Tongue-base hamartoma in tuberous sclerosis

Area of Science:

• Medical Case Reports
• Surgical Oncology
• Genetics and Rare Diseases

Background:

• Tuberous sclerosis is a genetic disorder causing benign tumors in various organs.
• Hamartomas can occur in the head and neck region, posing surgical challenges.
• Large tongue-base masses can compromise the airway, necessitating careful pre-operative planning.

Observation:

• A 41-year-old female with tuberous sclerosis presented with a significant tongue-base hamartoma.
• The patient also had a large thyroid goiter, complicating surgical access.
• Airway management involved awake fiber-optic intubation, thyroidectomy, and tracheostomy.

Findings:

• This is the first reported instance of a tongue-base hamartoma in a patient diagnosed with tuberous sclerosis.
• The surgical approach highlights the importance of addressing concurrent conditions like goiter before tumor resection.
• Successful resection of the tongue-base hamartoma was achieved after securing the airway.

Implications:

• This case expands the known clinical manifestations of tuberous sclerosis.
• It underscores the need for multidisciplinary management in complex head and neck tumor surgeries.
• Highlights the critical role of advanced airway techniques in managing patients with compromised respiratory pathways.