Center for Childhood Cancer
SELECTED PUBLICATIONS
Chang, L.-S. and D.B. Welling. 2008. Molecular Biology of Vestibular Schwannomas. In Auditory/Vestibular Research, Sokolowski, B. (Ed.), The Humana Press, Totowa, NJ.
Dorogova, N.V., E.M. Akhmameteva, S.A. Kopyl, N.V. Gubanova, O.S. Yudina, L.V. Omelyanchuk, and L.-S. Chang. 2008. The Role of Drosophila Merlin in Spermatogenesis.  BMC Cell Biol. 9: 1-15.
LeGallo RD, Stelow EB, Ramirez NC, Atkins KA: Diagnosis of Hydatidiform Moles  Using p57 Immunohistochemistry and HER2 Fluorescent In Situ Hybridization. Am J Clin Pathol 2008;29:749-755  PubMed ID: 18426735

Chen C., Hsieh F., Lieblein, J.C., Brown J., Chan C., Wallace J., Cheng G., Hall B., and Lin J.   Stat3 activation in human endometrial and cervical cancers. British Journal of Cancer  In Press (2007).
Chandler, D.S. (2007). Regulation of p53 tumor suppressor activity by alternative splicing. Alternative Splicing in Cancer.  (ed. J.P. Venables) Research Signposts Publishers.
Amador N, Scheithauer BW, Giannini C, Raffel C. Acute cerebellitis presenting as tumor. Report of two cases. Journal of Neurosurgery 107(1 Suppl): 57-61, July 2007.  PubMed ID: 17644922
Diaz-Haderthauer M, Cohn DE, Ramirez NC: Aggressive Angiomyxoma of the Vulva.  Surgical Pathology No. SP 07-1 (SP-345), Check Sample, American Society for Clinical Pathology, Surgical Pathology, Vol. 35, No. 1, 2007, ISSN-1091-8655, 2007
Jacob, A., T.X. Lee, B.A. Neff, S. Miller, D.B. Welling, and L.-S. Chang. 2007.  Activation of the AKT Pathway in Human Vestibular Schwannomas. Otol. Neurotol. 29:58-68.
Ramirez NC, Barr TJ, Billiter DM: Utilizing Virtual Microscopy for Quality Control Review. Dis Markers, Vol.23, No.5-6, 2007, 459-466  PubMed ID: 18057529
Welling, D.B., M.D. Packer, and L.-S. Chang. 2007. Molecular Studies of Vestibular Schwannomas: A Review. Curr. Opin. Otolaryngol. Head Neck Surg. 15:341-346.
Welling, D.B., M.D. Packer, E.M. Akhmametyeva, and L.-S. Chang. 2007. The Biology and Genetics of Vestibular Schwannomas. In Tumors of the Cerebellopontine Angle, Bambakidis, N.C., Megerian, Porter, and R.F. Spetzler (Eds), B.C. Decker, Inc., Hamilton, Ontario, CA.
White JB, Raffel C, Balckwell RE. A case of infertility in a patient with a ventriculoperitoneal shunt. Pediatric Neurosurgery 43 (2): 146-8, 2007.  PubMed ID: 17337930

Chandler, D.S., Singh, R.K., Caldwell, L.C., Bitler, J.L., and Lozano, G. Genotoxic stress induces coordinately regulated alternative splicing of the p53 modulators MDM2 and MDM4.  Cancer Research, 66: 9502-9508, 2006.  
Akhmametyeva, E.M., M.M. Mihaylova, H. Luo, S. Kharzai, D.B. Welling, and L.-S. Chang. 2006. Regulation of the NF2 Gene Promoter Expression During Embryonic Development. Dev. Dyn. 235:2771-2785.
Bloomston M, Chanona-Vilchis J, Ellison EC, Ramirez NC, Frankel WL: Carcinosarcoma of the Pancreas arising in a mucinous cystic neoplasm. AM Surg. 2006 April; 72 (4): 351-5  PubMed ID: 16676863
Chang, L.-S., A. Jacob, M. Lorenz, J. Rock, E.M. Akhmametyeva, G. Mihai, P. Schmalbrock, A.R. Chaudhury, R. Lopez, J. Yamate, M.R. John, H. Wickert, B.A. Neff, E. Dodson, and D.B. Welling. 2006. Magnetic Resonance Imaging Noninvasively Quantifies Schwannoma Xenografts in SCID Mice. Laryngoscope 116:2018-2026.
