Adrenocortical Carcinoma (ACC) FAQs
By definition ACC is a rare cancer which can occur due to an inherited risk of genetic mutations. Conditions which can predispose to ACC are types of NETs such as Multiple Endocrine Neoplasia (MEN1), Neurofibromatosis Type 1 (NF1), Von Hippel Lindau (VHL) syndrome.
Yes. ACC is considered an aggressive cancer, particularly if diagnosed at a later stage. If you begin to show symptoms, see your GP.
Surgery is the primary treatment for localised ACC. Additional therapies such as mitotane, chemotherapy or radiation therapy may be used depending on the stage.
If detected early and completely removed with surgery, ACC can sometimes be cured. Advanced cases are usually managed rather than cured.
Common symptoms include unexplained weight gain, hormonal changes, high blood pressure, and abdominal pain.
Appendiceal Neuroendocrine Cancer FAQs
Yes. They are a type of cancer, but most are low-grade and slow-growing, with excellent outcomes.
Most are discovered incidentally during appendicitis surgery or during abdominal surgery performed for unrelated reasons.
No. Tumours smaller than 1 cm usually only require an appendectomy. Larger tumours may need further surgery.
Yes, but the risk is low for small tumours. Risk increases with size and certain pathological features.
Small, low-risk tumours may need little to no follow-up. Larger or higher-risk cases require imaging and monitoring.
Breast Neuroendocrine Cancer FAQs
Yes. While it shares many features with typical breast cancer, it also has neuroendocrine characteristics that can influence diagnosis and treatment.
It is very rare, making up around 0.1% to 5% of all breast cancers.
Treatment usually follows standard breast cancer guidelines, including surgery, hormone therapy, chemotherapy, and radiotherapy, with additional NET-specific options in some cases.
It depends on the subtype. Some are slow-growing, while others, especially poorly differentiated tumours, can be aggressive.
Rarely. Most breast neuroendocrine cancers do not cause hormone-related syndromes.
In many cases, yes. A team experienced in both breast cancer and neuroendocrine cancers can provide more tailored care.
Duodenal Neuroendocrine Cancer FAQs
It is a rare cancer that forms in hormone-producing cells in the duodenum, part of the small intestine.
Yes. Many are slow-growing and less aggressive, but some can behave more aggressively depending on grade.
Non-functioning tumours are the most common, making up the majority of cases.
Small tumours may be removed endoscopically, while larger or advanced tumours may require surgery or systemic therapies.
Well-differentiated tumours have a high survival rate, often around 80–85% at five years.
Yes. Due to their rarity and complexity, treatment by a NET specialist team is strongly recommended.
If you’ve been diagnosed with duodenal neuroendocrine cancer, early specialist care and access to support services can significantly improve both outcomes and quality of life.
Gastric Neuroendocrine Cancer FAQs
Gastric neuroendocrine cancer is a rare cancer that starts in neuroendocrine cells in the stomach lining. These cells help regulate digestion and stomach acid production.
It is a type of stomach cancer, but it is different from the more common gastric adenocarcinoma. Gastric NETs arise from neuroendocrine cells rather than the glandular cells usually involved in common stomach cancers.
The main types are Type 1, Type 2, Type 3, and poorly differentiated gastric neuroendocrine carcinoma, sometimes described as Type 4.
Type 1 gastric NETs are the most common. They are usually linked to autoimmune atrophic gastritis and high gastrin levels.
Many gastric NETs are slow-growing, especially Type 1 and Type 2 tumours. Type 3 tumours and poorly differentiated neuroendocrine carcinomas can behave more aggressively and may require more intensive treatment and monitoring.
Diagnosis usually involves gastroscopy, biopsy, pathology testing, blood tests, and possible imaging such as CT, MRI, endoscopic ultrasound, or Gallium-68 DOTATATE PET/CT.
Yes, some gastric NETs can spread to lymph nodes, the liver, or other organs. The risk depends on factors such as tumour type, size, grade, depth of invasion, and whether the tumour is well differentiated or poorly differentiated. Type 3 and Type 4 tumours have a higher risk of spreading than Type 1 and Type 2 tumours.
Treatment may include endoscopic removal, active surveillance, surgery, somatostatin analogues, PRRT, chemotherapy, or liver-directed therapies depending on the tumour type and stage.
Many people need ongoing monitoring, especially because some gastric NETs can recur or new tumours can develop. The follow-up schedule depends on the tumour type and risk level.
