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Neuroendocrine Tumors FRCR CO2A Flashcards

1
Q

What are neuroendocrine tumours (NETs)?

A

NETs are a heterogeneous group of neoplasms with significant variation in their mode of presentation and biological behaviour.

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2
Q

From which cells do neuroendocrine tumours arise?

A

They arise from neuroendocrine cells, which are widely distributed in the body.

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3
Q

Where do NETs typically arise?

A

NETs can arise within the bronchial or gastroenteropancreatic systems.

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4
Q

What are carcinoid tumours?

A

Carcinoid tumours are historically termed NETs arising within the bronchial or gastroenteropancreatic systems.

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5
Q

How is the management of NETs improving?

A

Management is improving due to advances in imaging and the increased use of multidisciplinary teams in specialist centres.

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6
Q

What are some treatment options for NETs?

A

Treatment options may include surgery, medical therapy, chemotherapy, radionuclide therapy, ablative therapy, and embolisation.

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7
Q

What types of medical therapies are used for NETs?

A

Medical therapies include somatostatin analogues, interferon alpha, sunitinib, and everolimus.

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8
Q

What are current clinical trials focused on for NETs?

A

Current clinical trials are focused on defining the optimal use of existing therapies and exploring novel agents such as angiogenesis and MTOR inhibitors.

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9
Q
A
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10
Q

How are NETs classified?

A

NETs are classified according to histological differentiation and grading, and staging based on primary tumour site.

This classification is essential for determining prognosis and treatment options.

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11
Q

What is the basis of the WHO 2010 classification of NETs?

A

The WHO 2010 classification is based on the concept that all NETs have malignant potential and is organised according to grade and stage.

This classification helps in understanding the biological behavior of NETs.

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12
Q

What factors are considered for grading NETs?

A

Grading is based upon morphological criteria and the proliferative activity of the tumour, including mitotic activity and/or Ki-67 labelling index.

These factors are critical in assessing the aggressiveness of the tumour.

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13
Q

What are the categories for well-differentiated tumours?

A

Well-differentiated tumours are divided into low-grade (G1) and intermediate-grade (G2) categories.

This differentiation helps in treatment planning.

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14
Q

How are poorly differentiated NETs classified?

A

All poorly differentiated NETs are classified as high-grade (G3) neuroendocrine carcinomas.

High-grade tumours typically have a worse prognosis.

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15
Q

What staging systems are used for NETs?

A

Staging systems include the Union for International Cancer Control (UICC) 7th edition and the European Neuroendocrine Tumor Society (ENETS) staging systems.

These systems provide a framework for assessing the extent of disease.

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16
Q

What is the estimated annual incidence of NETs in the UK?

A

The estimated annual incidence is 2–5 cases per 100,000 patients.

This statistic reflects the growing recognition of the disease.

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17
Q

Is there a gender predominance in NETs incidence?

A

Yes, there is a slight female predominance.

Understanding gender differences can aid in targeted research and treatment.

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18
Q

What is the average age at diagnosis for NETs?

A

The average age at diagnosis is 61 years.

This information can guide screening and awareness efforts.

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19
Q

What do autopsy studies reveal about NETs?

A

Autopsy studies indicate that small, clinically unrecognised NETs are relatively common, with up to 10% for pancreatic NETs.

This highlights the importance of improved diagnostic techniques.

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20
Q

Are there geographical variations in NETs incidence?

A

There is no definite geographical variation in tumour incidence, although there are reported ethnic differences.

African-Americans have the highest incidence of 6.5 per 100,000 per year.

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21
Q

What is the general understanding of the risk factors for NETs?

A

Poorly understood

Most NETs are sporadic, but there is a small familial risk.

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22
Q

What is the increased risk of developing NETs with one affected first-degree relative?

A

4-fold increased risk

The risk increases to 12-fold with two affected first-degree relatives.

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23
Q

What inherited cancer syndrome is associated with parathyroid and pituitary adenomas, pancreatic NETs, and bronchial/gastric NETs?

A

Multiple endocrine neoplasia type 1 (MEN1)

MEN1 includes various types of tumors.

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24
Q

What conditions are associated with multiple endocrine neoplasia type 2 (MEN2)?

A

Hyperparathyroidism, medullary thyroid carcinoma (MTC), phaeochromocytoma

MTC is discussed in Chapter 38.

