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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

Four-phase flow diagram of systematic review in accordance with PRISMA guidelines * Two individual publications of single case-reports were excluded from analysis as these had previously been published, Schultz et al. [2] and Kang et al. [3].** See Table 1
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Fig3: Four-phase flow diagram of systematic review in accordance with PRISMA guidelines * Two individual publications of single case-reports were excluded from analysis as these had previously been published, Schultz et al. [2] and Kang et al. [3].** See Table 1

Mentions: A systematic review was undertaken in accordance with PRISMA guidelines [1]. No systematic review protocol was used, however our systematic review methodology is described below and a four-phase flow diagram is represented in Fig. 3. All published case-reports of NCMH were included in the review. A PubMed search (MEDLINE) (1975 to May Week 2, 2015) was carried out using the following terms [(chondromesenchymal hamartoma) AND (nasal OR sinus OR maxillary OR ethmoid OR sphenoid OR frontal OR orbit OR cranial)]. An EMBASE search (1975 to May Week 2, 2015) was carried out using a best sensitivity-combination strategy. The PubMed search resulted in 32 citations of which 24 were relevant, 6 were not NCMH case-reports, one was a Chinese language case-report, and one case was a duplicate case-report publication [2]. An EMBASE search and a manual search through references of relevant publications yielded 11 further relevant citations. Of these only 6 were included in the analysis; one case was found to have been a duplicate case report [3, 4] and four other possible cases of NCMH were found through publication citation search, but were labelled as “Mesenchymal chondrosarcoma” [5] “nasal hamartoma” [6], “nasopharyngeal hamartoma” [7], and “congenital mesenchymoma” [8], and were therefore not included. Thirty-one publications that report 47 cases of NCMH were included in this systematic review. Data was collected on patient demographics (age, gender), laterality, size and site of NCMH, presentation, co-morbidities, investigations, treatment and follow-up. These were also the principle summary measures. Two authors performed the database search, the manual search through references of relevant publications, and extracted the relevant data from the case-reports. Data was entered into an Excel 2013 Microsoft Office™ database which was used to carry out basic statistical analysis.Fig. 3


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)

Four-phase flow diagram of systematic review in accordance with PRISMA guidelines * Two individual publications of single case-reports were excluded from analysis as these had previously been published, Schultz et al. [2] and Kang et al. [3].** See Table 1
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig3: Four-phase flow diagram of systematic review in accordance with PRISMA guidelines * Two individual publications of single case-reports were excluded from analysis as these had previously been published, Schultz et al. [2] and Kang et al. [3].** See Table 1
Mentions: A systematic review was undertaken in accordance with PRISMA guidelines [1]. No systematic review protocol was used, however our systematic review methodology is described below and a four-phase flow diagram is represented in Fig. 3. All published case-reports of NCMH were included in the review. A PubMed search (MEDLINE) (1975 to May Week 2, 2015) was carried out using the following terms [(chondromesenchymal hamartoma) AND (nasal OR sinus OR maxillary OR ethmoid OR sphenoid OR frontal OR orbit OR cranial)]. An EMBASE search (1975 to May Week 2, 2015) was carried out using a best sensitivity-combination strategy. The PubMed search resulted in 32 citations of which 24 were relevant, 6 were not NCMH case-reports, one was a Chinese language case-report, and one case was a duplicate case-report publication [2]. An EMBASE search and a manual search through references of relevant publications yielded 11 further relevant citations. Of these only 6 were included in the analysis; one case was found to have been a duplicate case report [3, 4] and four other possible cases of NCMH were found through publication citation search, but were labelled as “Mesenchymal chondrosarcoma” [5] “nasal hamartoma” [6], “nasopharyngeal hamartoma” [7], and “congenital mesenchymoma” [8], and were therefore not included. Thirty-one publications that report 47 cases of NCMH were included in this systematic review. Data was collected on patient demographics (age, gender), laterality, size and site of NCMH, presentation, co-morbidities, investigations, treatment and follow-up. These were also the principle summary measures. Two authors performed the database search, the manual search through references of relevant publications, and extracted the relevant data from the case-reports. Data was entered into an Excel 2013 Microsoft Office™ database which was used to carry out basic statistical analysis.Fig. 3

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