Molecular Oncology
Volume 4, Issue 1 , Pages 19-37 , February 2010

Targeted Therapies: The Rare Cancer Paradigm

  • Marco A. Pierotti

      Affiliations

    • Scientific Directorate, Fondazione IRCCS – Istituto Nazionale dei Tumori Milano, Via Venezian 1, 20133 Milan, Italy
    • Corresponding Author InformationCorresponding author. Tel.: +39 02 2390 2300.
  • ,
  • Tiziana Negri

      Affiliations

    • Laboratory of Experimental Molecular Pathology, Fondazione IRCCS – Istituto Nazionale dei Tumori Milano, Via Venezian 1, 20133 Milan, Italy
  • ,
  • Elena Tamborini

      Affiliations

    • Laboratory of Experimental Molecular Pathology, Fondazione IRCCS – Istituto Nazionale dei Tumori Milano, Via Venezian 1, 20133 Milan, Italy
  • ,
  • Federica Perrone

      Affiliations

    • Laboratory of Experimental Molecular Pathology, Fondazione IRCCS – Istituto Nazionale dei Tumori Milano, Via Venezian 1, 20133 Milan, Italy
  • ,
  • Sabrina Pricl

      Affiliations

    • Molecular Simulation Engineering Laboratory, DICAMP, University of Trieste, Piazzale Europa 1, 34127 Trieste, Italy
  • ,
  • Silvana Pilotti

      Affiliations

    • Laboratory of Experimental Molecular Pathology, Fondazione IRCCS – Istituto Nazionale dei Tumori Milano, Via Venezian 1, 20133 Milan, Italy

Received 15 October 2009 ,Accepted 21 October 2009.

