FAQs

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.

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.

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.

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.

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.

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.

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.

No. Many neuroendocrine cancers (NETs) are "non-functioning" and do not produce excess hormones. Hormonal syndromes only occur with "functioning" tumours.

Yes. Treatment may include medication to control symptoms, somatostatin analogues, surgery to remove the tumour, or other targeted therapies depending on the tumour type and location.

No. Carcinoid syndrome is one specific type of hormonal syndrome. Others, such as Zollinger-Ellison syndrome or VIPoma, are caused by different hormones and have different symptoms.

Some can. Certain hormone-producing NETs are linked to inherited genetic syndromes, such as MEN1, MEN2, VHL, and NF1. Genetic counselling can help clarify the risk to you and your family.

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.  

No. MTC arises from C cells, which are neuroendocrine cells, not the follicular cells that are the origin of the more common papillary and follicular thyroid cancers. This makes MTC biologically different and means it is diagnosed and treated differently.

C cells do not absorb iodine. Radioiodine treatment only works for cancers arising from follicular cells. MTC does not respond to radioiodine and is also not managed with TSH suppression, which is a standard part of treatment for other thyroid cancers.

Yes. Approximately 25 to 30 per cent of MTC cases are hereditary, caused by an inherited mutation in the RET gene. This is why genetic testing is recommended for everyone diagnosed with MTC, regardless of their family history.

Common symptoms include a painless neck lump, hoarseness, difficulty swallowing, and a feeling of pressure in the neck. Rarely, some people experience diarrhoea or flushing caused by excess calcitonin, particularly when the cancer has spread. Many early-stage MTCs cause no symptoms.

Diagnosis usually involves thyroid ultrasound, fine needle aspiration biopsy, blood tests for calcitonin, and RET genetic testing. Imaging scans are used to assess whether the cancer has spread.

Surgery, specifically total thyroidectomy with central neck lymph node dissection, is the primary and only potentially curative treatment. Lifelong thyroid hormone replacement is required after surgery. Targeted therapies, including selective RET inhibitors, are used for advanced or progressive disease.

If an undetected phaeochromocytoma is present, the stress of anaesthesia during thyroid surgery can trigger a dangerous and potentially life-threatening surge in blood pressure. Screening for phaeochromocytoma before surgery is a critical safety step, particularly for people with hereditary MTC or MEN2.

Calcitonin doubling time measures how quickly calcitonin levels are rising in the blood after surgery. A shorter doubling time suggests faster disease progression, while a longer doubling time suggests more slowly progressive disease. It is an important tool for guiding surveillance and treatment decisions.

Multiple Endocrine Neoplasia type 2 (MEN2) is an inherited syndrome caused by mutations in the RET gene. MEN2A (the most common form) can involve MTC, phaeochromocytoma, and primary hyperparathyroidism. MEN2B is rarer but more aggressive and can also involve mucosal neuromas (small benign growths on the lips, tongue, or mouth) and marfanoid body type (disproportionately long arms, legs and long spider-like fingers)

When detected early and treated with complete surgical removal, MTC can often be cured or well-controlled for many years. Advanced or metastatic MTC is more complex to treat, but newer targeted therapies, including selective RET inhibitors, have significantly improved outcomes for people with advanced disease.

Yes. After total thyroidectomy, the thyroid gland is no longer present to produce thyroid hormones. Daily thyroxine (thyroid hormone replacement) is required for life to maintain normal thyroid hormone levels and prevent hypothyroidism.

NeuroEndocrine Cancer Australia provides education, resources, advocacy, and access to the NET Nurse service for people affected by medullary thyroid carcinoma and their families.

It most commonly starts in the adrenal glands, which sit on top of the kidneys. It can also develop in nerve tissue along the spine in the abdomen, chest, neck, or pelvis.

Neuroblastoma arises from random genetic changes in developing nerve cells during foetal or early infant development. The vast majority of cases occur by chance. Only one to two per cent are linked to an inherited genetic mutation. No environmental or lifestyle causes have been identified.

