29.5 Molecular Profiling and Its Potential Impact on Adrenal Pathology and Management of Patients with Adrenal Cancer
29.5.1 Diagnosis
29.5.2 Prognosis
29.5.3 Prediction
29.6 Conclusion
References
30 New Strategies for the Treatment of Adrenocortical Carcinoma
30.1 Therapy Targeted Towards Altered Signaling Pathways in Adrenocortical Carcinoma Cells
30.1.1 Identification of Potential Signaling Targets
30.1.1.1 Target Identification from the Study of Genetic Syndromes
30.1.1.2 Targets Identified from Gene Expression Analysis
30.1.2 Targeting the IGF2-IGF1R Pathway
30.1.2.1 Small Molecule TKIs
30.1.2.2 Anti-IGF1R Antibodies
30.1.3 Targeting the FGF Receptor
30.1.4 Targeting the EGF Receptor
30.1.5 Multi-kinase Inhibition Strategies
30.1.6 Targeting the Wnt Signaling Pathway
30.2 Enhancing the Effectiveness of Cytotoxic Chemotherapy
30.3 Proapoptoic Strategies
30.4 Novel Strategies
30.4.1 Targeting Tumor Vasculature
30.4.2 Targeting the Microenvironment
30.4.2.1 Fibroblasts of the Microenvironment
30.4.2.2 Immune Cells of the Microenvironment
30.4.2.3 Proteolytic Enzymes in the Microenvironment
30.4.3 Therapy Aimed at Metastasis Suppression
30.5 Delivery of Newer Modes of Therapy
30.6 Summary and Future Directions
References
Part IX Adrenal Cancer Networks and Registries
31 The Dutch Adrenal Network
31.1 The Dutch Adrenal Network
31.2 The Dutch Adrenal Registry
31.3 Results
31.4 Future Goals
31.5 Conclusion
32 The ENS@T Initiative
32.1 The History: From National Networks to ENS@T
32.2 Increasing the Patient Number
32.3 Increasing Technical and Methodological Exchange
32.4 The Idea of Exchange
32.4.1 Harmonization
32.4.2 Material
32.4.3 Science
32.5 Examples of Research Advancements
32.6 Organizing the Network
32.6.1 The Steering Committee
32.6.2 The Working Groups
32.6.3 ENS@T Website
32.7 Financing the Network
32.8 Perspectives
32.8.1 Integrational and Disseminative Effects of a European Network
References
Index
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[...]... gerard.zambetti@stjude.org Part I History of AdrenocorticalCarcinoma Research andClinical Care Chapter 1 The History of AdrenocorticalCarcinoma Treatment – A Medical Perspective David E Schteingart Knowledge of the genetic and molecular alterations in adrenocorticalcarcinoma (ACC) has advanced in the past two decades, as a result of newer laboratory methodology to study mechanisms of oncogenesis and tumor pathophysiology... et al (1979) Cushing’s syndrome due to adrenocorticalcarcinoma Acta Endocrinologica 91:303–318 29 Lewinsky BS et al (1974) The clinicaland pathologic features of “non-hormonal” adrenocortical tumors Cancer 33:778–790 30 Lipsett MB et al (1963) Clinicaland pathophysiologic aspects of adrenocorticalcarcinoma Am J Med 35:374–383 31 Richie JP, Gittes RE (1980) Carcinoma of the adrenal cortex Cancer... (1983) Adrenocorticalcarcinoma In: Thompson NW, Vinik AI (eds) Endocrine surgery update Grune and Stratton, New York, pp 119–128 7 Luton JP et al (1990) Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy N Engl J Med 322:1195–1201 8 Hogan TF et al (1978) o,p -DDD (mitotane) therapy of adrenal cortical carcinoma: observations on drug dosage, toxicity and. .. Am Physicians 48:224–235 24 Thompson NW (1983) Adrenocortical carcinoma. In: Thompson NW, Vinik AI (eds) Endocrine surgery update Grune and Straton, New York 25 Sahteingart DE et al (1982) Treatment of adrenal carcinomas Arch Surg 117:1142–1147 26 Cohn K et al (1986) Adrenocorticalcarcinoma Surgery 100:1170–1177 27 Icard P et al (1992) Adrenocorticalcarcinoma in surgically treated patients: a retrospective... T Broome Vanderbilt Endocrine Surgery Center, Vanderbilt University, 597 Preston Research Building, 2220 Pierce Ave, Nashville, TN, USA, james.broome@vanderbilt.edu Maria V Cicala Division of Endocrinology, Department of Medical and Surgical Sciences, University of Padua, Via Ospedale 105, 35128 Padova, Italy, mariaverena.cicala@unipd.it Fulvia Daffara Department of Clinicaland Biological Sciences,... adrenal tumors, four of which were carcinomas One tumor was palpable, one was seen on x-ray, and three found at operation Three developed acute adrenocortical failure, and one of them who was treated in 1923 before cortical extract was available died The other two received cortical extracts and recovered Three of the five with tumors 2 The History of AdrenocorticalCarcinoma Treatment – A Surgical Perspective... disease With cardiopulmonary bypass and cold arrest, tumor thrombus into right atrium excised and endovenectomy of all tumors in vena cava and both left and middle hepatic veins excised A pericardial patch was used for the vena cava and distal hepatic veins The patient had an uneventful course with complete relief of the Budd Chiari syndrome 2 The History of AdrenocorticalCarcinoma Treatment – A Surgical... toxicity and steroid replacement Cancer 42:2177–2181 9 Bilimoria KY et al (2008) Adrenocorticalcarcinoma in the United States Treatment utilization and prognostic factors Cancer 113(11):3130–3136 Chapter 2 The History of AdrenocorticalCarcinoma Treatment – A Surgical Perspective Norman W Thompson The early history of adrenocorticalcarcinoma (ACC) is obscure because of the rarity of the disease, confusing... manifestations, and a vague appreciation of its clinical course Most reported cases in the 19th century were based on autopsy findings, and the tumors were classified by a variety of terms including hypernephroma, sarcoma, fibromyxosarcoma, andcarcinoma The commonly used term, hypernephroma, was first proposed by Grawitz et al in 1893 and falsely assumed that tumors arose in rests of adrenocortical tissue... (1987) Adrenocorticalcarcinoma CA Cancer J Clin 37:348–365 2 Soffer LJ et al (1961) The human adrenal gland Lea & Febiger, Philadelphia, PA 3 Cecil HF (1933) Hypertension, obesity, virilism and pseudohermaphroditism as caused by suprarenal tumors J.A.M.A 100:463 4 Eisenstein AB (1967) The adrenal cortex Little, Brown and Company, Boston, MA 5 Schteingart DE et al (1982) The treatment of adrenal carcinoma .