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Granulosa Cell Tumour of the Ovary: A Hormone-Producing Cancer That Behaves Differently

Almost everything written about ovarian cancer describes high-grade epithelial disease in a woman past sixty, found late. A granulosa cell tumour is a different disease. It grows from the ovary’s hormone-producing supporting cells, it usually announces itself early through the bleeding that oestrogen causes, and it is far more often still inside one ovary when it is found.

  • It makes hormone — and the oestrogen it makes causes bleeding — which is why these tumours get found early.
  • Usually stage I, usually slow — most are confined to one ovary at diagnosis, and surgery does the bulk of the treatment.
  • Follow-up runs for decades — these tumours can return many years later, so being discharged at five years is the thing to guard against.
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What a granulosa cell tumour of the ovary is

If you have typed granulosa cell tumour ovary into a search box this week, you will already have noticed that most of what comes back describes a different disease. General ovarian cancer pages are about high-grade epithelial cancer in a woman past sixty, usually found once it has spread across the abdomen. A granulosa cell tumour is not that. It is found differently, treated differently, monitored with different blood tests, and the outlook is generally a great deal better.

Ovarian tumours are grouped by the cell they grow from. Most arise from the surface lining — the epithelial family. A smaller group arises from the egg-producing cells — the germ cell family. Granulosa cell tumours belong to a third and much smaller family, the sex cord-stromal tumours, which grow from the supporting cells that surround and nurture a developing egg. Their close relative in that family, the androgen-producing Sertoli-Leydig cell tumour, works the same way in reverse.

In the ovary, those supporting cells make hormone for a living, and the tumours they form usually keep doing it. That single fact shapes everything else. Because the tumour produces oestrogen — doctors sometimes call it a functioning or estrogen producing ovarian tumour — it tends to declare itself through bleeding, and bleeding gets investigated. Most granulosa cell tumours are therefore caught while still confined to one ovary, which is unusual in ovarian cancer. The trade-off is a long tail: they can return many years afterwards, so follow-up is measured in decades rather than the customary five years.

A cancer, but a slow one

These tumours are malignant — they can spread, and they can come back. They also tend to grow slowly and stay put, and the majority are still inside one ovary when they are found.

It makes hormone

The tumour cells behave like the cells they came from and keep producing oestrogen. That hormone causes the bleeding, the breast tenderness, or the early puberty that usually brings someone to a doctor.

Two separate diseases

The adult type and the juvenile type share a name and a family, but they affect different ages, behave differently and are followed differently. Which one your report names changes what happens next.

Did you know?

In 2009 a Canadian research group sequenced a handful of adult granulosa cell tumours and found the same single-letter change in the FOXL2 gene — c.402C>G — in every one of them. Tested across a larger series, that one mutation turned up in about 97% of adult granulosa cell tumours and almost never in other ovarian tumours. Two things follow from it for you. It gives pathologists a near-definitive test when the appearance under the microscope is uncertain, and it separates the adult type from the juvenile type, which does not carry it. It is also a somatic mutation — it arose in the tumour itself, not in the body you were born with, so it is not inherited and it is not passed on to your children. Source: Shah SP et al., New England Journal of Medicine (2009); WHO Classification of Tumours, Female Genital Tumours (5th ed.).

Two different diseases

Adult and juvenile granulosa cell tumour, compared

They share a name, and a pathologist tells them apart down a microscope. Almost everything else — who gets them, how they show up, and what they do if they come back — is different. Find the word adult or juvenile on your report before you read anything else about this tumour.

Adult type Juvenile type
Share of cases The great majority — roughly 95 in 100 granulosa cell tumours. Uncommon — roughly 5 in 100.
Who it affects Most often diagnosed around the menopause and in the years after it, though it occurs earlier too. Girls and women under thirty. It is the commonest hormone-producing ovarian tumour of childhood.
How it usually shows up Heavy, irregular or returning periods, or bleeding after the menopause; sometimes pelvic pressure or a mass. Puberty arriving years early — breast development and bleeding in a young girl — or a quickly enlarging pelvic mass, sometimes with sudden pain from torsion.
Under the microscope Grooved, coffee-bean nuclei and Call-Exner bodies. Few dividing cells. Rounded nuclei without grooves, more dividing cells, follicle-like spaces. A busier picture that does not carry the meaning it would in an epithelial cancer.
Molecular signature A somatic FOXL2 c.402C>G mutation in nearly every case. Usually no FOXL2 mutation. Other genetic changes are seen instead.
Stage when found Usually confined to one ovary. Usually confined to one ovary.
If it comes back Characteristically late — often five to twenty years afterwards, sometimes longer. Characteristically early — usually within the first two or three years, if at all.
Follow-up Long term, using examination and inhibin B or AMH rather than CA-125. Close at first, then long term. Which marker is used depends on what the tumour produced.