Symptoms vary depending on where the tumour is and whether it has spread. Common signs include a firm abdominal lump, dark circles around the eyes, bone pain, fatigue, fever, and poor appetite. Some children develop high blood pressure, persistent diarrhoea, or neurological symptoms such as weakness or unsteadiness.

MYCN is a gene that, when amplified (present in too many copies), drives more aggressive neuroblastoma behaviour. MYCN amplification is an important marker in risk classification and is associated with high-risk disease requiring more intensive treatment.

An MIBG (meta-iodobenzylguanidine) scan is a specialised nuclear medicine test that uses a radioactive tracer specifically absorbed by neuroblastoma cells. It maps the disease throughout the entire body and is used for diagnosis, staging, and monitoring treatment response.

Neuroblastoma is classified as low-risk, intermediate-risk, or high-risk based on the child's age, tumour stage, and tumour biology. The risk group determines how intensive the treatment needs to be.

Yes, in some infants, particularly those with MS stage disease, neuroblastoma can spontaneously shrink and disappear without treatment. This is one of the most distinctive biological features of the disease. Close monitoring is needed to determine whether a tumour will regress or requires active treatment.

High-risk neuroblastoma is treated intensively with multiple phases: induction chemotherapy, surgery, high-dose chemotherapy with stem cell rescue, radiation therapy, anti-GD2 immunotherapy, differentiation therapy with isotretinoin, and in some cases maintenance therapy. Treatment extends over many months and is coordinated by specialist paediatric oncology teams.

GD2 is a protein found on the surface of neuroblastoma cells. Anti-GD2 monoclonal antibodies such as dinutuximab and naxitamab help the immune system recognise and attack neuroblastoma cells. Anti-GD2 immunotherapy is a standard part of high-risk neuroblastoma treatment.

Opsoclonus-Myoclonus-Ataxia Syndrome (OMS) is a rare autoimmune condition associated with neuroblastoma. It causes rapid involuntary eye movements, jerky muscle spasms, and loss of coordination. OMS requires specialist neurological management and can persist beyond completion of cancer treatment.

Children treated for high-risk neuroblastoma may experience long-term effects including hearing loss, growth problems, thyroid or hormonal issues, heart or kidney effects, risk of secondary cancers, and neurodevelopmental challenges. Long-term follow-up through a survivorship programme is an important part of care.

In most cases, no. Around one to two per cent of neuroblastoma cases are linked to an inherited genetic mutation, most commonly in the ALK or PHOX2B genes. If an inherited cause is suspected, genetic counselling is recommended for the family.

Prognosis depends heavily on the risk group. Children with low-risk or intermediate-risk disease generally have very good outcomes. High-risk neuroblastoma remains challenging, with a significant risk of relapse despite intensive treatment. Research is ongoing to improve outcomes for high-risk disease.

NeuroEndocrine Cancer Australia assist with navigation to dedicated neuroblastoma organisations. provides education, resources, advocacy, and access to the NET Nurse and counsellor service for families affected by neuroblastoma.

Yes. NF1 follows an autosomal dominant inheritance pattern, meaning one copy of the altered gene is enough to cause the condition. A person with NF1 has a 50 per cent chance of passing it to each of their children. However, around 50 per cent of cases arise as a new (de novo) mutation with no family history.

NF1 is caused by a mutation in the NF1 gene on chromosome 17. This gene normally produces a protein called neurofibromin, which controls cell growth. Without functional neurofibromin, cells, particularly nerve cells, can grow abnormally and form tumours.

Café-au-lait spots are flat, light-brown skin patches. Having six or more of a specific size is one of the main diagnostic signs of NF1. They usually appear in the first year of life and are typically harmless in themselves.

Cutaneous neurofibromas are soft, benign lumps on or just under the skin. Plexiform neurofibromas are larger tumours that grow deeper in the body along major nerve pathways. Plexiform neurofibromas are more complex, can cause functional problems depending on their location, and carry a small but significant risk of becoming malignant over time.

