1. NET MASTERCLASS
GI-NEUROENDOCRINE TUMORS: THERAPEUTIC APPROACHES: SYMPTOMATIC MANAGEMENT
IOANNIS PILPILIDIS, MD, FEBGH
GASTROENTEROLOGY – ONCOLOGY DEPT
“THEAGENEIO” CANCER HOSPITAL OF THESSALONIKI

2. Long – term survival: A chronic disease
Yao, JCO 2008; 26:

3. < 10 ή > 10

5. Common symptoms are not typical

6. Typical symptoms are not common

7. Diagnosis according to the primary
Appediceal (8) 100% surgery
Upper GI track (duodenal) (31) 100% endoscopy (anemia, dyspepsia)
SI (18) 10% incidentaloma
15% liver biopsy
60% obstructive symptoms
15% diarrhea – flushing
P-NET (9) 20% hypoglycemia (2)
10% jaundice (1) % pain (4)
10% rash (1)
10% weight loss (1)
Colon – rectal (4) 25% colon – abdominal pain
75% rectal - incidentaloma

8. 3.000 years of misdiagnosis ΜΕΝ I Pituitary tumor Hyperparathyroidism
God’s help!!!
ΜΕΝ I
Pituitary tumor
Hyperparathyroidism
Insulnoma
Gene’s help!!!
MENIN GENE: 11q13

10. Liver mets
Peritoneal disease/ retroperitoneal involvement
ovary/testicular

11. TREATMENT DOES NOT NEED A DIAGNOSIS

14. Symptoms presentation-
Functionality/hormone activity
carcinoid syndrome
gastrin, insulin, etc
paraneoplastic syndromes
Location of primary
SI (peritoneal fibrosis, heart disease, etc)
p-NET
Tumor stage (tumor load, liver mets etc)

15. Symptoms presentation
Functionality/hormone activity
deal with symptoms even if no effective tumor treatment
Location of primary
Tumor stage (tumor load, liver mets etc)

19. Differential diagnosis:
Diarrhea: hormone secretion
bile acids
bacterial overgrowth
short bowel syndrome
intestinal bypass
pancreatic insufficiency (SSAs treatment)
Hypoglycemia: insulinoma ?
IGF-1, pro-IGF2
Hypergastrinemia: PTH ? (secondary hyperPTH)
HyperPTH: Gastrinoma ? (gastrin secretion)

20. Carcinoid syndrome: response to somatostatin analogues
Clinical response %
Biological response %
Objective response 0-5%

28. Symptoms presentation
Functionality/hormone activity
Location of primary
deal with complications
Tumor stage (tumor load, liver mets etc)

32. Patient selection bias?

33. Median Survival: 8.5y
Median Survival (non cytoreduction): y vs 11.5y
5-y Survival 75%

38. In the absence of other data, the resection of the primary tumour only for patients with PNETs and unresectable metastatic liver disease should only be considered in
high-volume referral centres, with strict selection criteria and in a multidisciplinary setting of patients’ care

40. Symptoms presentation
Functionality/hormone activity
Location of primary
Tumor stage (tumor load, liver mets etc)
cytoreduction

41. TACE/TAE: Symptom Control
Clinical response: 67% - 100%
Biological response: 46% - 91%
Ruszniewski Cancer 1993, Therasse Radiol 1993, Tomassetti 1994, Diaco Am J Surg 1995, Ruszniewski Eur J Gastroenterol Hepatol 2000, Roche Hepatogastroenterol 2004, Ruszniewski 2007
PRRT: Symptom Control
Global Health QoL = 36%
Fatigue = 49%
Nause – Vomiting = 70%
Pain = 53%
Dyspnoea = 44%
Insomnia = 59%
Appetite loss = 63%
Constipation = 60%
Diarrhoea = 67%

43. CLARINET
PROMID

44. MULTIDISCIPLINARY APPROACH
Diarrhea: (serotonin secretion,
bacterial overgrowth - bypasses):
medical treatment
Pain: (tumor load)liver directed therapies (TAE/PRRT)
Weight loss: (cachexia) tumor control (TAE/PRRT)
Pain: (mesenterial/ retroperitoneal fibrosis – ischemia): surgical intervention
Weight loss: (undernutrition) surgical intervention
MULTIDISCIPLINARY APPROACH