*Proportions as given in the WHO Classification of Tumours, Female Genital Tumours (5th ed.). How the markers are used, and what a rising value does and does not mean, is covered in our guide to inhibin B in granulosa cell tumours.

Your pathology report

Reading the report: what the words on it actually mean

Most women meet this diagnosis as a page of unfamiliar terms, often before anyone has had time to explain it. These are the phrases that appear, and what each one changes.

“Sex cord-stromal tumour”

This is the family name, and seeing it is the first useful thing on the report. It places your tumour outside the epithelial group that all the general ovarian cancer information describes. Sex cord-stromal tumours make up only a small fraction of ovarian cancers, and granulosa cell tumours are the commonest malignant member of the group.

Practically, it means three things. CA-125 is not your marker. Standard epithelial treatment protocols do not apply directly. And any prognosis you have read that was calculated across all ovarian cancer is describing a different disease from yours.

“Adult type” or “juvenile type”

These names describe the tumour, not the patient. A woman of sixty can, rarely, have the juvenile type, and a teenager can have the adult type. The distinction is made on the appearance of the cells and on the FOXL2 result, not on your age.

It matters because the two behave differently after treatment. The adult type is the one known for very late recurrence, which is why follow-up runs for decades. The juvenile type, when it does return, tends to do so within the first two or three years, so surveillance is closest early on.

Call-Exner bodies, coffee-bean nuclei and the immunostains

Call-Exner bodies are small rosette-like spaces between the tumour cells, and coffee-bean nuclei are nuclei with a lengthwise groove. Both are classical features of the adult type, though neither is present in every case and neither is required for the diagnosis.

Where the appearance is not clear-cut, the pathologist adds immunostains. Inhibin, calretinin, FOXL2 and SF-1 are the usual panel, and a positive pattern confirms the tumour belongs to the sex cord-stromal family rather than being an epithelial cancer or a spread from elsewhere. If your report mentions these, it means the diagnosis was checked rather than assumed.

Why there is no grade on the report

Women often look for a grade because every general article about cancer mentions one, and find none here. Granulosa cell tumours are not graded the way epithelial ovarian cancers are. There is no accepted grading system for them, and the number of dividing cells, while it is recorded, does not carry the weight it would elsewhere.

What predicts behaviour in this tumour is the stage, whether it was removed intact, and how much was left behind if anything was. Those are the things to ask about, rather than a grade that does not exist for this diagnosis.

The FIGO stage, and whether the capsule was intact

Stage I means the tumour was confined to the ovary or ovaries, and that is where most granulosa cell tumours sit. Within stage I the subdivisions matter: disease inside an intact ovary is stage IA, while a tumour that ruptured, or that had cells on the ovary surface or in the fluid washings, is stage IC.

That distinction is not paperwork. It is one of the main things used to decide whether any treatment beyond surgery is discussed at all. If the operation note or the pathology report is unclear about whether the capsule was intact, have it clarified rather than left ambiguous.

What the report says about your uterus

A granulosa cell tumour produces oestrogen with nothing opposing it, and that hormone stimulates the lining of the uterus continuously. A proportion of women are found to have endometrial hyperplasia alongside the ovarian tumour, and a smaller number have a separate endometrial cancer at the same time.

So the lining has to be assessed rather than assumed. If your uterus was removed, the report should say what the endometrium showed. If it was kept, sampling of the lining should already have been done, or should be arranged now. This is the most frequently missed step in the care of these tumours, and it is worth asking about directly.

What it does not mean: this is not usually inherited

The FOXL2 mutation that defines the adult type is somatic. It happened in the tumour cells during your lifetime, not in the genes you were born with, and it cannot be passed to your children. A granulosa cell tumour is not a BRCA-related cancer, and a diagnosis of one is not by itself a reason for BRCA testing.