References 

  1. Alman BA, Li C, Pajerski ME, Diaz-Cano S, Wolfe HJ. Increased beta-catenin protein and somatic APC mutations in sporadic aggressive fibromatoses (desmoid tumors). Am. J. Pathol. 1997;151:329–334
  2. Argani P, Antonescu CR, Illei PB, Lui MY, Timmons CF, Newbury R, et al. Primary renal neoplasms with the ASPL-TFE3 gene fusion of alveolar soft part sarcoma: a distinctive tumor entity previously included among renal cell carcinomas of children and adolescents. Am. J. Pathol. 2001;159(1):179–192
  3. Argani P, Lal P, Hutchinson B, Lui MY, Reuter VE, Ladanyi M. Aberrant nuclear immunoreactivity for TFE3 in neoplasms with TFE3 gene fusions: a sensitive and specific immunohistochemical assay. Am. J. Surg. Pathol. 2003;27(6):750–761
  4. Aubin JE, Bonnelye E. Osteoprotegerin and its ligand: a new paradigm for regulation of osteoclastogenesis and bone resorption. Medscape Womens Health. 2000 Mar;5(2):5
  5. Azizi AA, Haberler C, Czech T, Gupper A, Prayer D, Breitschopf H, et al. Vascular-endothelial-growth-factor (VEGF) expression and possible response to angiogenesis inhibitor bevacizumab in metastatic alveolar soft part sarcoma. Lancet Oncol. 2006;7:533–535
  6. Bheda A, Creek KE, Pirisi L. Loss of p53 induces epidermal growth factor receptor promoter activity in normal human keratinocytes. Oncogene. 2008;27:4315–4323
  7. Blay JY, El Sayadi H, Thiesse P, Garret J, Ray-Coquard I. Complete response to Imatinib in relapsing pigmented villonodular synovitis/tenosynovial giant cell tumor (PVNS/TGCT). Ann. Oncol. 2008;19:821–822
  8. Bozulic, L., Morin, P., Jr., Hunter, T., Hemmings, B.A., 2007. Meeting report: targeting the kinome — 20years of tyrosine kinase inhibitor research in Basel. Sci. STKE, pe8.
  9. Buchdunger E, Zimmermann J, Mett H, Meyer T, Müller M, Regenass U, et al. Selective inhibition of the platelet-derived growth factor signal transduction pathway by a protein-tyrosine kinase inhibitor of the 2-phenylaminopyrimidine class. Proc. Natl. Acad. Sci. U.S.A. 1995;92:2558–2562
  10. Buchdunger E, Cioffi CL, Law N, Stover D, Ohno-Jones S, Druker BJ, et al. Abl protein-tyrosine kinase inhibitor STI571 inhibits in vitro signal transduction mediated by c-kit and platelet-derived growth factor receptors. J. Pharmacol. Exp. Ther. 2000;295:139–145
  11. Carney JA, Stratakis CA. Familial paraganglioma and gastric stromal sarcoma: a new syndrome distinct from the Carney triad. Am. J. Med. Genet. 2002;108:132–139
  12. Casali PG, Messina A, Stacchiotti S, Tamborini E, Crippa F, Gronchi A, et al Imatinib mesylate in chordoma. Cancer. 2004;101:2086–2097
  13. Chugh R, Tawbi H, Lucas DR, Biermann JS, Schuetze SM, Baker LH. Chordoma: the nonsarcoma primary bone tumor. Oncologist. 2007;12:1344–1350
  14. Corless CL, Heinrich MC. Molecular pathobiology of gastrointestinal stromal sarcomas. Annu. Rev. Pathol. 2008;3:557–586
  15. Corless CL, Fletcher JA, Heinrich MC. Biology of gastrointestinal stromal tumors. J. Clin. Oncol. 2004;22:3813–3825
  16. de Bree E, Keus R, Melissas J, Tsiftsis D, van Coevorden F. Desmoid tumors: need for an individualized approach. Expert Rev. Anticancer Ther. 2009;9:525–535
  17. Dempke W, Rie C, Grothey A, Schmoll HJ. Cyclooxygenase-2: a novel target for cancer chemotherapy?. J. Cancer Res. Clin. Oncol. 2001;127:411–417
  18. Dewar AL, Cambareri AC, Zannettino AC, Miller BL, Doherty KV, Hughes TP, et al. Macrophage colony-stimulating factor receptor c-fms is a novel target of Imatinib. Blood. 2005;105:3127–3132
