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Kidney cancer · Hereditary & genetics
HLRCC (hereditary leiomyomatosis and RCC) — what an inherited FH gene change means for your kidneys
HLRCC is a rare inherited condition caused by a change in the FH gene. It links three things that at first seem unrelated: firm lumps in the skin, fibroids in the uterus that arrive earlier and in greater number than usual, and a kidney tumour type that is taken seriously even when small. Most people who carry an FH change never develop a kidney tumour — but because this tumour type is not one that is safely left alone, carriers are offered planned kidney monitoring rather than being asked to wait for symptoms.
- Fibroids alone are not HLRCC — fibroids and harmless skin lumps are common in people with nothing inherited at all. It is the combination, and the family pattern, that matters.
- Carrying the change is not a diagnosis — it removes one safeguard. Most carriers never develop a kidney tumour, and monitoring exists precisely to keep it that way.
- Genetic counselling is led in-house — medical oncology at CION draws out the skin, gynaecological and family history first, and tests only where the result changes something.
- One family, one plan — once the exact change is known, relatives can be tested for that change alone, and those who do not carry it are released from surveillance.
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The short answer
What HLRCC is, in plain language
The name describes the two halves. “Hereditary leiomyomatosis” means smooth-muscle growths — leiomyomas — that run in a family. Smooth muscle sits in the skin around each hair and in the wall of the uterus, which is why the growths turn up in those two places. “RCC” is renal cell carcinoma, the usual kind of kidney cancer. HLRCC is the condition that ties them together, and it is also written as FH-deficient renal cell carcinoma or, in older writing, Reed syndrome.
One gene explains all of it. The FH gene carries the instructions for an enzyme called fumarate hydratase, which cells use in one of the steps that converts food into energy. In HLRCC a person inherits one altered copy of that gene, so the fault is present in every cell of the body from birth rather than appearing in one kidney cell by chance. When a cell loses its remaining working copy, the enzyme stops doing its job, a chemical builds up inside the cell, and the cell starts behaving as though it were starved of oxygen — switching on the growth and blood-vessel signals that let a tumour establish itself.
It is inherited in an autosomal dominant pattern. That means each child of a carrier can inherit the altered copy, sons and daughters equally, and a single altered copy is enough for the condition to be present. It also means the condition does not skip generations in the way people often assume — what varies is how visibly it shows up in each person, not whether the gene is there.
Carrying the change is not the same as having cancer. This is the sentence most people searching for HLRCC actually need. The skin and uterine features are far more commonly seen than kidney tumours, and most carriers never develop one. What HLRCC changes is not your certainty but your plan: instead of assuming your kidneys are fine, they are looked at on a schedule, so that anything that does appear is found while it is still small and straightforward to deal with. If you want the wider picture of the disease itself — types, symptoms, staging and treatment — start with our kidney cancer guide.
HLRCC is rare. If you have arrived here because you have fibroids, or a few small lumps on your skin, the odds overwhelmingly favour a common and unrelated explanation. This page is here so you can tell whether your particular pattern is worth raising with a specialist — not so you can diagnose yourself. If you are unsure, book a free consultation and have it looked at properly.
Did you know?
The clue that most often puts HLRCC on a specialist’s list is not the kidney at all — it is the skin. Small firm lumps that can be tender to cold, pressure or touch are easy to dismiss for years, yet they are frequently the earliest visible sign, and they often appear long before anything is found in a kidney. That is why a dermatology finding sometimes leads to a genetics referral rather than the other way around.
What a specialist looks for
How HLRCC shows up — and what it is often mistaken for
No single feature below makes a diagnosis. They are the signals that make a specialist stop and take the history seriously enough to formalise it. Read them to work out which conversation you need, not to reach a conclusion.
Skin
Firm lumps in the skin
Smooth-muscle growths in the skin are typically small, firm and skin-coloured to brownish, often on the trunk, arms or face. They can be tender when pressed, knocked or exposed to cold, and they tend to appear over years rather than overnight. They are not cancerous. Their importance is as a marker: multiple lesions of this specific type are one of the strongest reasons to look for an FH change.
Uterus
Fibroids that arrive early and in number
Fibroids are common in the general population, so their presence proves nothing on its own. What draws attention in HLRCC is the pattern — fibroids that appeared at an unusually young age, that were numerous, or that led to surgery far earlier than expected. Where that is the history, and particularly where it repeats across the female relatives of a family, it is worth mentioning to a specialist.