Family history is still worth going through properly, because it stands on its own regardless of this tumour, and the juvenile type is occasionally associated with the enchondromatosis syndromes, Ollier disease and Maffucci syndrome. Genetic counselling at CION is in-house, so this can be settled in one conversation rather than left as an open worry.

Do not sit on these

Situations that should not wait for the next routine visit

None of these means the tumour has returned. Each is a reason to be seen this week rather than at the appointment already in your diary.

Sudden, severe one-sided pain

An ovarian mass that twists or ruptures causes abrupt, severe pain, often with vomiting. That needs assessment the same day.

Any bleeding after the menopause

Whether or not you have been treated before, bleeding after the menopause is never normal and always warrants examination and an assessment of the womb lining.

Bleeding that returns years later

For a woman who has kept her uterus, bleeding starting again long after treatment can be the first sign of a recurrence producing hormone once more. It is one of the earliest signals these tumours give.

New abdominal swelling

Distension that builds over weeks and does not settle, particularly with clothes fitting differently, needs imaging rather than reassurance.

Being discharged from follow-up at five years

The adult type recurs late, sometimes decades on. If you have been told you no longer need review, ask for that decision to be revisited rather than accepting it quietly.

Nobody has checked your womb lining

If you still have your uterus and no one has scanned or sampled the endometrium since the diagnosis, raise it yourself.

If any of these apply, bring the appointment forward rather than waiting. Most turn out to be something other than recurrent disease — but with a tumour that can return after twenty quiet years, the cost of asking early is nothing and the cost of waiting is real.

No cost, no obligation

A granulosa cell tumour report deserves an unhurried second read

These tumours are uncommon enough that general ovarian cancer advice fits them badly. The decisions that matter most — whether the lining of the womb was assessed, whether fertility-sparing surgery is still possible, which marker your follow-up should use, and how long that follow-up should run — are usually settled in the first few weeks.

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Talk to a CION specialist about granulosa cell tumour treatment

No referral needed and no cost for the first consultation. Bring the scan, the operation note and the pathology report, along with any inhibin B or AMH results — those documents answer most of the questions women arrive with.

What actually happens

How a granulosa cell tumour is treated

Surgery does most of the work in this disease, and for the majority of women it is the only treatment needed. The rest of the pathway is about establishing the stage properly and then watching, for a very long time.

01

Markers taken before anything is removed

Inhibin B and anti-Mullerian hormone are the blood tests that follow this tumour, and they are far more useful when a baseline exists from before surgery. CA-125 is the wrong test here, and a normal value reassures nobody. If surgery has not yet happened, this is the most valuable thing to get right this week. What the values mean afterwards is set out in our guide to inhibin B monitoring.

02

Surgery, with proper staging

The affected ovary and tube are removed, the abdomen is inspected, washings are taken and any suspicious area is biopsied. For a woman past childbearing, the uterus and the second ovary are usually removed as well. Extensive lymph node surgery is generally not required in this tumour, because nodal spread is uncommon. At CION this surgery is coordinated with specialist gynaecologic-oncology surgeons at partner centres and may be billed there — we would rather say that now than have you discover it at the billing counter.

03

Fertility-sparing surgery, where the disease allows it

For a young woman with disease apparently confined to one ovary, the standard option is to remove that ovary and tube and leave the uterus and the opposite ovary in place, with the rest of the staging still carried out. This is a routine approach in early-stage sex cord-stromal tumours rather than an exception anyone has to argue for — but it has to be planned before the operation, not discussed afterwards.

04

The lining of the womb assessed at the same time

Because the tumour has been producing oestrogen, the endometrium is sampled during or around the surgery in every woman who keeps her uterus. A proportion are found to have hyperplasia, and a smaller number a separate endometrial cancer that needs treating in its own right. Skipping this step is the commonest gap in the care of these tumours.

05

What follows surgery depends on the stage

For most women with stage I disease completely removed, nothing further is needed and observation is the correct treatment. Where disease was advanced, incompletely removed, or has recurred, platinum-based chemotherapy is the usual approach, and hormonal treatment of the aromatase-inhibitor class has real activity here because the tumour is hormone-driven. Radiotherapy is used occasionally for a single localised recurrence. Chemotherapy, hormonal treatment and supportive care are delivered in-house at CION across 35+ centres. The wider picture is on our ovarian cancer treatment page.

