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Background <p>Tuberous sclerosis complex (TSC) is a multisystem genetic disorder characterized by hamartoma formation. Craniofacial osseous hamartomas involving the paranasal sinuses are exceedingly rare.</p> Case Presentation <p> A five-year-old female presented in 2007 with progressive left-eye proptosis and diplopia due to an expansile $46 \times 53$ mm osseous lesion involving the left maxillary, ethmoid, and frontal sinuses. Diagnosis was confirmed via histopathology and a TSC1 mutation. The patient underwent complete surgical excision. At a 17-year post-operative reassessment in 2024, the patient remained asymptomatic with excellent cosmesis and no evidence of recurrence.</p> Conclusions <p>This case describes a rare triple-sinus hamartoma in TSC. Complete surgical excision may provide a durable functional and aesthetic outcome for symptomatic mature osseous lesions.</p>

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Maxillo-Ethmoido-Frontal Osseous Hamartoma in Tuberous Sclerosis Complex: A 17-Year Post-operative Reassessment

  • 期刊论文

  • 发表日期：

    2026年5月7日

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  • Dency Hansalia,
  • Akshay Kantha,
  • Paras Kumar,
  • Manoj Pandey

摘要

Background

Tuberous sclerosis complex (TSC) is a multisystem genetic disorder characterized by hamartoma formation. Craniofacial osseous hamartomas involving the paranasal sinuses are exceedingly rare.

Case Presentation

A five-year-old female presented in 2007 with progressive left-eye proptosis and diplopia due to an expansile $46 \times 53$ mm osseous lesion involving the left maxillary, ethmoid, and frontal sinuses. Diagnosis was confirmed via histopathology and a TSC1 mutation. The patient underwent complete surgical excision. At a 17-year post-operative reassessment in 2024, the patient remained asymptomatic with excellent cosmesis and no evidence of recurrence.

Conclusions

This case describes a rare triple-sinus hamartoma in TSC. Complete surgical excision may provide a durable functional and aesthetic outcome for symptomatic mature osseous lesions.