  19. Di Nicolantonio F, Martini M, Molinari F, Sartore-Bianchi A, Arena S, Saletti P, et al Wild-type BRAF is required for response to panitumumab or cetuximab in metastatic colorectal cancer. J. Clin. Oncol. 2008;26:5705–5712
  20. Dimitropoulos VA. Dermatofibrosarcoma protuberans. Dermatol. Ther. 2008;21:428–432
  21. Druker BJ, et al. Efficacy and safety of a specific inhibitor of the BCR-ABL tyrosine kinase in chronic myeloid leukemia. N. Engl. J. Med. 2001;344:1031–1037
  22. Erkizan HV, Kong Y, Merchant M, Schlottmann S, Barber-Rotenberg JS, Yuan L, et al. A small molecule blocking oncogenic protein EWS-FLI1 interaction with RNA helicase A inhibits growth of Ewing's sarcoma. Nat. Med. 2009;7:750–756
  23. Fletcher CD. Clinicopathologic correlation in gastrointestinal stromal tumors. Hum. Pathol. 2002;33:455
  24. Frattini M, Saletti P, Romagnani E, Martin V, Molinari F, Ghisletta M, et al. PTEN loss of expression predicts cetuximab efficacy in metastatic colorectal cancer patients. Br. J. Cancer. 2007;97:1139–1145
  25. Goettsch WG, Bos SD, Breekveldt-Postma N, Casparie M, Herings RM, Hogendoorn PC. Incidence of gastrointestinal stromal tumours is underestimated: results of a nation-wide study. Eur. J. Cancer. 2005;41:2868–2872
  26. Goldberg J, Demetri GD, Choy E, Rosen L, Pappo A, Dubois S, et al. Preliminary results from a phase II study of ARQ 197 in patients with microphthalmia transcription factor family (Mit)-associated tumors. 2009 ASCO Annual Meeting. Abstract No: 10502. J. Clin. Oncol. 2009;27:15s
  27. Greco A, Roccato E, Miranda C, Cleris L, Formelli F, Pierotti MA. Growth-inhibitory effect of STI571 on cells transformed by the COL1A1/PDGFB rearrangement. Int. J. Cancer. 2001;92:354–360
  28. Groffen J, Stephenson JR, Heisterkamp N, de Klein A, Bartram CR, Grosveld G. Philadelphia chromosomal breakpoints are clustered within a limited region, bcr, on chromosome 22. Cell. 1984;36:93–99
  29. Heinrich MC, Rubin BP, Longley BJ, Fletcher JA. Biology and genetic aspects of gastrointestinal stromal tumors: KIT activation and cytogenetic alteration. Hum. Pathol. 2002;33:484–495
  30. Heinrich MC, McArthur GA, Demetri GD, Joensuu H, Bono P, Herrmann R, et al Clinical and molecular studies of the effect of Imatinib on advanced aggressive fibromatosis (desmoid tumor). J. Clin. Oncol. 2006 Mar 1;24(7):1195–1203
  31. Heinrich MC, Maki RG, Corless CL, Antonescu CR, Harlow A, Griffith D, et al. Primary and secondary kinase genotypes correlate with the biological and clinical activity of sunitinib in Imatinib-resistant gastrointestinal stromal tumor. J. Clin. Oncol. 2008;26:5352–5359
  32. Heinrich MC, Joensuu H, Demetri GD, Corless CL, Apperley J, Fletcher JA, et al Phase II, open-label study evaluating the activity of Imatinib in treating life-threatening malignancies known to be associated with Imatinib-sensitive tyrosine kinases. Imatinib Target Exploration Consortium Study B2225. Clin. Cancer Res. 2008;14:2717–2725
  33. Hof H, Welzel T, Debus J. Effectiveness of cetuximab/gefitinib in the therapy of a sacral chordoma. Onkologie. 2006;29:572–574
  34. Huang L, Cheng YY, Chow LT, Zheng MH, Kumta SM. Receptor activator of NF-kappaB ligand (RANKL) is expressed in chondroblastoma: possible involvement in osteoclastic giant cell recruitment. Mol. Pathol. 2003;56(2):116–120
  35. Italiano A, Follana P, Caroli FX, Badetti JL, Benchimol D, Garnier G, et al Cetuximab shows activity in colorectal cancer patients with tumors for which FISH analysis does not detect an increase in EGFR gene copy number. Ann. Surg. Oncol. 2008;15:649–654
