What is Cylindroma?

Cylindroma is a benign adnexal tumour that typically arises from the sweat glands. Although it is non-cancerous, its appearance can mimic malignant lesions, necessitating accurate diagnosis. These tumours generally manifest as solitary or multiple nodules on the scalp or face.

Cylindroma and Genetic Predisposition

One intriguing aspect of cylindroma is its association with genetic conditions. Notably, multiple familial trichoepithelioma (MFT) and Brooke-Spiegler syndrome are linked to cylindromas. These syndromes are characterized by mutations in the CYLD gene, resulting in tumour formation.

  • Brooke-Spiegler Syndrome: This hereditary condition predisposes individuals to develop multiple skin tumours, including cylindromas, trichoepitheliomas, and spiradenomas.
  • Multiple Familial Trichoepithelioma (MFT): In this autosomal dominant disorder, affected individuals exhibit numerous trichoepitheliomas, with a potential for co-existing cylindromas.

Causes of Cylindroma

Cylindromas primarily emerge due to genetic mutations. The CYLD gene, a tumour suppressor gene, plays a pivotal role in skin tumour regulation. Mutations in this gene lead to unchecked cellular proliferation, culminating in tumour development.

Distinction: Cylindroma vs. Trichoepithelioma

Differentiating cylindroma from trichoepithelioma is a common diagnostic hurdle. While both are skin tumours associated with genetic syndromes, they differ histologically. Cylindromas exhibit a characteristic "jigsaw puzzle" pattern in histological examinations, whereas trichoepitheliomas have a more structured, nodular appearance.

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Symptoms of Cylindroma

Cylindromas are typically asymptomatic, presenting as flesh-coloured, dome-shaped nodules. These lesions commonly appear on the scalp, hence the colloquial term "turban tumor" when they are numerous. While they are mostly benign, their location can cause discomfort or cosmetic concerns.

Potential Complications

Although benign, cylindromas can occasionally transform into malignant lesions, particularly if left untreated. Regular monitoring and histological examination are recommended for early detection of malignant changes.


Diagnosing Cylindroma

Accurate diagnosis of cylindroma necessitates a combination of clinical evaluation and histopathological analysis. Dermatologists often employ biopsy procedures to ascertain the tumour's nature.

  • Biopsy: A skin biopsy reveals the tumour's histological features, confirming the diagnosis. The "jigsaw puzzle" pattern is a hallmark of cylindroma.
  • Genetic Testing: In cases with a familial history, genetic testing for CYLD mutations can be informative, offering insights into hereditary predispositions.

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Treatment Options for Cylindroma

While cylindromas are benign, treatment is often pursued for cosmetic reasons or due to potential complications. Several therapeutic options are available, each with distinct advantages.

Surgical Excision

Surgical removal remains the definitive treatment for cylindromas. It ensures complete excision, minimizing recurrence risks. Depending on the tumour's size and location, different surgical techniques may be employed.

  • Simple Excision: Suitable for solitary, small tumors.
  • Mohs Micrographic Surgery: Preferred for larger or facial tumours, this technique minimizes tissue loss while ensuring complete tumour removal.

Non-Surgical Treatments

For patients contraindicated for surgery, non-surgical interventions may be considered. These include:

  • Cryotherapy: Utilizes extreme cold to destroy tumour cells. Suitable for small lesions.
  • Laser Therapy: Effective for superficial tumours, laser treatment offers a less invasive option.

Pharmacological Interventions

Emerging therapies targeting genetic pathways offer promising alternatives. Inhibitors targeting the defective pathways in CYLD mutations are under investigation, potentially offering non-invasive management options in the future.


Cylindroma and Skin Tumors: Broader Context

Cylindromas, while rare, are part of a broader spectrum of skin adnexal tumours. Understanding their relationship with genetic disorders enhances our comprehension of skin tumour pathogenesis.

Cylindroma in Clinical Practice

For clinicians, recognizing the signs and genetic implications of cylindroma is crucial for effective patient management. Regular follow-ups, patient education, and family counseling form the cornerstone of managing hereditary skin tumour syndromes.

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Frequently Asked Questions

1. What are the symptoms of cylindroma?

Symptoms may include painless, dome-shaped nodules on the skin, often found on the scalp or forehead.

2. What causes cylindroma?

Causes are typically linked to genetic factors and may be associated with familial cylindromatosis.

3. How is cylindroma diagnosed?

Diagnosis typically involves clinical evaluation, skin biopsy, and histopathological examination to confirm the tumor type.

4. What treatment options are available for cylindroma?

Treatment may involve surgical removal of the tumor for cosmetic reasons or to prevent complications.

5. How does cylindroma relate to skin tumors?

It is a type of benign skin tumor composed of glandular cells, differing from other skin tumors in histological appearance and behavior.

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