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25
Which inherited cancer syndrome is characterized by neurofibromas, café-au-lait macules, and optic glioma?
Type 1 neurofibromatosis (NF1) ## Footnote NF1 can also include phaeochromocytoma and rarely duodenal somatostatinoma.
26
What syndrome is associated with renal cell carcinoma, phaeochromocytoma, and pancreatic NETs?
Von Hippel Lindau syndrome (VHL) ## Footnote VHL may also include cerebellar haemangioblastoma and retinal angioma.
27
What are the key features of Carney complex?
Spotty skin pigmentation, cardiac myxomas, thyroid adenoma, nodular adrenocortical disease, sertoli cell tumours, ovarian cysts ## Footnote Carney complex can cause Cushing’s syndrome.
28
What should be undertaken in all cases of NET?
Family history and clinical examination for inherited cancer syndrome ## Footnote This is crucial for identifying potential hereditary links.
29
What is the macroscopic appearance of NETs?
Typically solid and yellow, reflecting high lipid content.
30
How do NETs typically appear microscopically?
Often trabecular, glandular, or forming rosettes with granular cytoplasm and round nuclei.
31
What is the proliferative potential of most NETs?
Majority display low proliferative potential with infrequent mitoses.
32
What is the significance of poorly differentiated NETs?
A proportion of NETs may be poorly differentiated.
33
What confirms the neuroendocrine origin of tumours?
Immunohistochemistry against neuroendocrine markers like chromogranin A, synaptophysin, and PGP9.5.
34
What specific immunohistochemical analysis may be valuable for NETs?
Analysis of hormone production, such as glucagon for glucagonomas or ACTH for ectopic ACTH production.
35
What is the Ki-67 index used for in NETs?
To estimate proliferative potential and has prognostic relevance.
36
What factors may influence the choice of therapy for NETs?
Proliferative potential indicated by the Ki-67 index.
37
Where may NETs spread in the body?
To regional lymph nodes, liver, lungs, or bone.
38
What does the presence of bony metastases indicate?
Generally a feature of advanced disease.
39
How do NETs often present clinically?
Many are asymptomatic and discovered incidentally.
40
What symptoms may suggest bowel obstruction in NET patients?
Symptoms suggestive of bowel obstruction even if abdominal radiology is normal.
41
What is classic carcinoid syndrome?
A presenting feature in around 20% of cases of intestinal NETs occurring when vasogenic amines and peptides, including serotonin and tachykinins, gain access to the systemic circulation ## Footnote Typically seen in intestinal NETs with liver metastases.
42
What are the common features of carcinoid syndrome?
* Dry flushing (90%) * Diarrhoea (70%) * Abdominal pain (40%) * Wheezing (25%) * Valvular heart disease (30%) * Pellagra (5%) ## Footnote Some patients may also experience increased lacrimation and rhinorrhoea.
43
What characterizes the carcinoid flush?
Usually pink, lasts for a few minutes, and involves the face and upper trunk ## Footnote Triggers may include alcohol, bananas, walnuts, or chocolate.
44
What is carcinoid crisis?
An extreme presentation characterized by fluctuating blood pressure, tachycardia, arrhythmias, profound flushing, and bronchospasm ## Footnote May be precipitated by tumour lysis, tumour handling, or anaesthesia.
45
What is the most common valvular abnormality in carcinoid heart disease?
Tricuspid regurgitation ## Footnote Left-sided problems can occur if a patent foramen ovale is present.
46
What percentage of patients present with carcinoid heart disease?
Up to 20% ## Footnote Often affects the right side of the heart.
47
What symptoms are suggestive of pulmonary NETs?
* Bronchial obstruction * Pneumonia * Dyspnoea * Pleuritic pain * Cough * Haemoptysis ## Footnote Many discovered incidentally on chest X-ray.
48
What are examples of ectopic hormone production caused by bronchial, thymic, and pancreatic NETs?
* Cushing’s syndrome from ectopic ACTH secretion * Acromegaly from ectopic growth hormone-releasing hormone production * Syndrome of inappropriate anti-diuretic hormone secretion ## Footnote These conditions can arise from ectopic hormone production.
49
What clinical features may pancreatic NETs present with?
* Hypersecretory syndrome * Abdominal pain * Weight loss * Symptoms related to metastases ## Footnote Their clinical features are detailed in specific tables.