Yes. People with NF1 have an increased lifetime risk of certain cancers, including malignant peripheral nerve sheath tumours (MPNSTs), phaeochromocytoma, breast cancer, gastrointestinal stromal tumours (GISTs), duodenal neuroendocrine tumours, and some brain tumours. Regular surveillance is an essential part of NF1 management.

A malignant peripheral nerve sheath tumour (MPNST) is an aggressive soft tissue cancer that typically develops within a pre-existing plexiform neurofibroma. People with NF1 have a lifetime risk of approximately 8 to 13 per cent of developing an MPNST. Warning signs include a rapidly enlarging tumour, new or worsening pain, or a previously soft tumour becoming hard.

Phaeochromocytoma is a tumour of the adrenal gland that produces excess stress hormones. In NF1, it is a recognised complication and can cause high blood pressure, headaches, sweating, and palpitations. It is important to screen for phaeochromocytoma before any planned surgery.

Yes. Up to half of children with NF1 experience learning difficulties, ADHD, or autism spectrum characteristics. These are not caused by intellectual disability but reflect specific processing and attention challenges. Early assessment and educational support are important.

There is no cure for NF1. Management focuses on regular monitoring and treating complications as they arise. Selumetinib, a targeted MEK inhibitor, has been approved for children with symptomatic, inoperable plexiform neurofibromas and can shrink these tumours in many cases. Surgery may be considered for troublesome or potentially malignant tumours.

Red flag symptoms include a rapidly enlarging tumour, persistent or worsening pain, a plexiform neurofibroma that becomes hard or changes texture, unexplained weight loss, new weakness or numbness in a limb, or pain that wakes a person from sleep. These may indicate malignant transformation and require prompt assessment.

NF1 is a lifelong condition and its features can change with age, particularly during puberty and pregnancy when neurofibromas may increase. The severity and complications vary greatly between individuals and cannot always be predicted, even within families.

Current clinical guidelines recommend that women with NF1 begin annual breast MRI screening from the age of 30, earlier than the general population, due to their elevated breast cancer risk.

NeuroEndocrine Cancer Australia provides education, resources, advocacy, and access to the NET Nurse service for people living with NF1 and their families.

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.

Endometrial neuroendocrine cancer develops from neuroendocrine cells in the inner lining of the uterus (the endometrium). It is even rarer than ovarian neuroendocrine cancer, is typically high-grade and aggressive, and often occurs alongside conventional endometrial adenocarcinoma.

Whether the cancer started in the ovary or spread there from another site (such as the bowel or lungs) changes the treatment approach significantly. Primary ovarian neuroendocrine cancers are treated according to gynaecological oncology protocols, while metastatic ovarian involvement may be treated as part of the underlying primary cancer. Thorough investigation is essential to make this distinction.

Symptoms often resemble more common gynaecological conditions. They may include pelvic or abdominal pain, bloating, abnormal or postmenopausal vaginal bleeding, and changes in bowel habits. Some women also develop symptoms of carcinoid syndrome, including flushing, diarrhoea, and wheezing, particularly if the cancer has spread to the liver.

Carcinoid syndrome occurs when a functional neuroendocrine tumour releases hormones into the bloodstream, typically after the cancer has spread to the liver. Symptoms include flushing, diarrhoea, and cramping. Carcinoid syndrome is managed with somatostatin analogues such as octreotide or lanreotide, and by treating the underlying tumour.

Carcinoid heart disease is a complication of longstanding carcinoid syndrome. Chronic exposure to high levels of serotonin can damage the heart valves, particularly on the right side of the heart. It requires specialist cardiology management alongside cancer treatment, with regular echocardiograms used to monitor heart valve changes.

Diagnosis involves imaging (ultrasound, MRI, CT), functional nuclear medicine scans such as Ga-68 DOTATATE PET/CT, and tissue biopsy with immunohistochemical analysis for neuroendocrine markers including chromogranin A, synaptophysin, and CD56.