  36. Jimeno A, Messersmith WA, Hirsch FR, Franklin WA, Eckhardt SG. KRAS mutations and sensitivity to epidermal growth factor receptor inhibitors in colorectal cancer: practical application of patient selection. J. Clin. Oncol. 2009;27:1130–1136
  37. Jonker DJ, O'Callaghan CJ, Karapetis CS, Zalcberg JR, Tu D, Au HJ, et al Cetuximab for the treatment of colorectal cancer. N. Engl. J. Med. 2007;357:2040–2048
  38. Kim JS, Lee C, Bonifant CL, Ressom H, Waldman T. Activation of p53-dependent growth suppression in human cells by mutations in PTEN or PIK3CA. Mol. Cell. Biol. 2007;27:662–677
  39. Konopka JB, Watanabe SM, Witte ON. An alteration of the human c-abl protein in K562 leukemia cells unmasks associated tyrosine kinase activity. Cell. 1984;37:1035–1042
  40. Ladanyi M, Lui MY, Antonescu CR, Krause-Boehm A, Meindl A, Argani P, et al. The der(17)t(X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25. Oncogene. 2001;20(1):48–57
  41. Lamoureux F, Moriceau G, Picarda G, Rousseau J, Trichet V, Rédini F. Regulation of osteoprotegerin pro- or anti-tumoral activity by bone tumor microenvironment. Biochim. Biophys. Acta. 2009;Sep 2
  42. Lasota J, Miettinen M. Clinical significance of oncogenic KIT and PDGFRA mutations in gastrointestinal stromal tumours. Histopathology. 2008;53:245–266
  43. Lazar AJ, Das P, Tuvin D, Korchin B, Zhu Q, Jin Z, et al Angiogenesis-promoting gene patterns in alveolar soft part sarcoma. Clin. Cancer Res. 2007;13:7314–7321
  44. Lazar AJ, Tuvin D, Hajibashi S, Habeeb S, Bolshakov S, Mayordomo-Aranda E, et al. Specific mutations in the beta-catenin gene (CTNNB1) correlate with local recurrence in sporadic desmoid tumors. Am. J. Pathol. 2008;173:1518–1527
  45. Lemm D, Muegge LO, Hoeffken K, Aklan T, Mentzel T, Thorwarth M, et al. Oral. Maxillofac. Surg. 2008;12:209–213
  46. Lemm D, Mügge LO, Mentzel T, Höffken K. Current treatment options in dermatofibrosarcoma protuberans. J. Cancer Res. Clin. Oncol. 2009;135:653–665
  47. Lenz HJ, Van Cutsem E, Khambata-Ford S, Mayer RJ, Gold P, Stella P, et al Multicenter phase II and translational study of cetuximab in metastatic colorectal carcinoma refractory to irinotecan, oxaliplatin, and fluoropyrimidines. J. Clin. Oncol. 2006;24:4914–4921
  48. Li C, Bapat B, Alman BA. Adenomatous polyposis coli gene mutation alters proliferation through its beta-catenin-regulatory function in aggressive fibromatosis (desmoid tumor). Am. J. Pathol. 1998;153:709–714
  49. Li FP, Fletcher JA, Heinrich MC, Garber JE, Sallan SE, Curiel-Lewandrowski C, et al. Familial gastrointestinal stromal tumor syndrome: phenotypic and molecular features in a kindred. J. Clin. Oncol. 2005;23:2735–2743
  50. Liegl B, Leithner A, Bauernhofer T, Windhager R, Guelly C, Regauer S, et al. Immunohistochemical and mutational analysis of PDGF and PDGFR in desmoid tumours: is there a role for tyrosine kinase inhibitors in c-kit-negative desmoid tumours?. Histopathology. 2006;49:576–581
  51. Lindén O, Stenberg L, Kjellén E. Regression of cervical spinal cord compression in a patient with chordoma following treatment with cetuximab and gefitinib. Acta Oncol. 2009;48:158–159
  52. Llombart B, Sanmartín O, López-Guerrero JA, Monteagudo C, Serra C, Requena C, et al Dermatofibrosarcoma protuberans: clinical, pathological, and genetic (COL1A1-PDGFB) study with therapeutic implications. Histopathology. 2009;54:860–872
  53. Loupakis F, Ruzzo A, Cremolini C, Vincenzi B, Salvatore L, Santini D, et al KRAS codon 61, 146 and BRAF mutations predict resistance to cetuximab plus irinotecan in KRAS codon 12 and 13 wild-type metastatic colorectal cancer. Br. J. Cancer. 2009;101:715–721