Kidney
A kidney tumour that behaves differently
The kidney tumour type linked to HLRCC is uncommon, and it does not follow the rules that apply to ordinary small kidney tumours. It is usually a single tumour in one kidney, and it can behave aggressively while still small. That single fact is what shapes everything else about how the condition is managed — the kind of imaging chosen, how often it is repeated, and how quickly a new finding is acted upon.
Family pattern
The same story in more than one relative
HLRCC becomes much more likely when the picture repeats: skin lumps in a parent, early hysterectomy for fibroids in a sister or aunt, kidney cancer at a young age in the family. Draw the family out properly before you judge it. People often remember “a cancer” or “a women’s operation” without remembering the organ or the age, and those two details decide almost everything.
Not this
What does not point to HLRCC
Fibroids alone in an adult woman. Moles, cysts, skin tags or lipomas. A single older relative with one kidney tumour and nothing else. Kidney stones, or a kidney removed for a non-cancer reason. None of these puts HLRCC on the table, and none of them is a reason to request a genetic test. Being able to rule your situation out is as useful an outcome as being able to rule it in.
Related, not the same
The syndromes it is confused with
Several inherited conditions raise kidney tumour risk, and they are not interchangeable — each has its own gene, tumour type and management. The most widely recognised is Von Hippel-Lindau (VHL) disease and kidney cancer. The one closest to HLRCC in tumour appearance, and most often confused with it, is hereditary papillary RCC (MET), which behaves very differently.
Telling these conditions apart is not a technicality. VHL, hereditary papillary RCC and HLRCC each carry a different tumour type, a different imaging schedule and a different threshold for acting on a small lesion. Getting the label right is what makes the plan right.
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What actually happens
If HLRCC is suspected or confirmed, what happens next
In the order it usually unfolds. Most people who start this list are told, somewhere in the first two steps, that their pattern does not fit — and that is a real answer, not a fobbing-off.
The history is taken across three systems at once
Skin, gynaecology and family, in one sitting. When did the skin lumps appear, how many, are they tender to cold or pressure? At what age did the fibroids start, how many, was surgery needed and how early? Then both sides of the family separately, with ages at diagnosis. This is the step that does most of the work, and the one you can prepare for. Ten minutes on the phone to an aunt is worth more here than any amount of reading.
The features are confirmed for what they actually are
A story is not a diagnosis. Where the skin lesions look like smooth-muscle growths, a small biopsy settles what type they are. Where a kidney tumour has already been removed or sampled elsewhere, the original pathology is reviewed rather than taken on trust, because the tumour’s appearance under the microscope can itself raise the suspicion. Old operation notes and discharge summaries from relatives are gold at this stage.
Genetic counselling comes before any test
NCCN guidance recommends genetic risk evaluation where the pattern fits an inherited kidney cancer syndrome — a young age at diagnosis, more than one tumour, or a suggestive family history. Counselling at CION is led in-house by medical oncology: you go through what a result would and would not tell you, what it means for your relatives, and what would change if it were positive. Only then is FH testing offered. Where nothing meets the criteria, counselling ends with a clear explanation of why a test would not help.
If a change is confirmed, the family is tested for that one change
This is where a confirmed diagnosis starts being useful. Once the exact FH change is known, close relatives can be tested for that specific change — a far simpler and quicker test than the first one. Relatives who do not carry it are released from surveillance for good. Testing of children is discussed carefully rather than assumed, because when to test is a decision with consequences of its own, and it belongs in a counselling room.
The kidneys go under planned imaging, not ultrasound alone
Confirmed carriers and at-risk relatives are offered kidney imaging on a defined schedule set by the treating team, with cross-sectional imaging — MRI is usually preferred — because small lesions of this type can be missed on ultrasound and MRI avoids repeated radiation over a lifetime of monitoring. Scans are arranged and reported in-house at CION and reviewed each time by the same team, so a change between studies is noticed rather than filed.
A suspicious lesion is acted on promptly, not watched
In several other inherited kidney conditions a small tumour can be measured on repeat scans and treated only once it reaches a threshold. HLRCC is deliberately handled differently, because this tumour type can behave aggressively while still small. Surgery is arranged with specialist urology and uro-oncology partner centres rather than performed in-house at CION, with the plan agreed jointly beforehand and the follow-up brought back to your medical oncology team.
If disease is advanced, systemic treatment is chosen by tumour biology
Where a kidney tumour has spread, treatment is systemic and delivered in-house by medical oncology. Options are chosen by class and mechanism rather than by habit: immune checkpoint inhibitors, drugs that block the VEGF blood-vessel pathway the tumour is relying on, and combinations of the two are all used in advanced kidney cancer, and FH-deficient tumours have their own considerations. What suits which situation is a conversation, not a page — kidney cancer treatment in Hyderabad sets out the full range.