NeuroEndocrine Cancer Australia provides education, resources, advocacy, and access to the NET Nurse service for people affected by gastric neuroendocrine cancer.
Goblet Cell Adenocarcinoma (GCA) FAQs
Goblet cell adenocarcinoma is a rare cancer of the appendix. It contains both neuroendocrine and gland-forming adenocarcinoma features.
No. GCA has minor neuroendocrine features, and therefore behaves differently from most classic appendiceal NETs and is usually treated more like an adenocarcinoma.
Older terminology described GCA as goblet cell carcinoid or adenocarcinoid. These terms are no longer preferred because GCA can behave more aggressively than typical carcinoid or neuroendocrine tumours and has only minor features of neuroendocrine cells.
GCA often causes symptoms similar to appendicitis, including lower right abdominal pain. Some people may have bloating, a mass, weight loss, bowel changes, or symptoms related to spread within the abdomen.
It is often diagnosed after appendix surgery. Diagnosis is confirmed by pathology, where the tumour is examined under a microscope and tested for specific markers.
Yes. GCA can spread to lymph nodes, the lining of the abdomen, and sometimes the ovaries in women.
Treatment usually involves surgery, often a right hemicolectomy. Chemotherapy may also be recommended, especially for higher-risk or advanced disease.
CRS/HIPEC is a specialised treatment used in some cases of cancer spread within the abdomen. It combines surgery to remove visible tumour with heated chemotherapy delivered into the abdominal cavity.
Prognosis depends on stage, grade, and whether the cancer has spread. Early-stage GCA may have a better outlook, while metastatic or high-grade disease is more difficult to treat.
Yes. Because GCA is rare and complex, review by a multidisciplinary team with relevant expertise is important.
Grading & Staging FAQs
Grade describes how quickly a neuroendocrine tumour is growing and how aggressive it appears under a microscope. It helps your healthcare team understand how the cancer may behave and which treatments are most appropriate.
Doctors use two main markers. The Ki-67 index measures how many tumour cells are actively dividing, and the mitotic rate counts how many cells are seen dividing under a microscope. Together, these help determine whether a tumour is Grade 1, Grade 2, or Grade 3.
Grade 1 tumours usually grow slowly and are well differentiated. Grade 2 tumours grow at a moderate rate. Grade 3 tumours grow more quickly and may be either well differentiated neuroendocrine cancer (NET) G3 or poorly differentiated neuroendocrine carcinomas (NECs).
Higher grade tumours often grow faster and may require more intensive treatment. However, every person’s situation is different, and outcomes depend on many factors including stage, tumour location, and response to therapy.
No. Grade describes how aggressive the tumour cells look, while stage describes how far the cancer has spread in the body. Both are important when planning treatment.
In some cases, the tumour biology may change. Doctors may repeat biopsies or imaging if the disease behaves differently, to ensure treatment decisions remain accurate.
The Ki-67 index refers to a key biomarker that shows how fast tumour cells are growing. A low Ki-67 suggests slower growth, while a higher number indicates more rapid cell division and often different treatment approaches.
Yes. Lower grade tumours may be managed with surgery, monitoring, or treatments like somatostatin analogues, while higher grade tumours may require chemotherapy or more intensive therapies.
A specialist pathologist reviews biopsy or surgical samples to assess the tumour. Your multidisciplinary NET team then uses this information to guide treatment planning.
NeuroEndocrine Cancer Australia provides education resources, assistance finding NET Specialists, and access to the NET Nurse service to help people living with NETs and their families understand grading, treatment options, and next steps.
Large Bowel Neuroendocrine Cancer FAQs
Colorectal neuroendocrine cancer is a rare type of neuroendocrine cancer that develops in neuroendocrine cells in the colon, caecum, or rectum.
It is a type of bowel cancer, but it is different from common bowel adenocarcinoma. It starts in neuroendocrine cells rather than glandular cells in the bowel lining.
A NET is well differentiated and may grow more slowly. A NEC is poorly differentiated, high grade, and usually grows more quickly.
A MiNEN is a mixed tumour that contains both neuroendocrine cancer cells and non-neuroendocrine cancer cells, often adenocarcinoma cells.
Symptoms may include abdominal pain, bloating, bowel habit changes, diarrhoea, constipation, rectal bleeding, weight loss, or bowel obstruction.
Yes, in some cases. Carcinoid syndrome is more likely if a functional tumour has spread to the liver.