  54. Loupakis F, Pollina L, Stasi I, Ruzzo A, Scartozzi M, Santini D, et al PTEN expression and KRAS mutations on primary tumors and metastases in the prediction of benefit from cetuximab plus irinotecan for patients with metastatic colorectal cancer. J. Clin. Oncol. 2009;27:2622–2629
  55. Lucas DR, Shroyer KR, McCarthy PJ, Markham NE, Fujita M, Enomoto TE. Desmoid tumor is a clonal cellular proliferation: PCR amplification of HUMARA for analysis of patterns of X-chromosome inactivation. Am. J. Surg. Pathol. 1997;21(3):306–311
  56. Luo J, Solimini NL, Elledge S. Principles of cancer therapies: oncogene and non oncogene addiction. Cell. 2009;136:823–837
  57. Mace J, Sybil Biermann J, Sondak V, McGinn C, Hayes C, Thomas D, et al. Response of extraabdominal desmoid tumors to therapy with Imatinib mesylate. Cancer. 2002;95:2373–2379
  58. Maki RG, Awan RA, Dixon RH, Jhanwar S, Antonescu CR. Differential sensitivity to Imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans. Int. J. Cancer. 2002;100:623–626
  59. Martin RC, Osborne DL, Edwards MJ, Wrightson W, McMasters KM. Giant cell tumor of tendon sheath, tenosynovial giant cell tumor, and pigmented villonodular synovitis: defining the presentation, surgical therapy and recurrence. Oncol. Rep. 2000;7:413–419
  60. McArthur GA, Demetri GD, van Oosterom A, Heinrich MC, Debiec-Rychter M, Corless CL, et al. Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with Imatinib: Imatinib Target Exploration Consortium Study B2225. J. Clin. Oncol. 2005;23:866–873
  61. Mendenhall WM, Zlotecki RA, Morris CG, Hochwald SN, Scarborough MT. Aggressive fibromatosis. Am. J. Clin. Oncol. 2005;28:211–215
  62. Mendenhall WM, Mendenhall CM, Reith JD, Scarborough MT, Gibbs CP, Mendenhall NP. Pigmented villonodular synovitis. Am. J. Clin. Oncol. 2006;29:548–550
  63. Miettinen M, Lasota J. Gastrointestinal stromal tumors—definition, clinical, histological, immunohistochemical, and molecular genetic features and differential diagnosis. Virchows Arch. 2001;438:1–12
  64. Miozzo M, Dalprà L, Riva P, Volontà M, Macciardi F, Pericotti S, et al A tumor suppressor locus in familial and sporadic chordoma maps to 1p36. Int. J. Cancer. 2000;87:68–72
  65. Mirra JM, Nelson SD, Della Rocca C, Mertens F. Chordoma. In:  Fletcher CDM,  Unni KK,  Mertens F editor. Pathology and Genetics of Tumors of Soft Tissue and Bone. World Health Organization Classification of Tumors. Lyon: IARC Press; 2002;p. 316–317
  66. Mizutani K, Tamada Y, Hara K, Tsuzuki T, Saeki H, Tamaki K, et al. Imatinib mesylate inhibits the growth of metastatic lung lesions in a patient with dermatofibrosarcoma protuberans. Br. J. Dermatol. 2004;151:235–237
  67. Morgan T, Atkins GJ, Trivett MK, Johnson SA, Kansara M, Schlicht SL, et al. Molecular profiling of giant cell tumor of bone and the osteoclastic localization of ligand for receptor activator of nuclear factor kappaB. Am. J. Pathol. 2005;167(1):117–128
  68. Negri T, Pavan GM, Virdis E, Greco A, Fermeglia M, Sandri M, et al. T670X KIT mutations in gastrointestinal stromal tumors: making sense of missense. J. Natl. Cancer Inst. 2009;101:194–204
  69. Nowell PC, Hungerford DA. Chromosome studies on normal and leukemic human leukocytes. J. Natl. Cancer Inst. 1960;25:85–109
  70. Oden-Gangloff A, Di Fiore F, Bibeau F, Lamy A, Bougeard G, Charbonnier F, et al TP53 mutations predict disease control in metastatic colorectal cancer treated with cetuximab-based chemotherapy. Br. J. Cancer. 2009;100:1330–1335
  71. Perrone F, Lampis A, Orsenigo M, Di Bartolomeo M, Gevorgyan A, Losa M, et al PI3KCA/PTEN deregulation contributes to impaired responses to cetuximab in metastatic colorectal cancer patients. Ann. Oncol. 2009;20:84–90
