If this is where you are
Newly diagnosedReports arriving, no plan yetAlready tested elsewhereYou have results you cannot readOn treatment nowYou want to know it is workingOr
Finished treatmentWatching for anything left behindWorried about familyInherited risk, and relativesWanting a second opinionA decision you are unsure aboutDecide
Blueprint CareThe flagship decision report and a dedicated clinical teamSignalOne free question answered on WhatsApp within four hoursNavigationRecords you already have, turned into a clarity timelineDecipherInterpretation of sequencing already done elsewhereTest
Signature, all tiersSolid tumour and blood cancer panels comparedSignature STb33 genes from a blood drawSignature STb O118 genes from a blood drawSignature STb O+523 genes, with TMB and MSISignature STt OProfiling from an existing tissue blockMonitor
SentinelSerial monitoring while treatment is runningSentinel SolidctDNA monitoring for solid tumoursClearMolecular residual disease surveillanceClear SolidTumour-informed surveillanceProtect
HeritageInherited risk, assessed properlyHeritage CoreA broad inherited-risk assessmentHeritage AdhocFocused testing for named relativesCancer DictionaryWhat is discussed in your specific cancerReference
Cancer DictionaryOne structured entry per cancer typeBiomarker LibraryOne page per marker, in plain languagePatient ResourcesGlossary, guides and how to use themWatch, read and ask
Video LibraryShort explainers, captioned and translatedStoriesPatient and clinician experiences, consentedFAQThe questions we are asked mostCancer Dictionary · Other and unknown primary
Neuroendocrine neoplasms are graded by proliferation rate and separated into well-differentiated tumours, which may grow slowly over many years, and poorly differentiated carcinomas, which behave aggressively. Site of origin, grade and somatostatin receptor expression drive the discussion more than genomic profiling does.
Also called NET, Neuroendocrine tumour, Carcinoid, Neuroendocrine carcinoma.
Start here
The distinction between a well-differentiated neuroendocrine tumour and a poorly differentiated neuroendocrine carcinoma separates two entirely different diseases that share a name, and getting it right is the first and most consequential step.
Neuroendocrine neoplasms are graded by proliferation rate and separated into well-differentiated tumours, which may grow slowly over many years, and poorly differentiated carcinomas, which behave aggressively. Site of origin, grade and somatostatin receptor expression drive the discussion more than genomic profiling does.
01Before any molecular question
Usually established first: site of origin, differentiation, grade based on Ki-67 index and mitotic rate, and functional status, meaning whether the tumour secretes hormones causing symptoms. Somatostatin receptor imaging is central to both staging and treatment planning in well-differentiated disease.
Usually already established
02
The divisions below are not labels. Each one changes what the treatment discussion is actually about.
Well-differentiated neuroendocrine tumour, grade 1 and 2
Often slow growing, sometimes over many years. Somatostatin receptor expression is central.
Well-differentiated neuroendocrine tumour, grade 3
Higher proliferation but retaining well-differentiated features. A distinct category from neuroendocrine carcinoma, and frequently confused with it.
Poorly differentiated neuroendocrine carcinoma
Aggressive, and managed more like small cell lung cancer than like a well-differentiated tumour.
Functional tumours
Secreting hormones and causing distinct clinical syndromes, which need managing alongside the tumour.
Pancreatic neuroendocrine tumours
A distinct group with their own molecular profile and their own inherited-risk associations.
03What is discussed
Pancreatic neuroendocrine tumours commonly carry MEN1, DAXX and ATRX alterations, and mTOR pathway alterations occur. Small intestinal neuroendocrine tumours have relatively quiet genomes, with chromosome 18 loss common. Poorly differentiated neuroendocrine carcinomas frequently carry TP53 and RB1 alterations, resembling small cell lung cancer. Genomic profiling changes management less often here than grade, site and somatostatin receptor status do, and setting that expectation matters.
Ki-67 index
A measure of proliferation. Central to grading, and not a genomic test.
Somatostatin receptor expression
Assessed by imaging. Determines eligibility for specific treatment approaches.
MEN1
Relevant in pancreatic neuroendocrine tumours, and raises an inherited-risk question.
DAXX and ATRX
Commonly altered in pancreatic neuroendocrine tumours.
TP53 and RB1
Characteristic of poorly differentiated neuroendocrine carcinoma.
Chromogranin A
A circulating marker, used with caution given its limitations.
This is not an exhaustive list, and which markers are relevant depends on the individual case. Not every marker listed is assessed in every patient.
04What is tested, and from what
Grading and somatostatin receptor imaging carry more weight than genomic profiling in well-differentiated disease. Profiling is discussed in poorly differentiated carcinoma and in advanced disease where trial options are being sought. Where the site of origin is unknown, profiling and specialised immunohistochemistry can help establish it.
Several inherited syndromes are associated: multiple endocrine neoplasia type 1 (MEN1), von Hippel-Lindau, neurofibromatosis type 1 and tuberous sclerosis. Pancreatic neuroendocrine tumours, multiple tumours, young age, or associated endocrine abnormalities are prompts for genetic evaluation. Phaeochromocytoma and paraganglioma, related neuroendocrine tumours, have a particularly high rate of inherited causes, and germline testing is discussed for essentially all of them.
Cancer genetics and hereditary riskctDNA has a limited established role. Circulating markers including chromogranin A and multi-analyte blood tests are used with caution, and imaging remains the primary means of assessing disease over time.
More on ctDNA monitoring05The timing question
Testing that arrives after the decision it was meant to inform has already been made is a common and avoidable problem. This is the sequence in which molecular information tends to become relevant.
06Take these to your team
When a decision is being made
Questions worth raising with a treating team.
At progression, recurrence or resistance
The molecular picture at diagnosis is not always the molecular picture later.
07What shapes eligibility
Eligibility is usually written around differentiation, grade, site of origin, somatostatin receptor status and prior therapy. The distinction between grade 3 well-differentiated tumour and neuroendocrine carcinoma is frequently decisive.
| Term | What it means |
|---|---|
| Well-differentiated | Retaining the appearance and behaviour of the normal cell type. Generally slower growing. |
| Ki-67 index | The percentage of cells actively dividing. The basis of grading. |
| Functional tumour | One that secretes hormones causing clinical symptoms. |
| Somatostatin receptor | A receptor on many neuroendocrine tumour cells, used for both imaging and treatment. |
These are the services most often relevant to this cancer. Which of them applies is a clinical question, not an automatic one.
This entry is educational. It describes what is commonly discussed in this cancer, not what should happen for any individual. Which of it applies depends on the specific diagnosis, stage, prior treatment and the person themselves. Nothing here is a recommendation, and no list of biomarkers here is exhaustive. Decisions belong with a treating team who know the whole case.
Take the next step
A no-obligation conversation with our care team, arranged through KPCIRC.
Book a consultationCommission your decision report and a dedicated clinical team.
Explore Blueprint CareSignal replies to your first question within four hours, at no cost.
Start with Signal