SpringerLink
Log in

Pediatric nasal chondromesenchymal hamartomas: a case series

  • Paediatric Neuroradiology
  • Published:
Neuroradiology Aims and scope Submit manuscript

Abstract

Purpose

Nasal chondromesenchymal hamartomas (NCMH) are rare, predominantly benign tumors of the sinonasal tract. The distinction from higher grade malignancy may be challenging based on imaging features alone. To increase the awareness of this entity among radiologists, we present a multi-institutional case series of pediatric NCMH patients showing the varied imaging presentation.

Methods

Descriptive assessment of imaging appearances of the lesions on computed tomography (CT) and magnetic resonance imaging (MRI) was performed. In addition, we reviewed demographic information, clinical data, results of genetic testing, management, and follow-up data.

Results

Our case series consisted of 10 patients, with a median age of 0.5 months. Intraorbital and intracranial extensions were both observed in two cases. Common CT findings included bony remodeling, calcifications, and bony erosions. MRI showed heterogeneous expansile lesion with predominantly hyperintense T2 signal and heterogenous post-contrast enhancement in the majority of cases. Most lesions exhibited increased diffusivity on diffusion weighted imaging and showed signal drop-out on susceptibility weighted images in the areas of calcifications. Genetic testing was conducted in 4 patients, revealing the presence of DICER1 pathogenic variant in three cases. Surgery was performed in all cases, with one recurrence in two cases and two recurrences in one case on follow-up.

Conclusion

NCMHs are predominantly benign tumors of the sinonasal tract, typically associated with DICER1 pathogenic variants and most commonly affecting pediatric population. They may mimic aggressive behavior on imaging; therefore, awareness of this pathology is important. MRI and CT have complementary roles in the diagnosis of this entity.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price includes VAT (Germany)

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Mason KA, Navaratnam A, Theodorakopoulou E, Chokkalingam PG (2015) Nasal chondromesenchymal hamartoma (NCMH): a systematic review of the literature with a new case report. J Otolaryngol Head Neck Surg 44(1):28

    Article  PubMed  PubMed Central  Google Scholar 

  2. Saunders TFC, Bruijnzeel H, Ahmed S, Paruleka M, McDermott AL (2022) Nasal chondromesenchymal hamartoma: an update. J Laryngol Otol 136(12):1140–1147

    Article  CAS  PubMed  Google Scholar 

  3. Johnson C, Nagaraj U, Esguerra J, Wasdahl D, Wurzbach D (2007) Nasal chondromesenchymal hamartoma: radiographic and histopathologic analysis of a rare pediatric tumor. Pediatr Radiol 37(1):101–104

    Article  PubMed  Google Scholar 

  4. Li Y, Yang QX, Tian XT, Li B, Li Z (2013) Malignant transformation of nasal chondromesenchymal hamartoma in adult: a case report and review of the literature. Histol Histopathol 28(3):337–344

    PubMed  Google Scholar 

  5. Kim JE, Kim HJ, Kim JH, Ko YH, Chung SK (2009) Nasal chondromesenchymal hamartoma: CT and MR imaging findings. Korean J Radiol 10(4):416–419

    Article  PubMed  PubMed Central  Google Scholar 

  6. Thirunavukkarasu B, Chatterjee D, Mohindra S, Dass Radotra B, Prashant SJ (2020) Nasal chondromesenchymal hamartoma. Head Neck Pathol 14(4):1041–1045

    Article  PubMed  PubMed Central  Google Scholar 

  7. Wang T, Li W, Wu X, Li Q, Cui Y, Chu C et al (2014) Nasal chondromesenchymal hamartoma in young children: CT and MRI findings and review of the literature. World J Surg Oncol 12:257

    Article  PubMed  PubMed Central  Google Scholar 

  8. Kitayama T, Akaki S, Hisazumi K, Yoshio K, Inoue D, Tajiri N et al (2019) An adult case of nasal chondromesenchymal hamartoma: imaging characteristics including diffusion-weighted images. Acta Med Okayama 73(6):529–532

    PubMed  Google Scholar 

  9. Finitsis S, Giavroglou C, Potsi S, Constantinidis I, Mpaltatzidis A, Rachovitsas D et al (2009) Nasal chondromesenchymal hamartoma in a child. Cardiovasc Intervent Radiol 32(3):593–597

    Article  PubMed  Google Scholar 

  10. Uzomefuna V, Glynn F, Russell J, McDermott M (2012) Nasal chondromesenchymal hamartoma with no nasal symptoms. BMJ Case Rep. 2012. https://doi.org/10.1136/bcr.11.2011.5148

  11. Silkiss RZ, Mudvari SS, Shetlar D (2007) Ophthalmologic presentation of nasal chondromesenchymal hamartoma in an infant. Ophthalmic Plast Reconstr Surg 23(3):243–244

    Article  PubMed  Google Scholar 

  12. Norman ES, Bergman S, Trupiano JK (2004) Nasal chondromesenchymal hamartoma: report of a case and review of the literature. Pediatr Dev Pathol 7(5):517–520

    Article  PubMed  Google Scholar 

  13. Nakaya M, Yoshihara S, Yoshitomi A, Baba S (2017) Endoscopic endonasal excision of nasal chondromesenchymal hamartoma with intracranial extension. Eur Ann Otorhinolaryngol Head Neck Dis 134(6):423–425

    Article  CAS  PubMed  Google Scholar 

  14. Moon SH, Kim MM (2014) Nasal chondromesenchymal hamartoma with incomitant esotropia in an infant: a case report. Can J Ophthalmol 49(1):e30–e32

