Supporting the

Neuroendocrine Cancer Community

Gynaecological Neuroendocrine Cancer

Sep 24, 2021

September is Gynaecological Cancer Awareness Month (GCAM).

Gynaecological cancer refers to cancers that start in 5 of the main parts of the female reproductive system – ovary, womb (uterine or endometrium), cervix, vagina and vulva.

Sex assigned at birth, hormones and/or surgeries will be relevant for some types of gynaecological cancer from assessing risk to screening programme suitability :  gynaecological cancers may affect trans men ( female-to-male – FTM or F2M) and/or non-binary if gynaecological structures are still present: further information on screening is available from CRUK.

In a 2016 survey by The Eve Appeal  a significant number of respondents reported to know little about their own anatomy; with nearly half of those surveyed unable to identify the vagina or cervix on an anatomical diagram.

Neuroendocrine Cancers of the female reproductive System (gynaecological organs) range from the uncommon to incredibly rare, depending on site of origin. The cervix and ovary are the most common locations, however, they can also occur in the endometrium, fallopian tubes, vagina, and vulva.

As in Lung Neuroendocrine Cancers there are 4 main categories :

  • Typical or Atypical Carcinoid (low-moderate grade NETs) and
  • Small or Large Cell Neuroendocrine Carcinoma (high grade NECs).

Please note that even if you are under surveillance for a non-gynaecological cancer eg Lung, Small Bowel or Pancreatic NET or NEC, starting or continuing routine cancer screening programmes are important to your overall health – your risk of developing a more common cancer is not reduced by having Neuroendocrine Cancer, and not all surveillance scans and tests will necessarily provide adequate common cancer screening for you.

Symptoms, if present, may be similar to those experienced by women with more common gynaecological problems and/or cancers – diagnosis is usually confirmed by expert histology review, following surgery or a biopsy.

Gynaecological (Neuroendocrine) Cancer symptoms by site (Cervix, Ovary, Uterus, Vagina & Vulva):

  • Unusual / Abnormal discharge :  ALL sites
  • Abnormal vaginal bleeding :  ALL sites
  • Early satiety – feeling full / constant bloating / ‘wind’ :  Ovary
  • Pelvic pressure or discomfort or pain :  Ovary, Uterus or Vagina
  • Abdominal (tummy) +/- Back pain :  Ovary or Uterus
  • Increased need to pee +/- constipation :  Ovary or Vagina
  • Itching/burning/tenderness of vagina / vulva :  Vagina or Vulva
  • Lump +/- skin changes – rashes, warts, sores :  Vagina or Vulva
  • Pain during +/- Bleeding after sex :  ALL sites


By Site:

Cervical Cancers

The overall incidence of cervical cancer is about 3,200 / year. It is more common in the under 35’s. Although HPV infection (HPV) may be very common and is considered a high risk factor for cervical cancer, cervical cancer itself is relatively uncommon and it is being detected earlier thanks to the success of the UK screening and vaccination programmes.

Vaccination of both girls and boys against HPV is believed to have played a major role in reducing the development of cervical cancer – including Neuroendocrine Cervical Cancers.

Neuroendocrine cancers of the Cervix account for less than 2% of all invasive cervical cancers (that is less than 100/yr – though some authors report up to 200 cases/yr in the US) and are classified as above – though some may be MiNEN ( a mix of Neuroendocrine and Non-Neuroendocrine Cancer within the same tumour) – therefore accurate and expert histology is essential to inform appropriate care.

Ovarian Cancers

Ovarian cancer, in general, has an incidence of approximately 7,500 / year. Ovarian cancer is less common in younger women, with around 80% of cases occurring in women over 50.

Neuroendocrine Cancer of the Ovary affects approximately 30 women a year in the UK. They are usually secondary deposits from the bowel or appendix, but primary disease can and does occur. Ovarian Neuroendocrine Cancers can be classified into the 4 groups mentioned above or rarely as a paraganglioma (a type of neuroendocrine tumor that forms near certain blood vessels and nerves outside of the adrenal glands that may or may not hyper secrete catecholamines such as adrenaline and noradrenaline – which can affect blood pressure and heart rate).

Most cases of ovarian cancer do not run in families, but genes known as BRCA1 and BRCA2 can increase your risk of developing ovarian and/or breast cancer.

Paragangliomas can occur as part of a hereditary syndrome or in individuals with certain rare inherited disorders, such as von Hippel-Lindau syndrome, Carney-Stratakis syndrome, and certain types of multiple endocrine neoplasia.

Further information about pheochromocytoma, paragangliomas and their associated genetic disorders can be found at AMEND and PheoParaSupportUK.

We recommend speaking to your GP, and specialist team, if you have a family history of breast and/or ovarian cancer, pheochromocytoma, paraganglioma and/or one of the genetic conditions mentioned. They may refer you for a specialist endocrinology review and/or an NHS genetic test, which will tell you if you have inherited one of the cancer-risk genes.

Uterine, Vaginal and Vulval Cancers

Primary Neuroendocrine Cancers of the uterus may be even rarer than either of the above, with vaginal and vulval primary sites the most rare:  only a handful of women, across the world, are diagnosed each year.

  • Although endometrial (uterine or womb) cancer is the 4th most common cancer – with an estimated 9,000 new cases being diagnosed each year in the UK – there are very few cases of Neuroendocrine Cancer reported. Expert histology review is required to confirm diagnosis. Endometrial cancer is more common in post-menopausal women, although 20% of cases present in women of childbearing age.
  • Cancer of the vagina is rare with fewer than 300 women diagnosed each year in the UK. Less than 30 cases of Neuroendocrine Cancer of the Vagina have been reported (worldwide), in medical literature.
  • Vulval cancer affects around 1,300 a year in the UK. Less than 20 cases (worldwide) of Neuroendocrine Cancer of the Vulva had been reported in medical publications.

For both Vaginal and Vulval Neuroendocrine Cancers, expert histology is once again essential to clarify diagnosis – especially as there is a further type of Neuroendocrine Cancer that may affect these more externally placed parts of the female reproductive system – and that is Merkel Cell Carcinoma (also known as Neuroendocrine Cancer of the Skin).

Further information about diagnostic tests, treatments and follow up for Gynaecological Neuroendocrine Cancers can be found on our website here

LIFTING THE LID ON CANCER AND SEX : Trekstock Event 28th September

Let’s talk about sex : Cancer impacts every part of your life and that includes sex.

If your specialist team isn’t talking about it and/or you find it difficult to discuss, it’s a stalemate. But it need not be . . .

If you’ve just been diagnosed, living with or now finished cancer treatment, or are a loved one or healthcare professional then this event may be of interest to you.

Join Trekstock (one of our Cancer52 partner charities) on the 28th of September for a free event, hosted by the fierce and funny Lauren Mahon and a stellar panel of experts. Trekstock are bringing together members of their community and known experts to help us all become a little less anxious or nervous when talking about sex and cancer.

If you’re not quite ready for meeting people in person yet, or are unable to get to the venue, don’t worry –  the event will be live streamed so you can join in from the comfort and privacy of home.

To find out more and / or register for the event – click here