06

Follow-up that does not stop at five years

Review continues with clinical examination and marker testing long past the point at which most cancers are considered settled. Recurrence, when it happens, is often a single deposit that can be removed surgically, which is exactly why finding it early is worth decades of appointments. Ask explicitly how long your follow-up is planned to run and who is responsible for it.

Treatment is decided from your stage, your age and your own priorities, not from a protocol applied to everyone. Every case that raises a question at CION goes to a tumour board rather than being settled by one doctor.

An unhurried, expert opinion

Getting a granulosa cell tumour reviewed at CION Hyderabad

The practical problem with an uncommon tumour is not that treatment is unclear — for granulosa cell tumours it is reasonably well settled — but that the reflexes built for the common disease get applied to it anyway. A CA-125 sent instead of an inhibin B. An operation planned before any markers were drawn. A womb lining never sampled. A woman discharged from follow-up at five years, for a tumour that is known to come back at fifteen.

Your first consultation at CION is free and runs to about 45 minutes, which is long enough to go through the scan, the operation note and the pathology report line by line and say what each one actually changes. If surgery has not happened yet, the useful work of that visit is making sure the baseline markers are sent and the right operation is planned. If it has, the questions become the stage, whether the capsule was intact, whether the endometrium was assessed, and how long follow-up should run.

Chemotherapy, hormonal treatment, genetic counselling, nutrition support and long-term follow-up are delivered in-house at CION, across 35+ centres in Telangana and Andhra Pradesh. Surgery, including staging and fertility-sparing surgery, is coordinated with specialist gynaecologic-oncology surgeons at partner centres and may be billed there. That division is worth knowing before you commit to a pathway rather than after.

45-minute first consultation

Free and unhurried. Long enough to read the report with you and explain which findings change the plan and which do not.

Tumour board for every case

An uncommon tumour is exactly where a single opinion is least reliable. Cases that raise a question are reviewed by the group rather than decided alone.

The right marker, from the start

Inhibin B and AMH rather than CA-125, with a baseline taken before surgery wherever that is still possible.

Straight talk on who does what

Medical oncology, genetic counselling and follow-up in-house across 35+ centres. Surgery coordinated with partner centres and possibly billed there. Said upfront.

Reading the statistics

Why the ovarian cancer survival figures do not describe this tumour

Search ovarian cancer survival and the numbers that come back are sobering. They are also, for a woman with a granulosa cell tumour, close to meaningless. Those figures are averaged across all ovarian cancer and are dominated by advanced high-grade epithelial disease in older women, because that is the overwhelming majority of cases. Your tumour is a different disease with a different natural history: usually early stage, usually slow-growing, and usually removed completely.

Subtype-specific figures are not much better. The published series for granulosa cell tumours are small, gathered over decades during which surgery and imaging changed considerably, and they mix adult and juvenile types, complete and incomplete surgery, and every stage together. Worse, because recurrence can happen twenty years out, any study with five or ten years of follow-up is reporting on a period during which nothing had yet had time to happen. That is why there is no survival percentage on this page. The honest version is that stage, complete removal and sustained follow-up are what matter, and the person to have that conversation with is the oncologist holding your report.

For completeness, and because we publish it everywhere rather than only where it flatters: 81.0% of CION ovarian cancer patients are alive at one year, against a national figure of 73.7%. That pair covers all ovarian cancer and all stages, is a one-year figure rather than a cure rate, and — for the reasons above — is not the number that describes a granulosa cell tumour.

81.0% at one year

CION ovarian cancer patients alive at one year from diagnosis. *One-year survival, CION treated population, all types and stages.

73.7% at one year

The comparable national figure for ovarian cancer. *One-year survival; national registry data.

Why neither describes your tumour

Both are averages driven by advanced epithelial disease in older women — a different cancer, found later, behaving differently.

*One-year survival rates across all ovarian cancer types and stages. CION figures reflect CION's treated patient population; national figures are drawn from published Indian cancer registry data. Survival statistics describe groups rather than individuals, and averaged ovarian cancer figures are a particularly poor guide in sex cord-stromal tumours — discuss your own outlook with your treating oncologist.

