Nasal Chondromesenchymal Hamartoma masquerading as malignant paediatric tumour

Nasal Chondromesenchymal Hamartoma (NCMH) is a rare, benign tumour of sinonasal tract usually presents in infants. Even though it is benign in origin, its aggressive clinical behaviour and florid radiological finding always mislead the initial diagnosis, mimicking a malignant sinonasal tumour. We re...

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Main Authors: Tan, Sui Teng, Abu Bakar, Saraiza, Abdul Wahab, Abdul Fattah, Mohd Nor, Khadijah
Format: Article
Language:English
Published: Logos Medical Publishing 2019
Online Access:http://psasir.upm.edu.my/id/eprint/81456/
http://psasir.upm.edu.my/id/eprint/81456/1/NASAL.pdf
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author Tan, Sui Teng
Abu Bakar, Saraiza
Abdul Wahab, Abdul Fattah
Mohd Nor, Khadijah
author_facet Tan, Sui Teng
Abu Bakar, Saraiza
Abdul Wahab, Abdul Fattah
Mohd Nor, Khadijah
author_sort Tan, Sui Teng
building UPM Institutional Repository
collection Online Access
description Nasal Chondromesenchymal Hamartoma (NCMH) is a rare, benign tumour of sinonasal tract usually presents in infants. Even though it is benign in origin, its aggressive clinical behaviour and florid radiological finding always mislead the initial diagnosis, mimicking a malignant sinonasal tumour. We report a case of NCMH in a 2-year-old boy who was referred to by an ophthalmologist for progressive facial distortion, left eye proptosis, persistent left rhinorrhoea and epiphora. Biopsy from the nasal cavity which appeared to be lobulated mass on endoscopy examination confirmed it was Nasal Chondromesenchymal Hamartoma (NCMH). Surgical excision of the tumour was performed. The symptoms of NCMH depend on the tumour location, thus the clinical presentation can be varied. The only way to establish the diagnosis of NCMH is by histopathology and immunohistochemistry of the tumour. Complete surgical treatment is advocated once the diagnosis is confirmed. This benign tumour has an established relation with the DICER1 mutations, hence other DICER1 tumours need to be ruled out at the time of diagnosis.
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spelling upm-814562021-06-13T14:20:45Z http://psasir.upm.edu.my/id/eprint/81456/ Nasal Chondromesenchymal Hamartoma masquerading as malignant paediatric tumour Tan, Sui Teng Abu Bakar, Saraiza Abdul Wahab, Abdul Fattah Mohd Nor, Khadijah Nasal Chondromesenchymal Hamartoma (NCMH) is a rare, benign tumour of sinonasal tract usually presents in infants. Even though it is benign in origin, its aggressive clinical behaviour and florid radiological finding always mislead the initial diagnosis, mimicking a malignant sinonasal tumour. We report a case of NCMH in a 2-year-old boy who was referred to by an ophthalmologist for progressive facial distortion, left eye proptosis, persistent left rhinorrhoea and epiphora. Biopsy from the nasal cavity which appeared to be lobulated mass on endoscopy examination confirmed it was Nasal Chondromesenchymal Hamartoma (NCMH). Surgical excision of the tumour was performed. The symptoms of NCMH depend on the tumour location, thus the clinical presentation can be varied. The only way to establish the diagnosis of NCMH is by histopathology and immunohistochemistry of the tumour. Complete surgical treatment is advocated once the diagnosis is confirmed. This benign tumour has an established relation with the DICER1 mutations, hence other DICER1 tumours need to be ruled out at the time of diagnosis. Logos Medical Publishing 2019 Article PeerReviewed text en http://psasir.upm.edu.my/id/eprint/81456/1/NASAL.pdf Tan, Sui Teng and Abu Bakar, Saraiza and Abdul Wahab, Abdul Fattah and Mohd Nor, Khadijah (2019) Nasal Chondromesenchymal Hamartoma masquerading as malignant paediatric tumour. Medeniyet Medical Journal, 34 (2). pp. 223-228. ISSN 2149-2042; ESSN: 2149-4606 https://medeniyetmedicaljournal.org/jvi.aspx?pdir=medeniyet&plng=eng&un=MEDJ-73555 10.5222/MMJ.2019.73555
spellingShingle Tan, Sui Teng
Abu Bakar, Saraiza
Abdul Wahab, Abdul Fattah
Mohd Nor, Khadijah
Nasal Chondromesenchymal Hamartoma masquerading as malignant paediatric tumour
title Nasal Chondromesenchymal Hamartoma masquerading as malignant paediatric tumour
title_full Nasal Chondromesenchymal Hamartoma masquerading as malignant paediatric tumour
title_fullStr Nasal Chondromesenchymal Hamartoma masquerading as malignant paediatric tumour
title_full_unstemmed Nasal Chondromesenchymal Hamartoma masquerading as malignant paediatric tumour
title_short Nasal Chondromesenchymal Hamartoma masquerading as malignant paediatric tumour
title_sort nasal chondromesenchymal hamartoma masquerading as malignant paediatric tumour
url http://psasir.upm.edu.my/id/eprint/81456/
http://psasir.upm.edu.my/id/eprint/81456/
http://psasir.upm.edu.my/id/eprint/81456/
http://psasir.upm.edu.my/id/eprint/81456/1/NASAL.pdf