Combination targeted therapy of VEGFR inhibitor, sorafenib, with an mTOR inhibitor, sirolimus induced a remakable response of rapid progressive Uterine PEComa

References

• Zamboni G, Pea M, Martignoni G, Zancanaro C, Faccioli G, Gilioli E, Pederzoli P, Bonetti F. Clear cell "sugar" tumor of the pancreas. A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells. Am J Surg Pathol 1996; 20:722-30; PMID:8651352; http://dx.doi.org/10.1097/00000478-199606000-00010
   PubMed Web of Science ®Google Scholar
• Armah HB, Parwani AV. Perivascular epithelioid cell tumor. Arch Pathol Lab Med 2009; 133:648-54; PMID:19391667; http://dx.doi.org/10.1043/1543-2165-133.4.648
   PubMed Web of Science ®Google Scholar
• Folpe AL, McKenney JK, Li Z, Smith SJ, Weiss SW. Clear cell myomelanocytic tumor of the thigh: report of a unique case. Am J Surg Pathol 2002; 26:809-12; PMID:12023589; http://dx.doi.org/10.1097/00000478-200206000-00018
   PubMed Web of Science ®Google Scholar
• Folpe AL, Weiss SW. Lipoleiomyosarcoma (well-differentiated liposarcoma with leiomyosarcomatous differentiation): a clinicopathologic study of nine cases including one with dedifferentiation. Am J Surg Pathol 2002; 26:742-9; PMID:12023578; http://dx.doi.org/10.1097/00000478-200206000-00007
   PubMed Web of Science ®Google Scholar
• Bleeker JS, Quevedo JF, Folpe AL. Malignant perivascular epithelioid cell tumor of the uterus. Rare Tumors 2012; 4:e14; PMID:22532912; http://dx.doi.org/10.4081/rt.2012.e14
   PubMedGoogle Scholar
• Bergamo F, Maruzzo M, Basso U, Montesco MC, Zagonel V, Gringeri E, Cillo U. Neoadjuvant sirolimus for a large hepatic perivascular epithelioid cell tumor (PEComa). World J Surg Oncol 2014; 12:46; PMID:24575738; http://dx.doi.org/10.1186/1477-7819-12-46
   PubMed Web of Science ®Google Scholar
• Wu JH, Zhou JL, Cui Y, Jing QP, Shang L, Zhang JZ. Malignant perivascular epithelioid cell tumor of the retroperitoneum. Int J Clin Exp Pathol 2013; 6:2251-6; PMID:24133607
   PubMed Web of Science ®Google Scholar
• Shi H, Cao Q, Li H, Zhen T, Lai Y, Han A. Malignant perivascular epithelioid cell tumor of the kidney with rare pulmonary and ileum metastases. Int J Clin Exp Pathol 2014; 7:6357-63; PMID:25337291
   PubMed Web of Science ®Google Scholar
• Bleeker JS, Quevedo JF, Folpe AL. "Malignant" perivascular epithelioid cell neoplasm: risk stratification and treatment strategies. Sarcoma 2012; 2012:541626; PMID:22619565; http://dx.doi.org/10.1155/2012/541626
   PubMedGoogle Scholar
• Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol 2005; 29:1558-75; PMID:16327428; http://dx.doi.org/10.1097/01.pas.0000173232.22117.37
   PubMed Web of Science ®Google Scholar
• Benson C, Vitfell-Rasmussen J, Maruzzo M, Fisher C, Tunariu N, Mitchell S, Al-Muderis O, Thway K, Larkin J, Judson I. A retrospective study of patients with malignant PEComa receiving treatment with sirolimus or temsirolimus: the Royal Marsden Hospital experience. Anticancer Res 2014; 34:3663-8; PMID:24982384
   PubMed Web of Science ®Google Scholar
• Scheppach W, Reissmann N, Sprinz T, Schippers E, Schoettker B, Mueller JG. PEComa of the colon resistant to sirolimus but responsive to doxorubicin/ifosfamide. World J Gastroenterol 2013; 19:1657-60; PMID:23539498; http://dx.doi.org/10.3748/wjg.v19.i10.1657
   PubMed Web of Science ®Google Scholar
• Dickson MA, Schwartz GK, Antonescu CR, Kwiatkowski DJ, Malinowska IA. Extrarenal perivascular epithelioid cell tumors (PEComas) respond to mTOR inhibition: clinical and molecular correlates. Int J Cancer 2013; 132:1711-7; PMID:22927055; http://dx.doi.org/10.1002/ijc.27800
   PubMed Web of Science ®Google Scholar
• Lee DW, Chang H, Kim YJ, Kim KM, Lee HJ, Lee JS. Sorafenib-induced tumor response in a patient with metastatic epithelioid angiomyolipoma. J Clin Oncol 2014; 32:e42-5; PMID:24449236; http://dx.doi.org/10.1200/JCO.2012.48.1960
   PubMedGoogle Scholar
• Ray-Coquard I, Italiano A, Bompas E, Le Cesne A, Robin YM, Chevreau C, Bay JO, Bousquet G, Piperno-Neumann S, Isambert N, et al. Sorafenib for patients with advanced angiosarcoma: a phase II Trial from the French Sarcoma Group (GSF/GETO). Oncologist 2012; 17:260-6; PMID:22285963; http://dx.doi.org/10.1634/theoncologist.2011-0237
