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HLRCC: skin lumps, fibroids and an inherited kidney cancer risk | CION Cancer Clinics
HLRCC is an inherited condition caused by a fault in the FH gene. It can cause small firm lumps in the skin, fibroids in the womb that appear young and in large numbers, and in some people a fast-growing kind of kidney cancer. The kidney risk is why it matters. This page explains the condition, who should suspect it, and why kidney scans start early. At CION Cancer Clinics, our oncologists plan screening and care for families with an inherited cancer syndrome, explained in plain words.
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Medically reviewed by Dr. Naresh GunduConsultant Medical Oncologist · MBBS, DNB (Internal Medicine), DM (Medical Oncology, AIIMS) · last reviewed September 2026, next review due September 2027
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The short answer
What is HLRCC?
HLRCC stands for hereditary leiomyomatosis and renal cell cancer. It is an inherited condition caused by a fault in the FH gene, and it is now also called FH tumour predisposition syndrome. It can cause small firm lumps in the skin, fibroids in the womb, and in some people a fast-growing type of kidney cancer.
Three features, not always together
Some carriers have skin lumps and nothing else. Some women have fibroids that appear young and in large numbers. A smaller group develop kidney cancer, sometimes as the very first sign in the family. Two people in the same family, carrying the same fault, can look completely different.
How it passes down a family
HLRCC is inherited in a dominant way. A parent who carries the fault has a one in two chance of passing it to each child, son or daughter. It does not skip generations, although a carrier with very few signs can make it look as if it has. Men carry and pass it on too, and they can develop the skin lumps and the kidney cancer, even though fibroids affect only women.
Why the kidney part is taken so seriously
Most carriers never develop kidney cancer. But when it does appear, the kind linked to HLRCC can spread even while it is still small. That is very different from the kidney tumours in most other inherited conditions, which are often watched until they grow. It is the reason HLRCC kidney scans start young and happen regularly, and the reason any tumour found is removed promptly.
A fault in the FH gene is a statement about risk, not a diagnosis of cancer.What it can cause
The signs of HLRCC, one by one
Each feature on its own is common and usually not inherited. It is the combination, or an unusual pattern, that raises the question.
Skin lumps
Small, firm, skin-coloured or pinkish bumps, often on the chest, back or arms. They are not cancer. Some become painful with cold or touch.
Often looks like
- Several lumps, sometimes in a cluster
- Appearing in early adult life
- Slowly increasing in number
Fibroids in the womb
Many women have fibroids. In HLRCC they tend to be numerous, appear young and cause heavy bleeding or pain, sometimes leading to surgery earlier than usual.
Kidney cancer
A specific type, called FH-deficient kidney cancer, usually in one kidney. It can grow and spread quickly, which is why regular scans matter.
A pathologist may be the first to suspect HLRCC, from how a removed tumour looks under the microscope.When both parents carry it
If two carriers have a child, the child can inherit two faulty copies. That causes fumarate hydratase deficiency, a rare and severe condition affecting the brain from infancy. It matters most in families where relatives marry each other.
Not sure whether this applies to you?
Ask an oncologistHow it comes to light
How HLRCC is usually found in a family
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A first clue
Painful skin lumps, fibroids in a young woman needing surgery, or a kidney cancer in someone younger than expected. Often the clue is noticed by a skin doctor, a gynaecologist or a pathologist.
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A closer look at the tissue
A skin lump or a removed tumour can be checked for signs of a missing FH protein. This points towards HLRCC but does not prove it.
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Counselling and a blood test
A genetic counsellor draws the family tree and arranges a blood test for the FH gene. The result confirms whether the fault is inherited.
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Relatives are offered testing
Each child, brother and sister of a carrier has a one in two chance of sharing the fault. Because kidney checks start young, testing children is often recommended.
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Surveillance begins
Carriers usually have a kidney MRI once a year, from childhood in most guidelines, along with skin and gynaecology reviews. Your team will set the exact plan.
On your report
The words you will meet, in plain language
- FH gene
- The gene for fumarate hydratase, an enzyme that helps cells turn food into energy.
- Leiomyoma
- A non-cancerous lump made of smooth muscle. In the womb it is called a fibroid.
- FH-deficient kidney cancer
- The kidney cancer linked to HLRCC, in which the tumour cells have lost the FH protein.
- Dominant inheritance
- One faulty copy from either parent is enough to raise risk, giving each child a one in two chance.
- Penetrance
- How often carriers actually develop a feature. For HLRCC kidney cancer, it is a minority.
- Surveillance
- Planned, repeated checks in someone who is well, done to find a problem early.
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Side by side
Why HLRCC kidney tumours are handled differently
In HLRCC
In most other inherited kidney conditions
Usually one tumour, in one kidney
Often several tumours, in both kidneys
Can spread while still small
Small tumours tend to grow slowly
Removed promptly once found
Small tumours are often watched for a time
MRI preferred, as ultrasound can miss them
Scan type varies by condition
Commonly believed
Four things families tell us, and what is actually true
"Fibroids are common, so they cannot mean anything."
Most fibroids are not inherited. But many fibroids in a young woman, especially with skin lumps or kidney cancer in the family, are a recognised clue worth mentioning to a doctor.
