Neuroendocrine Tumours Flashcards Preview

Y3M - General Surgery > Neuroendocrine Tumours > Flashcards

Flashcards in Neuroendocrine Tumours Deck (20)
Loading flashcards...
1
Q

What are neuroendocrine cells?

A

Cells that receive input from neurotransmitters to release hormones into the blood stream.

This allows communication between nervous system and endocrine systems.

2
Q

What neuroendocrine tumours can be found in the tubular GI tract and pancreas?

A

Gastroenteropancreatic Neuroendocrine tumours (GEP-NETs)

They all have malignant potential

3
Q

Where are most GEP-NETs found?

A

In the small intestine

Rest are in rectum or in the stomach

4
Q

Grading classification of GEP-NETs

A

Grade 1 - Well differentiated, mitotic count <2 per 10 HPF, KI index <3%

Grade 2 - Well differentiated, mitotic count 2-20 per 10 HPF, KI index 3-20%

Grade 3 - Poorly differentiated, mitotic count >20 per 10HPF, KI index >20%

5
Q

Risk factors

A

Genetic

Multiple Endocrine Neoplasia Type 1 (MEN1)

Von Hippel-Lindau disease (VHL)

Neurofibromatosis (NF-1)

Tuberous sclerosis complex (TSC)

6
Q

Clinical features

A

Often non-specific symptoms like vague abdo pain, N+V

Abdo distension

Can have bowel obstruction

Unintentional weight loss

Palpable abdo mass

Also assess for underlying inherited disorder.

7
Q

How can GEP-NETs be classified?

A

Funcitioning or non-functioning depending on evidence of hormonal hypersecretion.

Majority are non-functioning.

8
Q

What can well-differentiated midgut NETs present as?

A

Carcinoid syndrome

9
Q

Explain carcinoid syndrome

A

Well-differentiated GEP-NETs can spread and lead to carcinoid syndrome developing.

It occurs following metastasis of a carcinoid tmour.

The metastis will start secreting mediators like serotonin, prostaglandin or gastrin e.g.

This can lead to flushing, abdo pain, diarrhoea, wheezing or palpitations.

10
Q

Laboratory tests for GEP-NET

A

Chromogranin A

5-HIAA levels

FBC + LFTs and other routine bloods.

Chromogranin B and pancreatic peptide might be useful as well.

Genetic testing can also be done if clinical history suggests it.

11
Q

Specialised tests if bloods are suggestive of GEP-NET

A

Depending on the location endoscopy should be used.

CT enteroclysis is the imaging of choice in case it is a small bowel NET

Metastatic disease warrants whole body somatostatin receptor scintigraphy (SSRS)

12
Q

Management

A

Surgery is the only curative treatment

Metastatic disease is a common presentation which means that any surgical treatment would only be palliative.

13
Q

Management of poorly differentiated GEP-NETs

A

Poor prognosis but if the disease is localised surgical resection -> chemotherapy can be done.

In metastatic dsiease palliative chemo alone is advised

14
Q

Management of well-differentiated GEP-NETs

A

Managed according to site, staging and functionality.

Localised -> resected

Liver metastases can also be resected along with the primary tumour

15
Q

Surgical management of gastric NETs

A

Depends on subtype.

Type 1 and Type 2 have low metastatic potential -> endoscopic resection + annual surveillance.

Type 3 -> Aggressive lesions which requires partial or total gastrectomy with lymph node clearance.

16
Q

Surgical management of small intestinal NETs

A

Almost always malignant.

Resection of the tumour with mesenteric LN clearance is done

17
Q

Management of appendiceal NETs

A

Usually more benign

Management depends on size

>2cm tumours -> appendicectomy with right hemicolectomy

<2cm may be only appendicectomy

18
Q

Colonic NETs

A

Worse prognosis than GI NETs

Treatment is usually partial colectomy and regional LN clearance

19
Q

Explain carcinoid crisis

A

Caused by an overwhelming release of hormones from NET leading to resistant severe hypotension.

It can happen during surgical procedure intra- or post-operatively

20
Q

Prophylaxis of carcinoid crisis

A

Somatostatin analogues

Octreotide can be started 24h prior to the operation and continued for 48h post-op