Related Experiment Video
Updated: Jul 16, 2026
09:53
Role of Diffusion MRI Tractography in Endoscopic Endonasal Skull Base Surgery
Published on: July 5, 2021
[Familial presentation of multiple scalp tumors]
A Novo-Torres1, C Laredo-Ortiz, E Castellar-Nájera
1Especialista de Cirugía Plástica Reparadora, Hospital General Universitario de Alicante, Alicante, España. ash_novo@yahoo.com
Actas Dermo-Sifiliograficas
|April 3, 2007
Summary
Familial cylindromatosis syndrome, a rare genetic condition, was diagnosed in a woman and two of her daughters presenting with progressively growing scalp tumors. Surgical excision and histological findings confirmed the presence of cylindromas, highlighting the importance of family history in diagnosis.
Area of Science:
- Dermatology
- Oncology
- Genetics
Background:
- Familial cylindromatosis syndrome is a rare genodermatosis characterized by the development of multiple cylindromas, benign skin tumors.
- These tumors typically arise on the scalp and face, often presenting in adulthood.
- Early diagnosis and management are crucial to prevent disfigurement and potential complications.
Observation:
- A 74-year-old woman presented with a progressively growing, firm, reddish tumor with telangiectases in the left preauricular region.
- She had a 15-year history of using a wig to conceal scalp lesions.
- Physical examination revealed extensive scalp and facial tumors.
Findings:
- Surgical excision of the scalp tumors was performed, with defect reconstruction using partial-thickness grafts.
- Suspicion of familial cylindromatosis syndrome led to the examination of her six children.
- Two daughters were found to have scalp tumors, and histological examination confirmed the diagnosis of cylindromas in all affected individuals.
Implications:
- This case underscores the importance of considering hereditary conditions like familial cylindromatosis syndrome in patients with multiple or recurrent skin tumors.
- Genetic counseling and screening of family members are essential for early detection and management.
- Prompt diagnosis and treatment can improve patient outcomes and quality of life by addressing both the cosmetic and potential functional aspects of the tumors.