   PubMed Web of Science ®Google Scholar
• Filho Jdo E, Meneses de Amorim D, Sweet GM, Rodrigues de Freitas LA, Athanazio PR, Athanazio DA. Renal epithelioid angiomyolipoma with epithelial cysts: demonstration of Melan A and HMB45 positivity in the cystic epithelial lining. Ann Diagn Pathol 2012; 16:397-401; PMID:21676635; http://dx.doi.org/10.1016/j.anndiagpath.2011.03.004
   PubMed Web of Science ®Google Scholar
• Palleschi G, Pastore AL, Evangelista S, Silvestri L, Rossi L, Di Cristofano C, Porta N, Petrozza V, Tomao S, Carbone A. Bone metastases from bladder perivascular epithelioid cell tumor - an unusual localization of a rare tumor: a case report. J Med Case Rep 2014; 8:227; PMID:24965209; http://dx.doi.org/10.1186/1752-1947-8-227
   PubMedGoogle Scholar
• Escudier B, Eisen T, Stadler WM, Szczylik C, Oudard S, Siebels M, Negrier S, Chevreau C, Solska E, Desai AA, et al. Sorafenib in advanced clear-cell renal-cell carcinoma. N Engl J Med 2007; 356:125-34; PMID:17215530; http://dx.doi.org/10.1056/NEJMoa060655
   PubMed Web of Science ®Google Scholar
• Wagner AJ, Malinowska-Kolodziej I, Morgan JA, Qin W, Fletcher CD, Vena N, Ligon AH, Antonescu CR, Ramaiya NH, Demetri GD, et al. Clinical activity of mTOR inhibition with sirolimus in malignant perivascular epithelioid cell tumors: targeting the pathogenic activation of mTORC1 in tumors. J Clin Oncol 2010; 28:835-40; PMID:20048174; http://dx.doi.org/10.1200/JCO.2009.25.2981
   PubMed Web of Science ®Google Scholar
• Gennatas C, Michalaki V, Kairi PV, Kondi-Paphiti A, Voros D. Successful treatment with the mTOR inhibitor everolimus in a patient with perivascular epithelioid cell tumor. World J Surg Oncol 2012; 10:181; PMID:22943457; http://dx.doi.org/10.1186/1477-7819-10-181
   PubMed Web of Science ®Google Scholar
• Italiano A, Delcambre C, Hostein I, Cazeau AL, Marty M, Avril A, Coindre JM, Bui B. Treatment with the mTOR inhibitor temsirolimus in patients with malignant PEComa. Ann Oncol 2010; 21:1135-7; PMID:20215136; http://dx.doi.org/10.1093/annonc/mdq044
   PubMed Web of Science ®Google Scholar
• Cheng AL, Kang YK, Chen Z, Tsao CJ, Qin S, Kim JS, Luo R, Feng J, Ye S, Yang TS, et al. Efficacy and safety of sorafenib in patients in the Asia-Pacific region with advanced hepatocellular carcinoma: a phase III randomised, double-blind, placebo-controlled trial. Lancet Oncol 2009; 10:25-34; PMID:19095497; http://dx.doi.org/10.1016/S1470-2045(08)70285-7
   PubMed Web of Science ®Google Scholar
• Peng CL, Guo W, Ji T, Ren T, Yang Y, Li DS, Qu HY, Li X, Tang S, Yan TQ, et al. Sorafenib induces growth inhibition and apoptosis in human synovial sarcoma cells via inhibiting the RAF/MEK/ERK signaling pathway. Cancer biology & therapy 2009; 8:1729-36; PMID:19633425; http://dx.doi.org/10.4161/cbt.8.18.9208
   PubMed Web of Science ®Google Scholar
• Maki RG, D'Adamo DR, Keohan ML, Saulle M, Schuetze SM, Undevia SD, Livingston MB, Cooney MM, Hensley ML, Mita MM, et al. Phase II study of sorafenib in patients with metastatic or recurrent sarcomas. J Clin Oncol 2009; 27:3133-40; PMID:19451436; http://dx.doi.org/10.1200/JCO.2008.20.4495
   PubMed Web of Science ®Google Scholar
• Lee N, Woodrum CL, Nobil AM, Rauktys AE, Messina MP, Dabora SL. Rapamycin weekly maintenance dosing and the potential efficacy of combination sorafenib plus rapamycin but not atorvastatin or doxycycline in tuberous sclerosis preclinical models. BMC Pharmacol 2009; 9:8; PMID:19368729; http://dx.doi.org/10.1186/1471-2210-9-8
   PubMedGoogle Scholar
• Gangadhar TC, Cohen EE, Wu K, Janisch L, Geary D, Kocherginsky M, House LK, Ramirez J, Undevia SD, Maitland ML, et al. Two drug interaction studies of sirolimus in combination with sorafenib or sunitinib in patients with advanced malignancies. Clin Cancer Res 2011; 17:1956-63; PMID:21447721; http://dx.doi.org/10.1158/1078-0432.CCR-10-2061
   PubMed Web of Science ®Google Scholar