The rest of the body is not forgotten
HLRCC care is not only kidney care. Skin lesions are reviewed and treated where they are uncomfortable rather than because they are dangerous. Women are given gynaecology input on fibroid management, which often matters far more to daily life than anything happening in the kidney. And the plan is written down once for the whole family, so nobody is repeating the same explanation to a new doctor every few years.
One thing this page will not do is put a number on your personal risk. Published figures describe populations, not individuals, and quoting one to you without knowing your family and your own findings in detail would be dishonest. A consultation can tell you which category you fall into — and that is the answer that actually changes what you do next.
You do not have to work this out alone
One conversation settles whether HLRCC applies to you
Bring what you know — the skin, the fibroid history, who in the family was affected and at what age. You will leave knowing whether this is something to act on or something to stop carrying.
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Common questions
HLRCC and the FH gene - your questions answered
What is HLRCC?
HLRCC stands for hereditary leiomyomatosis and renal cell carcinoma. It is an inherited condition caused by a change in the FH gene, which carries the instructions for an enzyme called fumarate hydratase. When that enzyme does not work properly, the affected cell behaves as though it is short of oxygen and switches on growth signals it should not. In practice the condition shows up in three places: firm smooth-muscle lumps in the skin, fibroids in the uterus that tend to appear younger and in greater number than usual, and a kidney tumour type that needs to be taken seriously even when it is small. Most carriers never develop a kidney tumour, but the possibility is the reason the kidneys are kept under planned review.
What does the FH gene do, and how is HLRCC inherited?
The FH gene tells the body how to make fumarate hydratase, an enzyme that cells use in one of the steps that turns food into energy. In HLRCC one working copy of the gene is inherited altered. That single altered copy is present in every cell from birth, which is why the condition can affect the skin, the uterus and the kidney rather than one organ alone. It is passed on in an autosomal dominant pattern, meaning each child of someone who carries the change can inherit it, and it is inherited equally by sons and daughters. Inheriting the change does not mean cancer will follow; it means one safeguard is missing, which is exactly what surveillance is designed to compensate for.
I have fibroids and some small skin lumps. Do I have HLRCC?
Almost certainly not. Uterine fibroids are extremely common in women who have nothing inherited at all, and harmless skin lumps of many kinds are common too. HLRCC is rare, and what raises it as a possibility is a combination rather than either feature alone: skin lumps of the specific smooth-muscle type, particularly when they are multiple or tender to cold, pressure or touch, alongside fibroids that appeared unusually young or needed surgery early, and often a family history of the same pattern or of kidney cancer at a young age. If that combination describes you, ask for genetic counselling. If it does not, it is far more likely you have two common and unrelated things.
How is HLRCC diagnosed?
It usually starts with a careful history rather than a scan: the skin, the gynaecological history and both sides of the family are gone through in detail. If the picture fits, a skin lesion may be biopsied to confirm what type of growth it is, and genetic counselling follows so you understand what a test result would and would not tell you before any blood is drawn. Confirmation comes from testing the FH gene. Where a kidney tumour has already been removed or sampled, the pathology itself can raise the suspicion and prompt referral for genetic evaluation. At CION, genetic counselling and the diagnostic work-up are led in-house by medical oncology.
Why are HLRCC kidney tumours not simply watched like other small kidney tumours?
Because they do not behave like the ordinary small kidney tumour. In several other inherited kidney conditions the tumours grow slowly enough that a small one can be measured on repeat scans and treated only when it reaches a certain size. The kidney tumour type associated with HLRCC can behave aggressively while it is still small, so waiting is not treated as a safe option. That is why guidance from bodies such as NCCN sets HLRCC apart: surveillance is with detailed imaging rather than ultrasound alone, and a suspicious lesion is acted on promptly. Surgery itself is arranged with specialist urology and uro-oncology partners, with the plan agreed jointly beforehand.
If HLRCC is confirmed in my family, what happens to my relatives?
This is where a confirmed diagnosis becomes useful rather than frightening. Once the exact FH change is known in one person, close relatives can be tested for that specific change, which is a far simpler test than the first one. Relatives who do not carry it are released from surveillance and can stop worrying. Relatives who do carry it are offered planned kidney imaging on a schedule the team sets, along with skin review and, for women, gynaecology input on fibroid management. Testing of children is discussed carefully in counselling rather than assumed, because timing matters. All of this is coordinated in-house at CION so the family is followed as one.
This page is general information about an inherited condition, not a diagnosis or a personal risk assessment. Only a doctor who has taken your history, examined you and reviewed your family details can tell you whether HLRCC applies to you and what, if anything, needs following up.