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The nose is composed of an observable exterior segment (external nose) and an internal segment within the skull known as the nasal cavity (internal nose). The external nose, visible on the face, consists of a framework of bone and hyaline cartilage enveloped in skin and muscle and lined with a mucous membrane. This structure is supported by the frontal bone, nasal bones, and maxillary bone and is supplemented by a cartilaginous framework comprising the septal nasal cartilage, lateral nasal...
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Nasal Chondromesenchymal Hamartoma.

Balamurugan Thirunavukkarasu1, Debajyoti Chatterjee2, Satyawati Mohindra3

  • 1Department of Histopathology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Head and Neck Pathology
|May 29, 2020
PubMed
Summary

Nasal chondromesenchymal hamartoma (NCMH) is a rare sinonasal tumor. Recent findings link NCMH to DICER1 mutations, aiding diagnosis and treatment.

Keywords:
DICER1 proteinHamartomaNasal polyps

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Area of Science:

  • Pathology
  • Genetics
  • Pediatric Oncology

Background:

  • Nasal chondromesenchymal hamartoma (NCMH) is a rare, benign sinonasal tumor.
  • Typically presents as a polypoid mass in infants and children.
  • Requires imaging and endoscopy for surgical planning.

Observation:

  • NCMH histopathology reveals proliferating mesenchymal and cartilaginous elements.
  • Recent studies establish a genetic link between NCMH and DICER1 mutations.
  • This association aids in differentiating NCMH from other sinonasal lesions.

Findings:

  • The identification of DICER1 mutations provides a molecular basis for NCMH.
  • This genetic marker assists pathologists in accurate diagnosis.
  • Understanding the genetic underpinnings is crucial for NCMH management.

Implications:

  • Familiarity with NCMH and its genetic associations is vital for pathologists.
  • Accurate diagnosis prevents misclassification and unnecessary treatments.
  • Surgical excision remains the curative treatment for this benign lesion.