Small cell neuroendocrine carcinoma (SCNEC) is a true neuroendocrine cancer treated with platinum-based chemotherapy. Small cell carcinoma of the ovary, hypercalcaemic type (SCCOHT) is not a neuroendocrine cancer: it is caused by a SMARCA4 gene mutation, typically affects younger women, and requires a different treatment approach. This distinction is critical and must be made by a specialist pathologist.

Treatment depends on the type, grade, and stage of the cancer. Surgery is the main treatment for localised disease. High-grade carcinomas are typically treated with platinum-based chemotherapy. Low-grade functional tumours may be treated with somatostatin analogues and, in selected cases, PRRT. Immunotherapy and other targeted therapies are being studied for advanced or recurrent disease.

Yes. Molecular testing, including mismatch repair (MMR) status, can help identify patients who may benefit from immunotherapy and provides prognostic information. This profiling is increasingly recommended as part of the diagnostic workup.

For low-grade, localised disease that is completely removed surgically, long-term remission or cure is possible. High-grade or metastatic disease is more difficult to treat, though advances in systemic therapy and targeted treatments are improving outcomes. Prognosis varies significantly based on grade, stage, and tumour biology.

This depends on your age, tumour type, grade, and stage. For selected young women with low-grade, early-stage ovarian neuroendocrine cancer, fertility-sparing surgery may sometimes be an option. Fertility should be discussed with the treating team before any surgery or treatment begins.

NeuroEndocrine Cancer Australia provides education, resources, advocacy, and access to the NET Nurse service for people affected by ovarian and endometrial neuroendocrine cancer.

Common prostate cancer (adenocarcinoma) grows in response to male sex hormones and can be monitored using PSA blood tests. NEPC grows independently of hormones, meaning standard hormone therapies do not necessarily work, and PSA is usually low or absent even when the cancer is active and spreading.

NEPC can arise from scratch (de novo) or, more commonly, develop as a form of treatment resistance in men who have been on long-term androgen deprivation therapy or other hormone treatments. In the latter case, cancer cells transform into a neuroendocrine phenotype to escape hormone-targeted treatment.

Pure NEPC accounts for less than 2 per cent of all prostate cancers. However, neuroendocrine transformation occurs in an estimated 10 to 17 per cent of men with metastatic castration-resistant prostate cancer after extended hormone therapy.

Because NEPC cells have lost their glandular identity, they no longer produce significant amounts of PSA. A man with NEPC may have rapidly progressing disease while his PSA blood test appears normal or stable. Other markers such as Chromogranin A and NSE may be more informative than PSA in some patients with NEPC and can be used as supportive markers during ongoing assessment.

Diagnosis requires a tissue biopsy from the prostate or a metastatic site, followed by specialist pathological review and immunohistochemical staining for neuroendocrine markers including Chromogranin A, Synaptophysin, and CD56. Imaging including CT and Gallium-68 DOTATATE PET/CT plays an important role in staging and characterising the disease.

PSMA PET scans are used for conventional prostate adenocarcinoma because PSMA is highly expressed in those cells. NEPC cells typically do not express PSMA but may have somatostatin receptors, which are detected by DOTATATE PET scanning. Not all NEPC have somatostatin receptors, and imaging findings should be interpreted alongside clinical features and histopathology.

Because NEPC generally does not respond to standard hormone therapies, treatment relies primarily on platinum-based chemotherapy combined with etoposide, similar to the treatment of small cell lung cancer. For tumours expressing somatostatin receptors, PRRT may be considered. Emerging treatments including DLL3-targeted therapies are being investigated in clinical trials.

Paraneoplastic syndromes are systemic effects caused by hormones or other substances secreted by neuroendocrine tumour cells. In NEPC, these can include Cushing's syndrome from excess ACTH, SIADH from excess antidiuretic hormone, and rarely carcinoid syndrome. These can occur even before the tumour is detected on imaging.