Common questions

Granulosa cell tumours — your questions answered

What is a granulosa cell tumour of the ovary?

It is a cancer that grows from the granulosa cells, the supporting cells that surround a developing egg inside the ovary. That places it in the sex cord-stromal family, which is separate from the epithelial tumours that make up most ovarian cancer and from the germ cell tumours seen in young women. Its defining feature is that it usually keeps making hormone, mainly oestrogen. That oestrogen causes heavy or irregular periods, bleeding after the menopause, or early puberty in a girl, and because bleeding gets investigated, most of these tumours are found while still confined to one ovary. They tend to grow slowly, they are monitored with inhibin B and anti-Mullerian hormone rather than CA-125, and they need follow-up lasting decades.

Is a granulosa cell tumour cancer, or something less than that?

It is cancer. These tumours can spread beyond the ovary and can return after treatment, so they are not benign and should not be described that way. But the word does not carry the meaning here that it does in high-grade epithelial ovarian cancer. Granulosa cell tumours usually grow slowly, are usually still inside one ovary when found, and for most women are cured by surgery alone with nothing further required. The important qualification is time. Recurrence is characteristically late, sometimes ten or twenty years afterwards, which is why long-term follow-up matters more in this tumour than in almost any other cancer of the ovary.

What is the difference between adult and juvenile granulosa cell tumour?

They are effectively two diseases sharing a name. The adult type accounts for the great majority, is most often diagnosed around and after the menopause, carries a characteristic FOXL2 mutation, and is known for recurring very late. The juvenile type occurs in girls and young women, does not carry that mutation, and looks more active under the microscope without that carrying the meaning it would in an epithelial cancer. When the juvenile type recurs it usually does so within the first two or three years, so surveillance is closest early on, whereas the adult type needs review continuing for decades. Both are usually confined to one ovary at diagnosis. The names describe the tumour rather than the age of the patient.

Will I need chemotherapy after surgery?

For most women, no. Where the tumour was confined to the ovary and was removed completely, observation with regular examination and marker testing is the correct treatment, and adding chemotherapy has not been shown to improve on it. Treatment beyond surgery is generally discussed where disease had spread beyond the ovary, where it could not be removed completely, where the tumour ruptured or cells were found in the abdominal washings, or if it returns later. In those situations platinum-based chemotherapy is the usual approach, and because this tumour is hormone-driven, hormonal treatment of the aromatase-inhibitor class also has genuine activity, particularly in recurrent disease. That decision belongs to a tumour board rather than to one clinician.

Can I still have children after a granulosa cell tumour?

Often, yes. Where the disease appears confined to one ovary, the standard operation for a young woman removes that ovary and its tube while leaving the uterus and the opposite ovary in place, with the rest of the surgical staging still performed. This is an accepted approach in early-stage sex cord-stromal tumours, not a concession that has to be argued for. Two things matter. It has to be planned before the operation, so raise fertility at the first consultation rather than afterwards. And because the tumour has been making oestrogen, the lining of the uterus must be sampled and treated if abnormal, since a retained uterus with untreated hyperplasia is a problem in its own right.

Can it come back after many years, and why does follow-up last so long?

Yes, and this is the single most important thing to understand about the adult type. Most cancers that are going to return do so within the first two or three years, and five clear years is treated as reassurance. Granulosa cell tumours do not follow that pattern. They can recur five, ten, twenty or occasionally more years after apparently successful treatment. Follow-up is therefore planned in decades, using clinical examination together with inhibin B or anti-Mullerian hormone. The reason it is worth the appointments is that a recurrence is often a single deposit, found while it is still small and still removable. Being discharged from follow-up early is the outcome to guard against.

Does CION treat granulosa cell tumours, and what does the first visit cost?

The first consultation is free and runs to about 45 minutes. CION delivers medical oncology in-house, which covers chemotherapy and hormonal treatment where they are needed, along with genetic counselling, nutrition support and long-term follow-up, across more than 35 centres in Telangana and Andhra Pradesh. Surgery for a granulosa cell tumour, including staging and fertility-sparing surgery, is coordinated with specialist gynaecologic-oncology surgeons at partner centres and may be billed there, and we say so at the start rather than leaving it to be discovered later. Bring the scan, the operation note, the pathology report and any inhibin B or AMH results. Every case that raises a question is reviewed at a tumour board.

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