50
What are the components of diagnosis and staging of NETs?
* Clinical symptoms * Biochemistry (general and specific neuroendocrine/hormone markers) * Radiological and nuclear imaging * Histological confirmation ## Footnote Histological confirmation is considered the gold standard.
51
What do biochemical tests in neuroendocrine tumours help establish?
Diagnosis and monitoring response to therapy ## Footnote Biochemical tests include general neuroendocrine markers and specific hormones.
52
What is plasma chromogranin A (CgA) used for?
A general tumour marker for neuroendocrine tumours (NETs) ## Footnote High sensitivity in all types of NETs, especially metastatic intestinal NETs.
53
What can cause false-positive elevations of CgA?
Renal impairment, hypergastrinaemia, proton pump inhibitors ## Footnote These factors can lead to misleading CgA test results.
54
What is the significance of 24-hour urinary 5-hydroxyindoleacetic acid (5-HIAA)?
It is a metabolite of serotonin useful in diagnosing intestinal NETs ## Footnote Has up to 70% sensitivity in metastatic disease.
55
What should be excluded before measuring 5-HIAA?
Various drugs and foodstuffs for 3 days before and during urine collection ## Footnote This exclusion is necessary to ensure accurate results.
56
What additional marker can be useful for pancreatic NETs?
Pancreatic polypeptide (PP) ## Footnote Especially useful when CgA is in the normal range.
57
What should complement plasma CgA measurement in suspected pancreatic NETs?
A fasting gut hormone profile ## Footnote Blood should be collected in a specific way to avoid false-positive results.
58
What imaging modalities are used for evaluating neuroendocrine tumours?
CT scans, MRI, EUS, SSRS, PET scans ## Footnote Each imaging method has specific applications and sensitivities.
59
How does somatostatin receptor scintigraphy (SSRS) help in NETs?
High sensitivity in locating primary NETs and assessing metastatic disease ## Footnote Less effective for insulino- mas, where sensitivity is below 50%.
60
What is the role of PET scanning in NETs?
Increasingly used for imaging NETs, limited by scanner availability ## Footnote 68Ga-labelled somatostatin analogues have high sensitivity.
61
What is the main aim of treatment for neuroendocrine tumours?
Cure the patient if possible, otherwise palliative care ## Footnote Palliative care aims to maintain quality of life.
62
What is the role of surgery in treating neuroendocrine tumours?
Only treatment that can achieve a cure; may also debulk tumours ## Footnote Surgical management is complex and varies by tumour type.
63
What should be assessed preoperatively in patients with functioning midgut NETs?
Presence of carcinoid heart disease ## Footnote Patients should receive octreotide to minimize risk of crisis.
64
What type of NETs require medical therapy and endoscopic resection?
Type 1 and Type 2 gastric NETs ## Footnote Type 3 gastric NETs are more aggressive and usually require gastrectomy.
65
What is somatostatin analogue therapy used for?
Symptomatic control in gastroenteropancreatic NETs ## Footnote Octreotide has a longer half-life than endogenous somatostatin.
66
What are the side effects of somatostatin analogue therapy?
Diarrhoea, steatorrhoea, abdominal discomfort, flatulence, anorexia, nausea ## Footnote Side effects are usually mild and diminish over time.
67
What is the role of interferon alfa in NET treatment?
Used in treatment but not widely due to conflicting data and tolerability issues ## Footnote May show biochemical and symptomatic response in 40-70% of patients.
68
What chemotherapy regimen may be effective in poorly differentiated NETs?
Cisplatin and etoposide-based combinations ## Footnote May achieve up to 70% response rate but with limited duration.
69
What is the significance of the PROMID study?
Confirmed that octreotide LAR® slows time to tumour progression in metastatic intestinal NETs ## Footnote Median progression time was 14 months compared to 6 months with placebo.
70
True or False: Somatostatin analogues are only used for functioning NETs.
False ## Footnote They are also considered for non-functioning NETs.
71
Fill in the blank: The mainstay of medical therapy in gastrinomas is _______.
Proton-pump inhibitors ## Footnote There is no evidence for added benefit from somatostatin analogue therapy.
72
What is the response rate for cisplatin and etoposide-based combinations in neuroendocrine tumors?