"The skin lumps are harmless, so they can be ignored."
The lumps themselves are not cancer. What they can signal matters, because they may be the first visible sign of a fault that also affects the kidneys.
"Only the person who had kidney cancer needs scans."
Every carrier in the family needs kidney checks, including those with no symptoms at all. That is the whole point of testing relatives.
"A normal kidney ultrasound means I am clear."
Ultrasound can miss small HLRCC tumours. MRI is the preferred scan for carriers. If MRI is not possible, your team will suggest the best alternative.
Being straight with you
What this page cannot tell you
It cannot tell you whether you carry an FH fault. That takes a blood test, arranged after a conversation with a genetic counsellor. It also cannot give you a personal kidney cancer risk. Published estimates vary widely between studies, and research in Indian families is still limited.
It cannot interpret a report you are holding
Some FH variants are clearly harmful and some are uncertain. What your specific variant means is a question for the counsellor who ordered the test. The decisions that follow, about scans, fibroid treatment and family planning, belong to a team that knows your history.
Who this does not apply to
Most women with fibroids do not have HLRCC. Most people with kidney cancer do not either. Testing is usually considered when the pattern is unusual: several skin lumps, many fibroids at a young age, a particular type of kidney cancer, or a relative already known to carry the fault.
If a pathology report mentions FH loss or FH deficiency, ask your doctor about a genetics referral.Questions we are asked
Common questions about HLRCC
Is HLRCC the same as FH tumour predisposition syndrome?
Yes. Both names describe the same inherited condition caused by a fault in the FH gene. The newer name reflects that the fault can cause other tumours too. You may see either name on a report or letter.
Will I definitely get kidney cancer if I carry the fault?
No. Most carriers never develop kidney cancer. The risk is real enough that regular kidney scans are advised for every carrier, so that any tumour is found and removed while it is small.
Why do children need kidney scans?
HLRCC kidney cancer has occasionally been found in teenagers and even younger children. Because it can spread while small, most guidelines start yearly MRI in childhood. Your counsellor will explain when testing and scans should begin for each child.
Can the skin lumps be removed?
Yes, painful lumps can be removed by a skin surgeon, and some medicines can ease the pain. New lumps often appear over time, so removal is usually kept for the ones that cause trouble.
Does HLRCC affect fibroid treatment?
It can. Your gynaecologist will want to know you carry the fault before planning surgery, and may prefer certain approaches. Tell every doctor involved in your care about the diagnosis, and bring your genetic report to appointments.
Does it matter if we married within the family?
It can. If both partners carry an FH fault, a child can inherit two faulty copies and develop fumarate hydratase deficiency, a severe condition affecting the brain. A counsellor can test both partners and explain the options before a pregnancy.
Is a blood test enough, or do they need tissue?
A blood sample is enough to test for an inherited FH fault. Tissue from a skin lump or tumour can add clues, and can help when a relative who had kidney cancer has died. Tumour testing to choose cancer treatment is covered on our targeted therapy pages.
Where can I get tested in Hyderabad?
Ask your dermatologist, gynaecologist or oncologist for a referral to genetic counselling, or call the CION helpline. Someone will explain the test, what to bring and how kidney scans can be arranged. Counselling in Telugu is available on request.
Your Specialists
Meet CION's oncologists. Bring your family history or genetic report to them.
Our medical oncologists see people with a strong family history of cancer, arrange genetic counselling and testing where it fits, and plan the checks that follow.
Medical Oncologist
Dr. C. Raghavendra Reddy
MBBS(Gold Medal), DNB(General Medicine), DM(Medical Oncology)(Gold Medal)
Medical Oncologist
Dr. Bharati Devi Gorantla
MBBS, MD(General Medicine), DM(Medical Oncology)(Adyar,Chennai), ECMO, MRCP SCE(UK)
Medical Oncologist
Dr. Owais Mohammed
MBBS, MD (General Medicine), DrNB (Medical Oncology), ECMO, MRCP SCE (Medical Oncology) (UK)
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Sources
- GeneReviews (NCBI) — FH Tumor Predisposition Syndrome
- MedlinePlus Genetics — Hereditary leiomyomatosis and renal cell cancer
- National Cancer Institute — Genetics of Kidney Cancer (Renal Cell Cancer) (PDQ) - Health Professional Version
- National Cancer Institute — Genetic Testing for Inherited Cancer Susceptibility Syndromes
This page is general information, not a prescription. Do not change or stop any treatment based on what you read here. If anything is worrying you, contact your own treating team — or call our helpline and we will help you reach the right specialist.
Keep reading
Related pages
Skin leiomyomas as the first clueWhat the skin lumps look like and when to mention themFibroids at a young age and HLRCCWhen fibroids are worth a genetics questionWhy HLRCC kidney surveillance starts earlyThe scan plan, and why children are includedHLRCC in Indian familiesConsanguinity, marriage and access to scans here
Talk to us
Could skin lumps, fibroids or a kidney cancer in your family be linked?
Tell us what has been diagnosed in your family and at what age. We will tell you honestly whether an FH gene test is worth doing and arrange counselling if it is. One helpline serves every CION centre.