Diagnosis may involve colonoscopy, biopsy, pathology testing, CT or MRI scans, PET imaging, and blood or urine tests.
Treatment may include surgery, somatostatin analogues, chemotherapy, PRRT, targeted therapies, or liver-directed treatments depending on the tumour type and stage.
Yes. It can spread to lymph nodes, liver, lungs, or other parts of the body.
NeuroEndocrine Cancer Australia provides education, resources, and access to specialist support services for people affected by neuroendocrine cancer.
Newly Diagnosed FAQs
Neuroendocrine cancer develops from neuroendocrine cells when they undergo changes, causing them to divide uncontrollably, growing into a mass called a tumour. These neuroendocrine cells create, store and secrete a variety of hormones for normal bodily function.
To learn more, watch our explainer video - What is the Neuroendocrine System and how do Neuroendocrine Tumours develop?
NeuroEndocrine Cancer Australia (NECA) offers face-to-face support groups across Australia, a national online group for those in rural and remote areas, private Facebook groups for connecting with the NET community and telehealth specialist support, including access to a neuroendocrine cancer nurse, counsellor, and dietitian. Learn more
NeuroEndocrine Cancer Australia (NECA) offers free in-services for healthcare professionals and teams, educational materials such as the Optimal Care Pathway – Quick Reference Guide and HCP booklet, and online education modules. Click here to access and download these educational resources and module information.
Spot the signs FAQs
Neuroendocrine cancer symptoms may be flushing, wheezing, palpitations, diarrhoea, fatigue and rashes. To learn more about neuroendocrine cancer symptoms click here.
When considered collectively, neuroendocrine cancer is now classified as a common cancer and is the 7th most commonly diagnosed cancer in Australia. However, when examined by individual tumour sites, each type of neuroendocrine cancer remains rare. Click here to view other neuroendocrine cancer facts.
Schedule an appointment with your GP and take the Optimal Care Pathway – Quick Reference Guide to assist with the diagnosis process. Click here to view and download the quick reference guide.
Von Hippel Lindau (VHL) FAQs
Many people with VHL lead full and active lives with appropriate monitoring and specialist care. Early detection and coordinated treatment are key to managing the condition successfully.
Treatment depends on the tumour type and location. Options may include surgery, targeted therapies, minimally invasive procedures, or careful monitoring if tumours are small and stable.
People with VHL often require regular MRI scans, eye and ear examinations, abdominal imaging, blood pressure monitoring and blood tests. Your specialist team will create a personalised surveillance plan.
There is currently no cure for VHL. Management focuses on lifelong monitoring and treating tumours early to prevent complications and improve quality of life.
Diagnosis usually involves a combination of clinical assessment, imaging scans, and genetic testing to confirm a mutation in the VHL gene.
Symptoms vary depending on where tumours develop. Common signs include vision changes, headaches, balance problems, high blood pressure, or abdominal pain. Many people are diagnosed through screening rather than symptoms.
VHL follows an autosomal dominant inheritance pattern. This means a child of an affected parent has a 50 percent chance of inheriting the altered gene. Around 20 percent of cases occur as new mutations without a family history.
VHL itself is not a cancer, but it is a genetic syndrome that has genetic mutations which increase the risk of several cancer types, including clear cell renal cell carcinoma and phaeochromocytoma. Some people with VHL may also develop neuroendocrine cancer.
Von hippel lindau (VHL) is a rare inherited genetic condition that increases the risk of developing tumours and cysts in different parts of the body, including the brain, spine, eyes, kidneys, pancreas, and adrenal glands. Some growths are benign, while others may become cancerous.
What are neuroendocrine cancers?
Neuroendocrine cancer develops from neuroendocrine cells when they undergo changes, causing them to divide uncontrollably, growing into a mass called a tumour. These neuroendocrine cells create, store and secrete a variety of hormones for normal bodily function. To learn more, watch our explainer video - What is the Neuroendocrine System and how do Neuroendocrine Tumours develop?
Neuroendocrine cancer can develop anywhere in the body, but most commonly arises in the gastrointestinal tract and pancreas (around 60% of cases), followed by the lungs (approximately 20%). Click here to view different types of neuroendocrine cancers.
Neuroendocrine cancer stage 4 means that the cancer has spread from where it first started to other parts of the body. This is also known as advanced or metastatic neuroendocrine cancer. To learn more about grading and staging click here.