  72. Personeni N, Fieuws S, Piessevaux H, De Hertogh G, De Schutter J, Biesmans B, et al Clinical usefulness of EGFR gene copy number as a predictive marker in colorectal cancer patients treated with cetuximab: a fluorescent in situ hybridization study. Clin. Cancer Res. 2008;14:5869–5876
  73. Prenen H, De Schutter J, Jacobs B, De Roock W, Biesmans B, Claes B, et al PIK3CA mutations are not a major determinant of resistance to the epidermal growth factor receptor inhibitor cetuximab in metastatic colorectal cancer. Clin. Cancer Res. 2009;15:3184–3188
  74. Rao S, Cunningham D, de Gramont A, Scheithauer W, Smakal M, Humblet Y, et al Phase III double-blind placebo-controlled study of farnesyl transferase inhibitor R115777 in patients with refractory advanced colorectal cancer. J. Clin. Oncol. 2004;22:3950–3957
  75. Rinehart J, Adjei AA, Lorusso PM, Waterhouse D, Hecht JR, Natale RB, et al Multicenter phase II study of the oral MEK inhibitor, CI-1040, in patients with advanced non-small-cell lung, breast, colon, and pancreatic cancer. J. Clin. Oncol. 2004;22:4456–4462
  76. Rowley JD. A new consistent chromosomal abnormality in chronic myelogenous leukaemia identified by quinacrine fluorescence and Giemsa staining. Nature. 1973;243:290–293
  77. Saltz LB, Meropol NJ, Loehrer PJ, SrNeedle MN, Kopit J, Mayer RJ. Phase II trial of cetuximab in patients with refractory colorectal cancer that expresses the epidermal growth factor receptor. J. Clin. Oncol. 2004;22:1201–1208
  78. Sartore-Bianchi A, Martini M, Molinari F, Veronese S, Nichelatti M, Artale S, et al PIK3CA mutations in colorectal cancer are associated with clinical resistance to EGFR-targeted monoclonal antibodies. Cancer Res. 2009;69:1851–1857
  79. Scotlandi K, Benini S, Sarti M, Serra M, Lollini PL, Maurici D, et al. Insulin-like growth factor I receptor-mediated circuit in Ewing's sarcoma/peripheral neuroectodermal tumor: a possible therapeutic target. Cancer Res. 1996;56:4570–4574
  80. Shaw RJ, Cantley LC. Ras, PI(3)K and mTOR signalling controls tumour cell growth. Nature. 2006;441:424–430
  81. Signoroni S, Frattini M, Negri T, Pastore E, Tamborini E, Casieri P, et al Cyclooxygenase-2 and platelet-derived growth factor receptors as potential targets in treating aggressive fibromatosis. Clin. Cancer Res. 2008;13:5034–5040Erratum in: Clin. Cancer Res. 2008;14(13):4354
  82. Sjöblom T, Shimizu A, O'Brien KP, Pietras K, Dal Cin P, Buchdunger E, et al. Growth inhibition of dermatofibrosarcoma protuberans tumors by the platelet-derived growth factor receptor antagonist STI571 through induction of apoptosis. Cancer Res. 2001;61:5778–5783
  83. Skubitz KM, Manivel JC, Clohisy DR, Frolich JW. Response of Imatinib-resistant extra-abdominal aggressive fibromatosis to sunitinib: case report and review of the literature on response to tyrosine kinase inhibitors. Cancer Chemother. Pharmacol. 2009;64:635–640
  84. Stacchiotti, S., Bertulli, R., Grosso, F., Dileo, P., Badalamenti, G., Messina, A., Gronchi, A., Tamborini, E., Pilotti, S., Bidoli, P., et al., 2007. Antitumor activity of Imatinib plus cisplatin in three patients with advanced chordoma. Proc. 12th Ann Connective Tissue. Oncology Society Meet., Abs. # 725, p. 58.
  85. Stacchiotti S, Tamborini E, Marrari A, Brich S, Rota SA, Orsenigo M, et al Response to sunitinib malate in advanced alveolar soft part sarcoma. Clin. Cancer Res. 2009;15:1096–1104
  86. Stacchiotti S, Marrari A, Tamborini E, Palassini E, Virdis E, Messina A, et al Response to Imatinib plus sirolimus in advanced chordoma. Ann. Oncol. 2009;20:1886–1894
