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

Mason KA, Navaratnam A, Theodorakopoulou E, Chokkalingam PG - J Otolaryngol Head Neck Surg (2015)

Bottom Line: 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).NCMH is a rare cause of nasal masses in young children and adults.

View Article: PubMed Central - PubMed

Affiliation: Barts and The London School of Medicine and Dentistry, The Blizard Institute of Cell and Molecular Science, 4 Newark Street, Whitechapel, E1 2AT, London, UK. katrina.a.mason@gmail.com.

ABSTRACT

Background: Nasal 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.

Methods: A 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.

Results: The 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.

Conclusions: NCMH 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.

No MeSH data available.


Related in: MedlinePlus

Nodule containing cartilage and aneurysmal bone and covered by stratified squamous epithelium with keratinisation (magnification ×25, haematoxylin & eosin stain)
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Fig1: Nodule containing cartilage and aneurysmal bone and covered by stratified squamous epithelium with keratinisation (magnification ×25, haematoxylin & eosin stain)

Mentions: The patient subsequently underwent excisional biopsy of the right nostril mass under general anaesthetic using a circumferential subperichondrial incision with a small margin. Intraoperatively, the mass had the macroscopic appearance of a 0.5 cm × 2 cm × 2 cm calcified nodule. Due to a small 0.5 cm base, subsequent healing was achieved by secondary intention aided by the routine application of topical antibacterial cream. Histopathological analysis showed the nodule to contain cartilage and aneurysmal bone covered in stratified squamous epithelium with keratinisation (Figs. 1 and 2). Histopathological diagnosis was made using a haematoxylin & eosin stain. These findings were consistent with a diagnosis of a nasal chondromesenchymal hamartoma.Fig. 1


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

Mason KA, Navaratnam A, Theodorakopoulou E, Chokkalingam PG - J Otolaryngol Head Neck Surg (2015)

Nodule containing cartilage and aneurysmal bone and covered by stratified squamous epithelium with keratinisation (magnification ×25, haematoxylin & eosin stain)
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4495949&req=5

Fig1: Nodule containing cartilage and aneurysmal bone and covered by stratified squamous epithelium with keratinisation (magnification ×25, haematoxylin & eosin stain)
Mentions: The patient subsequently underwent excisional biopsy of the right nostril mass under general anaesthetic using a circumferential subperichondrial incision with a small margin. Intraoperatively, the mass had the macroscopic appearance of a 0.5 cm × 2 cm × 2 cm calcified nodule. Due to a small 0.5 cm base, subsequent healing was achieved by secondary intention aided by the routine application of topical antibacterial cream. Histopathological analysis showed the nodule to contain cartilage and aneurysmal bone covered in stratified squamous epithelium with keratinisation (Figs. 1 and 2). Histopathological diagnosis was made using a haematoxylin & eosin stain. These findings were consistent with a diagnosis of a nasal chondromesenchymal hamartoma.Fig. 1

Bottom Line: 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).NCMH is a rare cause of nasal masses in young children and adults.

View Article: PubMed Central - PubMed

Affiliation: Barts and The London School of Medicine and Dentistry, The Blizard Institute of Cell and Molecular Science, 4 Newark Street, Whitechapel, E1 2AT, London, UK. katrina.a.mason@gmail.com.

ABSTRACT

Background: Nasal 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.

Methods: A 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.

Results: The 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.

Conclusions: NCMH 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.

No MeSH data available.


Related in: MedlinePlus