Overview
A proliferating trichilemmal cyst (also called a proliferating trichilemmal tumor or proliferating pilar cyst) is a rare growth that develops from the outer root sheath of a hair follicle, most often on the scalp. It starts as a slow-growing lump under the skin that can look and feel like a simple cyst at first. Over time, it may grow larger and, in some cases, can change into a more aggressive form that behaves like a skin cancer. Most of these growths are benign (not cancerous), but a small number can become malignant (cancerous), which is why doctors take them seriously. The condition most commonly affects middle-aged to older women, and the scalp is by far the most frequent location. The lump may be painless for a long time, but it can become tender, ulcerate (break open), or bleed as it grows. Because it can look similar to other skin tumors, a tissue biopsy is needed to confirm the diagnosis. Treatment usually involves surgical removal of the entire growth. When caught early and fully removed, the outlook is generally very good. However, if the tumor has developed cancerous features or has spread, more aggressive treatment such as wider surgery or radiation therapy may be needed. Regular follow-up after surgery is important to watch for any return of the growth.
Key symptoms:
A slow-growing lump or bump on the scalpFirm or rubbery texture under the skinPainless swelling that may gradually get largerSkin over the lump may become red or irritatedThe lump may open up, ulcerate, or bleedTenderness or soreness around the growthHair loss over the affected areaThe lump may feel attached to deeper tissue as it growsOccasional discharge or crusting from the surface
Clinical phenotype terms (3)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Proliferating trichilemmal cyst.
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Specialists
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
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Questions for your doctor
Bring these to your next appointment
- Q1.Is my tumor benign, borderline, or malignant, and what does that mean for my treatment?,Were the surgical margins clear, and is there any chance the tumor was not fully removed?,How often do I need follow-up appointments, and what should I watch for between visits?,Do I need any additional treatment such as radiation after surgery?,What are the chances this tumor could come back, and what would treatment look like if it does?,Should any of my family members be checked for similar growths?,Are there any clinical trials or specialist centers I should know about for this condition?
Common questions about Proliferating trichilemmal cyst
What is Proliferating trichilemmal cyst?
A proliferating trichilemmal cyst (also called a proliferating trichilemmal tumor or proliferating pilar cyst) is a rare growth that develops from the outer root sheath of a hair follicle, most often on the scalp. It starts as a slow-growing lump under the skin that can look and feel like a simple cyst at first. Over time, it may grow larger and, in some cases, can change into a more aggressive form that behaves like a skin cancer. Most of these growths are benign (not cancerous), but a small number can become malignant (cancerous), which is why doctors take them seriously. The condition most
How is Proliferating trichilemmal cyst inherited?
Proliferating trichilemmal cyst follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Proliferating trichilemmal cyst typically begin?
Typical onset of Proliferating trichilemmal cyst is adult. Age of onset can vary across affected individuals.