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.
Subject(s)
Hamartoma/diagnosis , Nose Diseases/diagnosis , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , DEAD-box RNA Helicases/genetics , Diagnosis, Differential , Female , Hamartoma/genetics , Hamartoma/pathology , Humans , Infant , Infant, Newborn , Male , Middle Aged , Mutation , Nose Diseases/genetics , Nose Diseases/pathology , Nose Diseases/surgery , Paranasal Sinus Diseases/diagnosis , Paranasal Sinus Diseases/genetics , Paranasal Sinus Diseases/pathology , Paranasal Sinus Diseases/surgery , Ribonuclease III/genetics , Young AdultABSTRACT
A fistula of the anterior table of the frontal sinus is an uncommon complication of acute frontal sinus abscess. Various methods to close a frontocutaneous fistula have been described. We present the second reported transfistula endoscopically assisted primary closure of a frontocutaneous fistula, which eliminated the need for major surgical intervention. We also review the etiology, pathogenesis, diagnosis, and management of frontocutaneous fistula.
