The presentation show the various case studies of patients suffering from these disease

1. How do I diagnose NETs?
G N Ramesh
ASTER Medcity
Cochin

3. Nomenclature
• Arising from Enterochromaffin cells
• Differentiation / grading
• ‘Carcinoids’ are the well differentiated tumors
• Poorly differentiated tumors are referred to as
Neurendocrine carcinomas – small cell or
large cell .
• Further nomenclatures are related to the
origin and stage – foregut , midgut , hindgut ,
pancreatic , metastatic , functioning / non-
functioning

5. Classification by origin

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Size is important

10. Clinical presentations

13. Case 1 : RB
• 56 yr old male with generalised abdominal
pain . OGD – ‘severe PUD with gastric ulcers”
• Repeat OGD – large mass at angularis ,
erythematous mucosa with shallow ulceration
• CT – distal gastric mass - submucosal
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18. When to suspect?
• Multiple gastric lesions
• Gastric lesions in patients with pernicious
anemia / chronic atrophic gastritis / MENs
• Gastric growth which is not an
adenocarcinoma

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26. Jejunoileal NETs
• Increased detection on endoscopy and
imaging
• 60s and 70s ; arise from intraepithelial
endocrine cells
• Most commonly located – distal 60 cms of
ileum
• Abd pain 40% ; intermittent obstruction 25% ;
duodenal/biliary obstruction , intussusception
• Metastasis – liver 47% if primary >2 cms ;
nodes 58% if primary > 1 cm

27. Appendix NET
• Most common neoplasm of the appendix
• Incidental detection ; most often tip / distal
third of appendix ; 10 % base of appendix –
obstruction
• 40s – 50s ( younger profile) – appendectomy
related ; younger women who undergo pelvic
surgeries
• Carcinoid syndrome related to metastasis

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40. Midgut NETs – when to suspect?
• Vague small bowel pain with mass
• Carcinoid syndrome
• Appendicitis with mass
• Base of appendix tumor
• Intussusception / mass with desmoplastic
reaction
• Ulcerated small bowel growth
• Multiple small bowel lesions

41. Hindgut NETs
• Usually nonsecretory , not associated with
carcinoid syndrome even when metastatic
• Symptoms – mimic adenoca - altered bowel
habits ; obstruction ; bleeding

42. Colonic NETs
• Elderly patients – 70s
• Presentation – usually ‘adenoca like’
• Rarely functional
• Majority – rt colon particularly caecum
• Symptoms related to size – ave size at
detection – 5 cms
• 2/3 – local nodal / distant metastasis

43. Rectal NETs
• Asymptomatic – found on colonoscopy
• 60s
• Uncommon manifestations – bleeding ; changed
bowel habits ; pain ;
• Carcinoid syndrome very rare
• 75-85% are localised – no mets
• Size more than 2 cms – 25% metastasise to liver
• Poor prognosis related to size , invasion into
muscularis propria , lymphovascular invasion , high
mitotic rate ( > 2 per 50 HPF)

49. Hind gut NETs – when to suspect?
• Colonic growth that is not an adenoca
• Rectal polyps ( submucosal) with or without
ulceration
• Small colonic / rectal primary with multiple
large liver mets

62. When to suspect PNETs?
• Clinical syndromes
• Well defined rounded lesions in the pancreas
• Symptoms of excessive hormone production

63. Suspicion
Clinical Imaging – endoscopic/radiologic
Staging / origin CT / MRI + EUS DOTATATE scanning
Histology Resection / FNA / Biopsy
Markers Chromogranin A / Pancreatic polypeptide / both
Functioning / non-functioning Insulin / glucagon / VIP / gastrin

69. CT
• Most NETs are highly vascular ; enhance in
arterial phase (20s ) ; washout in portal
venous phase (70s ) .
• > 80% sensitivity
• Small tumors – rounded enhancing lesions ,
some may be hypodense or cystic .
• Non-functionin symptomatic lesions are often
larger > 3 cms

70. MRI
• Typically – low signals on T1 ; high signals on
T2
• Sensitivity 85% ; specificty 100% ; PPV 100% ;
NPV 73%
• Better for hepatic lesions

78. 68 Ga – DOTATATE PET-CT imaging
• Improved detection and staging on P-NETs
• Increased sensitivity for smaller lesions
• Higher spatial resolution
• Preferred over OCTREOSCAN

84. More studies
• Transhepatic portal venous sampling ( THPV)
• Arterial stimulation and venous sampling
( ASVS )
• Intraoperative Ultrasound
• Hormonal studies

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