  87. Stam K, et al. Evidence of a new chimeric bcr/c-abl mRNA in patients with chronic myelocytic leukemia and the Philadelphia chromosome. N. Engl. J. Med. 1985;313:1429–1433
  88. Stockwin LH, Vistica DT, Kenney S, Schrump DS, Butcher DO, Raffeld M, et al. Gene expression profiling of alveolar soft-part sarcoma (ASPS). BMC Cancer. 2009;15:9–22
  89. Stommel JM, Kimmelman AC, Ying H, Nabioullin R, Ponugoti AH, Wiedemeyer R, et al. Coactivation of receptor tyrosine kinases affects the response of tumor cells to targeted therapies. Science. 2007;318(5848):287–290
  90. Storm PB, Magge SN, Kazahaya K, Sutton LN. Cervical chordoma in a patient with tuberous sclerosis presenting with shoulder pain. Pediatr. Neurosurg. 2007;43:167–169
  91. Subbiah V, Anderson P, Lazar AJ, Burdett E, Raymond K, Ludwig JA. Ewing's sarcoma: standard and experimental treatment options. Curr. Treat. Options Oncol. 2009;1–2:126–140
  92. Tamborini E, Pricl S, Negri T, Lagonigro MS, Miselli F, Greco A, et al. Functional analyses and molecular modeling of two c-Kit mutations responsible for Imatinib secondary resistance in GIST patients. Oncogene. 2006;25:6140–6146
  93. Tamborini E, Negri T, Miselli F, Lagonigro MS, Pricl S, Pilotti S. Re: response of a KIT-positive extra-abdominal fibromatosis to Imatinib mesylate and KIT genetic analysis. J. Natl. Cancer Inst. 2006;98:1583–1584
  94. Tamborini E, Miselli F, Negri T, Lagonigro MS, Staurengo S, Dagrada GP, et al Molecular and biochemical analyses of platelet-derived growth factor receptor (PDGFR) B, PDGFRA, and KIT receptors in chordomas. Clin. Cancer Res. 2006;12:6920–6928
  95. Thomas DM, Skubitz KM. Giant cell tumour of bone. Curr. Opin. Oncol. 2009;21(4):338–344
  96. Tsuda M, Davis IJ, Argani P, Shukla N, McGill GG, Nagai M, et al. TFE3 fusions activate MET signaling by transcriptional up-regulation, defining another class of tumors as candidates for therapeutic MET inhibition. Cancer Res. 2007;67:919–929
  97. Vistica DT, Hollingshead M, Borgel SD, Kenney S, Stockwin LH, Raffeld M, et al Therapeutic vulnerability of an in vivo model of alveolar soft part sarcoma (ASPS) to antiangiogenic therapy. J. Pediatr. Hematol. Oncol. 2009;31:561–570
  98. Weinberger PM, Yu Z, Kowalski D, Joe J, Manger P, Psyrri A, et al. Differential expression of epidermal growth factor receptor, c-Met, and HER2/neu in chordoma compared with 17 other malignancies. Arch. Otolaryngol. Head Neck Surg. 2005;131:707–711
  99. West RB, Rubin BP, Miller MA, Subramanian S, Kaygusuz G, Montgomery K, et al A landscape effect in tenosynovial giant-cell tumor from activation of CSF1 expression by a translocation in a minority of tumor cells. Proc. Natl. Acad. Sci. U.S.A. 2006;103:690–695
  100. World Health Organization Classification of Tumours . Pathology and Genetics. Tumours of soft Tissue and bone. Lyon: IARC Press; 2002;
  101. Yuzawa S, Opatowsky Y, Zhang Z, Mandiyan V, Lax I, Schlessinger J. Structural basis for activation of the receptor tyrosine kinase KIT by stem cell factor. Cell. 2007;130:323–334
  102. Zimmermann J, Caravatti G, Mett H, Meyer T, Müller M, Lydon NB, et al. Phenylamino-pyrimidine (PAP) derivatives: a new class of potent and selective inhibitors of protein kinase C (PKC). Arch. Pharm. (Weinheim). 1996;329:371–376
  103. Zuckerman DS, Clark W. Systemic therapy for metastatic colorectal cancer. Cancer. 2008;112:1879–1891

PII: S1574-7891(09)00131-8

doi: 10.1016/j.molonc.2009.10.003

Molecular Oncology
Volume 4, Issue 1 , Pages 19-37 , February 2010