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GI-NEUROENDOCRINE TUMORS: THERAPEUTIC APPROACHES: SYMPTOMATIC MANAGEMENT

NET MASTERCLASS 5-7-2014. GI-NEUROENDOCRINE TUMORS: THERAPEUTIC APPROACHES: SYMPTOMATIC MANAGEMENT. IOANNIS PILPILIDIS, MD, FEBGH GASTROENTEROLOGY – ONCOLOGY DEPT “THEAGENEIO” CANCER HOSPITAL OF THESSALONIKI. Long – term survival: A chronic disease. Yao, JCO 2008; 26: 3063-3072.

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GI-NEUROENDOCRINE TUMORS: THERAPEUTIC APPROACHES: SYMPTOMATIC MANAGEMENT

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  1. NET MASTERCLASS 5-7-2014 GI-NEUROENDOCRINE TUMORS: THERAPEUTIC APPROACHES:SYMPTOMATIC MANAGEMENT IOANNIS PILPILIDIS, MD, FEBGH GASTROENTEROLOGY – ONCOLOGYDEPT “THEAGENEIO” CANCER HOSPITAL OF THESSALONIKI

  2. Long – term survival: A chronic disease Yao, JCO 2008; 26: 3063-3072

  3. < 10 ή > 10

  4. Common symptoms are not typical

  5. Typical symptoms are not common

  6. 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) 40% pain (4) 10% rash (1) 10% weight loss (1) Colon – rectal (4) 25% colon – abdominal pain 75% rectal - incidentaloma

  7. 3.000 years of misdiagnosis God’s help!!! ΜΕΝ I Pituitary tumor Hyperparathyroidism Insulnoma Gene’s help!!! MENIN GENE: 11q13

  8. Liver mets • Peritoneal disease/ retroperitoneal involvement • ovary/testicular

  9. TREATMENT DOES NOT NEED A DIAGNOSIS

  10. 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)

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

  12. 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)

  13. Carcinoid syndrome: response to somatostatin analogues Clinical response 70-90% Biological response35-75% Objective response 0-5%

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

  15. Patient selection bias?

  16. Median Survival: 8.5y Median Survival (non cytoreduction): 7.9y vs 11.5y 5-y Survival 75%

  17. In the absence of other data, the resection of the primarytumour only for patients with PNETs and unresectablemetastatic liver disease should only be considered in high-volume referral centres, with strict selection criteriaand in a multidisciplinary setting of patients’ care

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

  19. TACE/TAE: Symptom Control Clinical response: 67% - 100% Biological response: 46% - 91% Ruszniewski Cancer 1993, TherasseRadiol 1993, Tomassetti 1994, Diaco Am J Surg 1995, RuszniewskiEur J GastroenterolHepatol 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%

  20. CLARINET PROMID

  21. 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

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