NEPC carries a poorer prognosis than conventional prostate adenocarcinoma due to its aggressive nature, tendency for rapid visceral spread, and resistance to standard hormone therapies. Prognosis varies based on the subtype (with well-differentiated neuroendocrine tumours carrying a more favourable outlook than small cell carcinoma), extent of spread, and response to treatment. Access to clinical trials may offer additional options.

Clinical trials investigating novel targeted therapies for NEPC are an important avenue to explore. Speaking with a specialist at a centre experienced in advanced prostate cancer and neuroendocrine tumours is the best way to identify relevant trial options.

Rectal neuroendocrine cancers are among the most frequently diagnosed gastrointestinal neuroendocrine cancers. Their detection rates have risen significantly as bowel cancer screening with colonoscopy has become more widespread.

Many rectal neuroendocrine cancers are small, low-grade, and carry an excellent prognosis when found and treated early. Larger or high-grade tumours carry a greater risk of spread and require more intensive management. The specific size and grade of the tumour are the most important factors in determining outlook.

Many rectal neuroendocrine cancers cause no symptoms and are found incidentally during colonoscopy. When symptoms do occur, they may include rectal bleeding, changes in bowel habits, rectal or anal discomfort, mucus discharge, and a feeling of incomplete bowel emptying.

Rarely. Unlike small bowel neuroendocrine cancers, rectal neuroendocrine cancers typically do not produce serotonin and therefore do not usually cause the flushing, wheezing, and diarrhoea that characterise carcinoid syndrome. This remains uncommon even in the setting of advanced or metastatic disease.

Treatment depends on tumour size and grade. Small tumours under 10 mm are usually removed endoscopically. Larger or more advanced tumours may require surgery, somatostatin analogues, PRRT, chemotherapy, or targeted therapies, depending on their grade and extent of spread.

Endoscopic resection removes the tumour through the colonoscope without external surgery and is appropriate for small, well-confined tumours. Surgery involves removing a section of the rectum and nearby lymph nodes and is required for larger, deeper, or more advanced tumours.

This is a specialised nuclear medicine scan that detects neuroendocrine tumour cells throughout the body using a tracer that binds to somatostatin receptors on neuroendocrine cells. It is an important tool for staging and monitoring well-differentiated NETs.

Follow-up recommendations after removal of a small rectal NET depend on the tumour characteristics, including size, grade, depth of invasion, lymphovascular invasion, and whether the tumour has been completely removed with clear margins. Some small, low-risk rectal NETs may not require ongoing surveillance specifically for the NET. If follow-up is recommended, the method and frequency of surveillance depend on the above factors.

Most cases are sporadic. A small number are associated with inherited genetic syndromes such as MEN1, NF1, VHL disease, and Tuberous Sclerosis Complex. If an inherited syndrome is suspected, genetic counselling is recommended.

NeuroEndocrine Cancer Australia provides education, resources, and access to the NET Nurse service and specialist NET counsellor and dietitian for people affected by rectal neuroendocrine cancer and their families.

An SDHx mutation is an inherited change in one of the genes that makes up the SDH enzyme complex, including SDHA, SDHB, SDHC, SDHD, and SDHAF2. These mutations impair the enzyme's function and significantly increase the risk of developing certain tumours.

SDHB mutations carry the highest risk of malignancy and metastatic spread among the SDH subunits. They are associated with aggressive extra-adrenal paragangliomas and SDH-deficient renal cell carcinoma.

SDH mutations are primarily associated with paragangliomas, phaeochromocytomas, SDH-deficient gastrointestinal stromal tumours (GISTs), SDH-deficient renal cell carcinoma, and, less commonly, pituitary adenomas.

Yes. All SDHx mutations follow an autosomal dominant inheritance pattern, meaning one altered copy of the gene is enough to increase the risk of developing tumours. Each child of a carrier has a 50 per cent chance of inheriting the mutation.

SDHD mutations have a unique inheritance pattern called a parent-of-origin effect. The SDHD gene copy inherited from the mother is usually switched off through a process called genomic imprinting, while the copy inherited from the father remains active. As a result, tumours most commonly develop in people who inherit an SDHD mutation from their father. People who inherited it from their mother generally do not develop tumours themselves but can pass the active mutation on to their children.