Up to 70% response rate ## Footnote Duration of response may not extend beyond 8–9 months.
73
What are the common treatments for pulmonary NETs?
Combinations of platinum and etoposide ## Footnote Treatment decisions should be based on individual tumor characteristics and radiological progression.
74
What is the reported response rate for pancreatic NETs?
40–70% ## Footnote The optimal regimen has not yet been determined.
75
Which drugs have been used in combination for pancreatic NETs?
* Streptozocin * 5-fluorouracil * Doxorubicin * Cisplatin * Dacarbazine * Adriamycin ## Footnote Temozolomide has been used with capecitabine, reporting up to 70% response rate.
76
What are the two new treatment options for advanced, progressive pancreatic NETs?
* Sunitinib * Everolimus ## Footnote Both have been approved as licensed therapies.
77
What was the effect of sunitinib in clinical trials for pancreatic NETs?
Improved progression-free and overall survival ## Footnote Toxicities included neutropenia and hypertension.
78
What adverse events were associated with everolimus treatment?
* Glucose intolerance * Stomatitis * Diarrhea * Infections ## Footnote It improved progression-free survival in patients with advanced low-grade or intermediate-grade pancreatic NETs.
79
What is the role of conventional external beam radiotherapy in treating NETs?
Limited role except for analgesic benefits in bony metastases ## Footnote Indolent NETs are relatively radioresistant.
80
What is Peptide-receptor radionuclide therapy (PRRT) used for?
Symptomatic patients with unresectable or metastatic progressive disease ## Footnote Requires abnormal uptake of corresponding imaging compound.
81
Which radioisotopes have been used in PRRT?
* 131I-MIBG * 90Y-DOTATOC * 90Y-DOTATATE * 177Lu-DOTATATE ## Footnote Beneficial effects include symptom palliation and improved quality of life.
82
What is the aim of embolisation therapy in NETs?
Control symptoms and reduce tumor size ## Footnote Indicated for patients with multiple and hormonally active liver metastases not amenable to surgical resection.
83
What are the potential side effects of embolisation therapy?
* Mortality of up to 4–7% * Postembolisation syndrome * Carcinoid crisis * Hepatic abscess formation (5%) ## Footnote Risk of carcinoid crisis should be minimized with octreotide infusion.
84
What is the main limitation of radiofrequency ablation?
Size, accessibility, and number of tumors ## Footnote It improves symptoms when at least 90% of the visible tumor is ablated.
85
What is Selective Internal Radiation Microsphere Therapy (SIRT) used for?
Patients with NET liver metastases ## Footnote Administers radioactive 90Y resin microspheres through a hepatic artery catheter.
86
How does prognosis vary in NETs?
Dependent on histological features, tumor size, location, and stage ## Footnote Overall 5-year survival varies from 20% to 80%.
87
What is the 5-year survival rate for rectal NETs?
74–88% ## Footnote Carries the best prognosis among gut tumors.
88
What is the prognosis for pancreatic NETs?
Variable, with overall 5-year survival in the order of 30–45% ## Footnote Depends on tumor type.
89
What is an area of current interest in NET research?
Molecular biology of NETs ## Footnote Conventional searches for mutations have been largely disappointing.
90
What types of studies are exploring new diagnostic and treatment strategies for NETs?
Large-scale genomic approaches ## Footnote Includes exome sequencing, DNA methylation, RNA expression, and copy number analyses.
91
What is the NET01 study about?
Comparing capecitabine plus streptozocin with or without cisplatin ## Footnote Focuses on unresectable or metastatic gastroenteropancreatic neuroendocrine tumors.
92
What is the RADIANT-4 study?
A phase III study of everolimus plus best supportive care versus placebo ## Footnote Targets patients with advanced NETs of gastrointestinal or lung origin.
93
MEN I
Parathyroid and pituitary adenomas Pancreatic NETs Bronchial/gastric NETs
94
MEN 2
Hyperparathyroidism Medullary carcinoma thyroid phaeochromocytoma
95
VHL syndrome
RCC Phaeochromocytomas Cerebellar hemangioblastomas retinal angioma pancreatic NETs
96
Carney Complex
spotty skin pigmentation cardiac myxomas throid adenomas nodular adrenocortical disease causing cushing's syndrome, sertoli cell tumors and ovarian cysts

FRCR CO2A (35 decks)

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