally aggressive, involving the paranasal sinuses, nasal cavities, and orbits.…”

Nasal Chondromesenchymal Hamartoma Masquerading As Malignant Paediatric Tumour

“…Mcdermott et al 6 was the first to recognise NCMH as a distinct disease entity in 1998 whereby they described a series of seven patients with a tumefactive process in the nasal cavity entending into paranasal sinuses and intracranial involvement 4,5 . According to the systemic review conducted by Mason et al 7 , vast majority of the cases that had been reported were predominantly involving the infants below 1-year-old, however, there had been up to seven cases which involved adult with the highest age of 69 7 . Although it is a benign lesion, a case of malignant transformation was reported by Li et al 8 in 2013.…”

“…Typically, NCMH presents with nasal obstruction, rhinorrhea, facial deformity and ophthalmic signs, which indicate the involvement of the NCMH along the nasal cavity,paranasal sinuses and around the orbits 4,7 . Ophthalmic signs include proptosis, strabismus and hypertelorism mainly due to the compression effect of the intranasal mass causing displacement of the eye globe 4,7,9 . Occasionally, patients may have intraoral symptoms due to the involvement of the oral cavity 7 .…”

“…Ophthalmic signs include proptosis, strabismus and hypertelorism mainly due to the compression effect of the intranasal mass causing displacement of the eye globe 4,7,9 . Occasionally, patients may have intraoral symptoms due to the involvement of the oral cavity 7 . Symptoms and signs of NCMH can be varied, and patient may present to the Ophthalmology or Oral Surgery clinics during their first visit instead of the Otorhinolaryngology (ORL) clinic.…”

Nasal chondromesenchymal hamartoma: a rare nasal benign tumor

“…Antrochoanal polyps have low signal intensity on T 1 sequences and high signal intensity on T 2 sequences, peripheral enhancement in post-contrast series as distinct from NCMHs. [23] Paranasal CT and MRI are two complementary radiologic modalities that should be undertaken before surgery. They can reveal the extent of tumor, site of origin, relationship with critical structures and help surgical planning.…”