Diagnosis involves genetic testing of a blood sample to identify the specific mutation. Testing may be initiated because of a personal or family history of relevant tumours, or because a pathologist identifies SDH deficiency in tumour tissue using immunohistochemistry.

Immunohistochemistry (IHC) is a test performed on tumour tissue that looks for the presence or absence of the SDHB protein. Loss of SDHB staining in a tumour indicates SDH deficiency and helps guide further genetic testing and management.

Surveillance typically involves regular biochemical testing for catecholamine metabolites (metanephrines), whole-body MRI imaging, and for SDHB carriers, renal imaging. The timing and frequency of surveillance is guided by the specific gene subunit and specialist advice.

International guidelines generally recommend starting surveillance between the ages of 6 and 10 for SDHB carriers and between the ages of 10 and 15 for carriers of other SDH subunit mutations. The treating specialist will provide individualised recommendations.

Yes. Because SDHx mutations are inherited, first-degree relatives including children, siblings, and parents should be offered cascade genetic testing. Early identification allows surveillance to begin before tumours develop or while they are still small and manageable.

No. SDH-deficient GISTs are resistant to imatinib, which is the standard treatment for KIT-driven GISTs. They require specialist management and treatment decisions should be made by an experienced multidisciplinary team.

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.

It is exceptionally rare, with the majority of knowledge coming from small case series and individual case reports. The median age at diagnosis is around 39 years, though it can occur at any age.

There are three main types: primary TNETs that arise directly within the testicle, secondary TNETs that represent spread from a neuroendocrine primary elsewhere in the body, and TNETs associated with a teratoma. Determining which type is present is critical, as management differs significantly.

The most common presentation is a painless, firm lump or swelling in one testicle, sometimes with a sense of heaviness or mild scrotal discomfort. Systemic hormone symptoms such as flushing, diarrhoea, and wheezing (carcinoid syndrome) are uncommon but can occur, usually when the disease has spread.

Usually not. The standard testicular tumour markers including AFP and beta-hCG are typically normal in testicular neuroendocrine cancerss. Diagnosis relies on imaging, neuroendocrine-specific biochemical tests such as Chromogranin A and urinary 5-HIAA, and pathological examination of the removed tumour.

Surgical removal of the testicle is performed through an inguinal (groin) incision to avoid disrupting normal lymphatic drainage pathways. If a scrotal incision were used, it could redirect lymphatic spread and compromise subsequent staging and treatment. This approach is standard for all testicular cancers.

In most cases, testosterone production and fertility are maintained by the remaining testicle after removal of one testicle. However, individual outcomes vary and anyone with specific concerns should discuss fertility preservation options and hormonal monitoring with their treating team before surgery.

Carcinoid syndrome, which includes flushing, diarrhoea, and wheezing, can occur but is uncommon in primary testicular neuroendocrine cancer. When it does occur, it usually indicates the tumour is secreting serotonin and that the disease has spread, typically to the liver.

Primary localised testicular neuroendocrine cancers generally have a favourable prognosis. Five-year survival rates reported in the literature are in the range of 78 to 85 per cent overall, with better outcomes for localised, low-grade disease treated with complete surgical removal. Long-term follow-up is important because slow-growing neuroendocrine tumours can occasionally recur years after surgery.

Yes. Long-term follow-up is recommended after treatment for testicular neuroendocrine cancer because some tumours can recur or spread, sometimes after a prolonged period. The type and frequency of surveillance depends on individual tumour characteristics, including tumour size, grade, stage, and whether there is evidence of disease elsewhere in the body. Follow-up may include clinical review, imaging, and selected biochemical tests where appropriate.

Primary testicular neuroendocrine cancers are almost always sporadic, occurring without a clear inherited genetic cause. They are not typically associated with hereditary syndromes such as MEN1 or VHL disease.

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.

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.