Malignant Cylindroma, from the CureRays guide library.

Malignant cylindroma is a rare skin-appendage cancer that usually arises from a long-standing benign scalp tumor; treatment is complete surgical removal, often with radiation, and people with the inherited CYLD syndrome need genetic counseling and monitoring.

What it is.

Malignant cylindroma (cylindrocarcinoma) is a very rare cancer of the skin's sweat-gland (adnexal) structures. It most often develops on the scalp, head, or neck, typically arising within a pre-existing benign cylindroma — a slow-growing nodule sometimes called a 'turban tumor' when many cluster on the scalp. A warning sign of malignant change is a long-standing benign nodule that suddenly grows quickly, ulcerates, bleeds, or becomes painful. Many cases are linked to an inherited condition called CYLD cutaneous syndrome (also known as Brooke-Spiegler syndrome), caused by mutations in the CYLD gene, in which people develop multiple skin-appendage tumors over time and have a higher risk of one turning malignant. Malignant cylindroma can invade locally and, less commonly, spread to lymph nodes or distant organs, so it is more serious than its benign counterpart. The mainstay of treatment is complete surgical removal with clear margins, often using margin-controlled techniques. Radiation is frequently added after surgery for high-grade tumors, close or positive margins, or nerve invasion, and is used as the primary treatment when surgery is not possible. Because of the inherited link, genetic counseling and long-term skin and family monitoring are important.

How radiation treats it.

Radiation damages the DNA inside cancer cells so they can no longer divide and survive, while healthy cells repair themselves more effectively. In malignant cylindroma, radiation is most often given after surgery to lower the chance the cancer returns — especially when the tumor is high-grade, the margins are close or involved, or it has invaded along nerves, in which case the radiation field is shaped to follow those nerve pathways. When a tumor on the scalp or face cannot be fully removed without unacceptable disfigurement, radiation can serve as the primary treatment. Because these cancers sit on the scalp near the skull and brain, the dose is carefully shaped using modern techniques. Radiation is given as short, painless daily sessions and leaves no radioactivity in your body, so you remain safe to be around family and children.

The ways we can treat it.

Surgery. Wide local excision or margin-controlled (Mohs) surgery removes the tumor with a cuff of healthy tissue; reconstruction may be needed on the scalp or face.

External-beam radiation. Focused radiation after surgery treats the tumor bed and, for nerve-invading tumors, the nerve pathways, improving local control while shaping the dose around the skull and brain.

Systemic therapy (selective). For the uncommon cases that spread, systemic treatment is individualized; research into targeted approaches reflecting CYLD biology is ongoing.

Questions we hear often.

How do I know if a long-standing scalp bump has turned cancerous? Warning signs include a benign nodule that suddenly grows quickly, ulcerates, bleeds, becomes painful, or changes in appearance. Any such change in a long-standing scalp or skin lump should be evaluated, and a biopsy confirms whether malignant transformation has occurred.

Is malignant cylindroma inherited? Often it is linked to an inherited condition — CYLD cutaneous syndrome (Brooke-Spiegler syndrome) — caused by mutations in the CYLD gene, in which people develop multiple skin-appendage tumors and have a higher risk of one becoming malignant. Genetic counseling and family screening are recommended when this is suspected.

Will I need radiation after surgery? Sometimes. Radiation is added when the tumor is high-grade, the margins are close or involved, or the cancer has invaded along nerves — features that raise the chance of return. For a small, completely removed low-grade tumor, surgery alone may be enough. Your team will tailor the plan to your tumor.

This guide is informational only. It is not medical advice — please confirm anything here with your care team.