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Gynaecological and testicular tumours in Peutz-Jeghers syndrome | CION Cancer Clinics
Peutz-Jeghers syndrome can cause growths in the ovaries, the cervix and the testes as well as the gut. Most of the ovarian and testicular ones are benign, but they can make hormones that bring on early puberty or breast growth in a boy. This page explains each tumour, the signs parents can watch for, and the checks that find them 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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On this page
- Which tumours of the ovaries, cervix and testes are linked to Peutz-Jeghers?
- The tumours in question, and what each one does
- How are these tumours watched for, from childhood to adulthood?
- The words you will meet, in plain language
- How this differs for boys and for girls
- What this page cannot tell you
- Four things parents tell us, and what is actually true
- Common questions about these tumours
The short answer
Which tumours of the ovaries, cervix and testes are linked to Peutz-Jeghers?
Peutz-Jeghers syndrome raises the chance of a few unusual tumours in the ovaries, the cervix and the testes. Most of the ovarian and testicular ones are not cancer. One rare type in the cervix can be serious, and it is easy to miss on a routine check unless the doctor knows to look for it.
Why the gut is not the whole story
STK11, the gene behind Peutz-Jeghers, acts as a brake on cell growth in many parts of the body. Families usually hear first about polyps in the bowel. The same missing brake also affects the reproductive organs, which is why a girl or a boy with this syndrome is examined in more places than just the gut.
Why hormones are often the first sign
The ovarian and testicular tumours linked to this syndrome often make oestrogen, the hormone that drives female puberty. That is why the first clue is rarely a lump. It is a change in the body: puberty arriving too early, periods that are irregular or very heavy, or breast growth in a young boy.
Most of these tumours are benign. They are watched because of what their hormones do, not because they spread.What doctors watch for
The tumours in question, and what each one does
Four areas come up in Peutz-Jeghers clinics.
Ovarian sex cord tumours
Small growths in the ovary, often in both ovaries, that are usually benign. They can make oestrogen, which changes periods and can bring puberty on early in a young girl.
Signs to mention
- Breast growth or periods in a young girl
- Irregular or very heavy periods
- Bleeding between periods
A rare cervical cancer
A rare type of cancer can grow high in the cervix, the neck of the womb. It is not caused by the HPV virus, and a routine smear can miss it. It is uncommon, but it is the one on this page that can be serious.
Signs to mention
- A watery or mucus-like discharge that does not settle
- Bleeding after sex or between periods
Testicular Sertoli cell tumours
Small growths in the testes of boys, often in both, that are usually benign. They can make oestrogen too, which can cause breast growth, a sudden growth spurt and bones that mature too early.
Signs to mention
- A testis that looks larger or feels firm
- Breast growth in a boy before puberty
Breast and womb
Women with this syndrome also have a raised risk of breast cancer, and a smaller rise in some cancers of the womb. These are covered on the page about cancer risks across the body.
Not sure whether this applies to you?
Ask an oncologistAcross the years
How are these tumours watched for, from childhood to adulthood?
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In boys, from early childhood
The testes are examined by hand at regular check-ups through childhood. An ultrasound is added if a testis looks larger, feels firm, or if breast growth appears.
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In every child, growth is tracked
Height and signs of puberty are recorded at each visit. Puberty that starts much earlier than expected, or a sudden growth spurt, is a reason to check hormone levels and look at the ovaries or testes.
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In young women, pelvic checks begin
From the late teens or early adulthood, women are offered regular pelvic examinations and cervical checks. Ask the gynaecologist to look beyond the routine smear, because the rare cervical type sits higher up.
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Breast checks follow in adulthood
Breast screening starts earlier than it does for most women. MRI is usually preferred at younger ages because it uses no radiation and sees dense breast tissue better.
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Between checks, symptoms count
A new discharge, unusual bleeding or a change in a testis should be reported when it happens. Do not wait for the next booked visit.
On the report
The words you will meet, in plain language
- Sex cord tumour with annular tubules
- The full name of the small ovarian growth seen in this syndrome. Usually benign, often in both ovaries, sometimes making oestrogen.
- Sertoli cell tumour
- A growth of the support cells in the testis. In Peutz-Jeghers it is usually benign and often shows small specks of calcium on ultrasound.
- Adenoma malignum
- An older name for the rare cervical cancer linked to this syndrome. Newer reports may call it a gastric-type cancer of the cervix.
- Gynaecomastia
- Breast growth in a boy or man. In a boy with this syndrome it can be a sign that a testicular tumour is making oestrogen.
- Precocious puberty
- Puberty that starts much earlier than expected, in a girl or a boy.
- Aromatase inhibitor
- A tablet that lowers the body's oestrogen. It is sometimes used in boys to slow breast growth and early bone maturing.
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Side by side
How this differs for boys and for girls
In boys and men
In girls and women
Tumours grow in the testes
Tumours grow in the ovaries, and rarely the cervix
Almost always benign
Ovarian ones usually benign; the cervical type can be serious
First sign is often breast growth or a growth spurt
First sign is often early puberty, unusual bleeding or discharge
Checked by hand, then ultrasound
Checked by pelvic examination, cervical tests and ultrasound
Often watched, or treated with tablets
Treated with surgery that spares fertility wherever possible
Being straight with you
What this page cannot tell you
It cannot tell you what a scan of your daughter's ovaries or your son's testes means. That depends on the size of the growth, what it looks like, what the hormone tests show and how the child is growing. Those are questions for a paediatric endocrinologist, a gynaecologist or a urologist who knows the syndrome, working alongside the gut team.
