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ACROMEGALY

ACROMEGALY. Prof. Gaetano Lombardi Dept. of Clinical and Molecular Endocrinology and Oncology University “Federico II” , Naples, Italy. Sporadic pituitary tumor. Syndromic/Familial Pituitary Tumors. MEN1. Pituitary Tumor Primary Hyperparathyroidism Endocrine Pancreatic Tumor.

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ACROMEGALY

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  1. ACROMEGALY Prof. Gaetano Lombardi Dept. of Clinical and Molecular Endocrinology and Oncology University “Federico II” , Naples, Italy

  2. Sporadic pituitary tumor

  3. Syndromic/Familial Pituitary Tumors

  4. MEN1 Pituitary Tumor Primary Hyperparathyroidism Endocrine Pancreatic Tumor Autosomal dominant Gene Men1 11q13

  5. McCune-Albright Syndrome Polyostotic fibrous dysplasia Skin pigmentation Hormonal dysfunction - Precocious puberty - Thyrotoxicosis - Gigantism - Cushing’s Syndrome Mutation di Gs-alpha Macroadenoma (50% of cases)

  6. Carney Syndrome Hyperplasia or multiple microadenomas Chiazze di iperpigmantazione cutanea Mixoma cardiaco Iperfunzione endocrina sindrome di Cushing acromegalia Autosomal dominant 2p16 Mutation of PRKAR1A

  7. RARE DISEASE

  8. Balance of GH influences on cell growth regulation Pathogenesis of cell proliferation/apoptosis in acromegaly

  9. COLON CANCER IN ACROMEGALY

  10. TREATMENT GOALS  Mortality rate reduction  Tumor shrinkage  Treatment of comorbidities  Relief of symptoms directly caused by GH excess

  11. Medical Therapy SSA, DA, GH-A Radiotherapy conventional stereotactic Surgery trans-cranium trans-sphenoidal

  12. Improvement in pituitary function in 60-97% Improvement of visual field defect in 70% Low morbidity and mortality (0-1%) Reduction in tumor size in 90% Tumor residual in 15-50% Complications in 5-18% SURGERY SUCCESS RATE: 72% microadenomas, 50% macroadenomas, 17% giant adenomas

  13. MEDICAL THERAPY Dopamine-Agonists Bromocriptine Cabergoline Lisuride – Pergolide - Quinagolide Somatostatin Analogues Octreotide Lanreotide GH-receptor antagonist

  14. SIDE EFFECTS Gastro-intestinal Hypotension Headache Nausea

  15. Clinical Improvement in 70-90% Normalisation of GH levels in 65-70% Normalisation of IGF-I levels in 65-70% Tumor shrinkage >50%     SOMATOSTATIN ANALOGUES EFFECTIVENESS

  16. Baseline 5 month-OCT LAR 10 month-OCT LAR

  17. SIDE EFFECTS Gastro-intestinal Gallstones Biliary sludge Diarrhea

  18. PEGVISOMANT • GH analog (191 amino acids) • 9 different amino acids • 4 - 5 PEG • molecular weight 42 - 46000 D • half-life >70 hours • subcutaneous administration GH is not a marker of disease Goal of therapy – to reduce IGF-I levels to normal range for age and sex

  19. STOP

  20. ◊ sst19.30.1 ◊sst21.00.1 ◊sst31.50.3 ◊sst4> 100 ◊sst50.20.1 IC50 nM

  21. SOMATOSTATIN AND DOPAMINE RECEPTOR AGONIST

  22. Dept. of Clinical and Molecular Endocrinology and Oncology R.S. Auriemma, A. Cozzolino, M. De Leo, M.C. De Martino, C. Di Somma, A. Faggiano, M. Galdiero, L.F.S. Grasso, E. Guerra, F. Milone, R. Pivonello, M.C. Savanelli, P. Vitale, L. Vuolo & A. Colao

  23. Induce clinical improvement in 30% Normalize GH levels in 30% Normalize IGF-I levels in 65-70% Induce tumor shrinkage in <20%     QUESTION 1 SOMATOSTATIN ANALOGUES:

  24. Normalize IGF-I levels in 30% Normalize IGF-I levels in 50% Normalize IGF-I levels in 70% Normalize IGF-I levels in up to 95%     QUESTION 2 THE GH-RECEPTOR ANTAGONIST PEGVISOMANT:

  25. Is correlated to GH levels Is correlated to IGF-BP1 levels Is correlated to insulin levels Is correlated to tumor size     QUESTION 3 COLONIC NEOPLASM DEVELOPMENT IN ACROMEGALY:

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