    Article  PubMed  Google Scholar 

  15. Kim DW, Low W, Billman G, Wickersham J, Kearns D (1999) Chondroid hamartoma presenting as a neonatal nasal mass. Int J Pediatr Otorhinolaryngol 47(3):253–259

    Article  CAS  PubMed  Google Scholar 

  16. Kato K, Ijiri R, Tanaka Y, Hara M, Sekido K (1999) Nasal chondromesenchymal hamartoma of infancy: the first Japanese case report. Pathol Int 49(8):731–736

    Article  CAS  PubMed  Google Scholar 

  17. Behery RE, Bedrnicek J, Lazenby A, Nelson M, Grove J, Huang D et al (2012) Translocation t(12;17)(q24.1;q21) as the sole anomaly in a nasal chondromesenchymal hamartoma arising in a patient with pleuropulmonary blastoma. Pediatr Dev Pathol. 15(3):249–53

    Article  PubMed  Google Scholar 

  18. Alrawi M, McDermott M, Orr D, Russell J (2003) Nasal chondromesynchymal hamartoma presenting in an adolescent. Int J Pediatr Otorhinolaryngol 67(6):669–672

    Article  CAS  PubMed  Google Scholar 

  19. Yao-Lee A, Ryan M, Rajaram V (2011) Nasal chondromesenchymal hamartoma: correlation of typical MR. CT and pathological findings Pediatr Radiol 41(5):675–677

    Article  PubMed  Google Scholar 

  20. Cicek MT, Bayindir T, Aslan M, Sigirci A, Gunduz E (2022) A rare cause of respiratory distress in newborn: huge nasal chondromesenchymal hamartoma; patient Report. J Craniofac Surg 33(4):e411–e413

    Article  PubMed  Google Scholar 

  21. Avci H, Comoglu S, Ozturk E, Bilgic B, Kiyak OE (2016) Nasal chondromesenchymal hamartoma: a rare nasal benign tumor. Kulak Burun Bogaz Ihtis Derg 26(5):300–303

    Article  PubMed  Google Scholar 

  22. Unal A, Kum RO, Avci Y, Unal DT (2016) Nasal chondromesenchymal hamartoma, a rare pediatric tumor: Case report. Turk J Pediatr 58(2):208–211

    Article  PubMed  Google Scholar 

  23. Schaerer D, Nation J, Rennert RC, DeConde A, Levy ML (2021) Pediatric nasal chondromesenchymal tumors: case report and review of the literature. Pediatr Neurosurg 56(1):61–66

    Article  PubMed  Google Scholar 

  24. Lee CH, Park YH, Kim JY, Bae JH (2015) Nasal chondromesenchymal hamartoma causing sleep-disordered breathing in an infant. Int J Clin Exp Pathol 8(8):9643–9646

    PubMed  PubMed Central  Google Scholar 

  25. Kim B, Park SH, Min HS, Rhee JS, Wang KC (2004) Nasal chondromesenchymal hamartoma of infancy clinically mimicking meningoencephalocele. Pediatr Neurosurg 40(3):136–140

    Article  CAS  PubMed  Google Scholar 

  26. Stewart DR, Messinger Y, Williams GM, Yang J, Field A, Schultz KA et al (2014) Nasal chondromesenchymal hamartomas arise secondary to germline and somatic mutations of DICER1 in the pleuropulmonary blastoma tumor predisposition disorder. Hum Genet 133(11):1443–1450

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Funding

No funding, grants, or other support was received to assist with the preparation of this manuscript.

Author information

Authors and Affiliations

  1. Clinical Institute of Radiology, University Medical Centre Ljubljana, Zaloška Cesta 7, 1000, Ljubljana, Slovenia

    Jernej Avsenik

  2. Department of Radiology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia

    Jernej Avsenik

  3. College of Medicine, Alfaisal University, Riyadh, Saudi Arabia

    Ibrahem Albalkhi

  4. Division of Neuroradiology, Department of Radiology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA

    Sanjay P. Prabhu

  5. Department of Radiology and Imaging Sciences, Indiana University School of Medicine, Indianapolis, IN, USA

    Rupa Radhakrishnan

  6. Department of Medical Imaging, The Children’s Hospital at Westmead, New South Wales, Australia

    Robert Goetti

  7. Department of Medical Imaging, Ann and Robert H Lurie Children’s Hospital of Chicago, Chicago, IL, USA

    Alok Jaju

  8. Neuropathology Department, Great Ormond Street Hospital, London, UK

    Ashirwad Merve

  9. Department of Neuroradiology, Great Ormond Street Hospital, London, UK

    Asthik Biswas & Kshitij Mankad

Authors
  1. Jernej Avsenik
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Ibrahem Albalkhi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Sanjay P. Prabhu
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Rupa Radhakrishnan
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Robert Goetti
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Alok Jaju
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Ashirwad Merve
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Asthik Biswas
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Kshitij Mankad
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jernej Avsenik.

Ethics declarations

Conflict of interest

The authors report no conflicts of interests/competing interests. The authors have no relevant financial or non-financial interests to disclose.

Ethics approval

Research ethics board approval was obtained from the participating hospitals. This study was performed in line with the principles of the Declaration of Helsinki.

Informed consent

Retrospective study approved by REB with consent waiver; no patient identifying information used.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Avsenik, J., Albalkhi, I., Prabhu, S.P. et al. Pediatric nasal chondromesenchymal hamartomas: a case series. Neuroradiology 66, 437–441 (2024). https://doi.org/10.1007/s00234-023-03276-w

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00234-023-03276-w

Keywords

Search

Navigation