It cannot interpret a genetic report
What your specific variant means is a question for the counsellor who ordered the test.
Who this does not apply to
Most girls with irregular periods and most boys with some breast swelling at puberty do not have Peutz-Jeghers syndrome. These are common and usually harmless. This page is for families where the syndrome has already been confirmed, either by a genetic test or by a specialist's diagnosis. Studies of these tumours are small, because the syndrome is rare, and advice on exactly when to start each check varies between guidelines.
If you are unsure whether a change in your child needs to be seen soon, call the helpline and describe it. Someone will tell you honestly how quickly it should be looked at.Commonly believed
Four things parents tell us, and what is actually true
"A lump in my son's testis must be cancer."
In a boy with Peutz-Jeghers, a testicular growth is almost always benign. It still needs to be seen, because its hormones can affect growth and puberty.
"Her smear test was normal, so her cervix is fine."
A routine smear looks for the common type of cervical cancer. The rare type linked to this syndrome can sit higher up and be missed. Tell every gynaecologist about the syndrome so they look properly.
"Breast growth in a boy is just puppy fat."
Often it is, especially around puberty. In a boy with this syndrome, though, it can be a sign of a hormone-making tumour. A quick examination and an ultrasound settle the question.
"Removing the ovaries or testes is the safe choice."
Surgery is rarely the first step, and when it is needed it usually removes only the growth. Keeping fertility is a central aim. Risk-reducing surgery is a separate decision, made with a specialist, and it is one option among several.
Questions we are asked
Common questions about these tumours
Are these tumours cancer?
Most are not. The ovarian and testicular growths seen in Peutz-Jeghers are usually benign, and are watched because of the hormones they make. The rare cervical type is the exception. It can be serious, which is why women with this syndrome need cervical checks that go beyond a routine smear.
When should my son's testes first be checked?
From early childhood, as part of his regular check-ups. A doctor examines the testes by hand, which is quick and painless. An ultrasound is only needed if something looks or feels different, or if breast growth appears. Your paediatrician will set how often.
My daughter's puberty started very early. Is it the syndrome?
It can be, in a girl known to have Peutz-Jeghers, because an ovarian tumour can make oestrogen. Early puberty also has many other causes. Tell her doctor. Hormone blood tests and a pelvic ultrasound usually show whether the ovaries are involved, and neither needs an anaesthetic.
Can my daughter still have children one day?
In most cases, yes. When an ovarian growth needs removing, surgeons aim to take only the growth and leave healthy ovary behind. Fertility is discussed openly before any operation. If she later wants to talk about passing the syndrome on, a genetic counsellor can explain the options.
Does breast growth in my son mean surgery?
Usually not. The first step is an examination and an ultrasound of the testes. If a tumour is making oestrogen, tablets that lower oestrogen can slow breast growth and protect his final height. Surgery on the testis is kept for growths that are getting bigger or do not respond.
Is the HPV vaccine still worth giving?
Yes. The vaccine protects against the common type of cervical cancer, and Peutz-Jeghers gives no protection against that. The rare type linked to the syndrome is not caused by HPV, so the vaccine does not cover it. Both the vaccine and the extra checks matter.
Which doctor should examine my daughter?
A gynaecologist who knows she has Peutz-Jeghers. Carry a short letter from her specialist that names the syndrome and the checks advised. It saves time with any new doctor, including in a district hospital, and makes sure the rare cervical type is looked for properly.
Can these checks be arranged in Hyderabad?
Yes. Examinations, ultrasound, hormone tests and cervical checks are all available in the city. The harder part is keeping the gut, gynaecology and paediatric teams working from one plan. Call the CION helpline if you need help putting that plan together.
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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.
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Dr. C. Raghavendra Reddy
MBBS(Gold Medal), DNB(General Medicine), DM(Medical Oncology)(Gold Medal)
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Dr. Bharati Devi Gorantla
MBBS, MD(General Medicine), DM(Medical Oncology)(Adyar,Chennai), ECMO, MRCP SCE(UK)
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Dr. Owais Mohammed
MBBS, MD (General Medicine), DrNB (Medical Oncology), ECMO, MRCP SCE (Medical Oncology) (UK)
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Sources
- GeneReviews (NCBI) — Peutz-Jeghers Syndrome
- National Cancer Institute — Genetics of Breast and Gynecologic Cancers (PDQ) - Health Professional Version
- MedlinePlus Genetics — Peutz-Jeghers syndrome
- NCCN — Genetic/Familial High-Risk Assessment: Colorectal, Endometrial, and Gastric
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
Peutz-Jeghers syndrome explainedThe syndrome as a whole, before the detailCancer risks across organs in Peutz-JeghersBreast, womb, pancreas and gut risks, in one placeLiving with Peutz-Jeghers from childhoodHow families fit the checks into ordinary lifeSTK11 surveillance scheduleEvery check, by age, on one page
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