Clarke MJ, Foy AB, Wetjen N, Raffel C.  Imaging characteristics and growth of subependymal giant cell astrocytomas.  Neurosurgical Focus 2006; 20(1):E5.   PubMed ID: 16459995
Cohn DE, Resnik KE, Ramirez NC, Morrison CD: Advanced endometrial cancer with serous metastasis in a 17-year-old. Gynecol Oncol 101 (2006) 356-359  PubMed ID: 16499956
Jiang, Y., W.D. Lo, E.M. Akhmametyeva, and L.-S. Chang. 2006. Over-expression of p73? Results in Apoptotic Death of Postmitotic hNT Neurons. J. Neurol. Sci. 240:1-6.
Morrison C, Zanagnolo V, Ramirez NC, Cohn DE, Kelbick N, Copeland L, Maxwell GL, Fowler JM: HER-2 is an independent prognostic factor in endometrial cancer: Association with outcome in a large cohort of surgically staged patients. J Clin Oncol 24 (15): 2376-2385, 2006  PubMed ID: 16710036
Neff, B.A., D.B. Welling, E.M. Akhmametyeva, and L.-S. Chang. 2006. The Molecular Biology of Vestibular Schwannomas: Dissecting the Pathogenic Process at the Molecular Level.  Otol. Neurotol. 27:197-208.
Neff, B.A., E. Oberstein, M. Lorenz,, A. Chadhury, D.B. Welling, and L.-S. Chang. 2006. Cyclin D1 and D3 Expression in Vestibular Schwannomas.  Laryngoscope 116:423-426.
Ben-Shachar I, Pavelka J, Cohn DE, Copeland LJ, Ramirez NC, Manolitsas T, Fowler JM: Surgical staging for patients presenting with grade 1 endometrial carcinoma. Obstet Gynecol 3: 487-493, 2005  PubMed ID: 15738013
Buell JF, Beebe TM, Gross TG, Trofe J, Hanaway MJ, Alloway RR, First MR, Woodle ES. United network for organ sharing publication on scientific registry of transplant recipients central nervous system donor cancer transmission data. Transplantation. 79:623, 2005.  PubMed ID: 15753859
Chang, L.-S., E.M. Akhmametyeva, M. Mihaylova, H. Luo, S. Tae, B. Neff, and D.B. Welling. 2005. Dissecting the Molecular Pathways in Vestibular Schwannoma Tumorigenesis. Recent Research Development in Genes & Genomes 1: 1-33.
Cordero L, Giannone PJ, Ramirez NC, Joy SD: Mean arterial pressure in concordant and discordant triplets during the first week of life. J Perinatol 3:198-204, 2005  PubMed ID: 15578030
Fowler JM, Ramirez NC, Cohn DE, Kelbick N, Pavelka J, Ben-Shachar I, Morrison C: Correlation of cyclooxygenase-2 (COX-2) and aromatase expression in human endometrial cancer: Tissue microarray analysis. Am J Obstet Gynecol 4:1262-1271, 2005
Golovnina, K., A. Blinov, E.M. Akhmametyeva, L.V. Omelyanchuk, and L.-S. Chang. 2005. Evolution and Origin of Merlin, the Product of the Neurofibromatosis Type 2 (NF2) Tumor-Suppressor Gene. BMC Evol. Biol. 5:69-86.
Popov, B., L.-S. Chang, and V. Serikov. 2005. Cell Cycle-Related Transformation of the E2F4-p130 Repressor Complex. Biochem. Biophys. Res. Commun. 336:762-769.
Song H., Wang R., Wang S., and Lin, J.  A small molecule inhibitor discovered through virtual database screening inhibits Stat3 oncogenic function in breast cancer cells. Proc Natl Acad Sci USA 102:4700-4725 (2005).
Trofe J, Buell JF, Beebe TM, First MR, Alloway RR, Gross TG, Succop P, Woodle ES. Analysis of factors that influence survival with post-transplant lymphoproliferative disorder in renal transplant recipients: the Israel Penn International Transplant Tumor Registry experience. Am J Transplant 4:775-80, 2005.  PubMed ID: 15760401
Raffel C. Control of B-catenin/Tcf-directed transcription in medulloblastoma.  Acta Paediatr 2004(Suppl 445):12-17
Raffel C.  Medulloblastoma: Molecular genetics and animal models.  Neoplasia 2004 Jul-Aug; 6(4):310-22.   PubMed ID: 15256053
Chandler, D.S., Lozano, G. Metastasis, Mouse Models of Human Cancer, 307-320,2004.  (ed. Eric C. Holland)
Aull MJ, Buell JF, Trofe J, First MR, Alloway RR, Hanaway MJ, Wagoner L, Gross TG, Beebe T, Woodle ES. Experience with 274 cardiac transplant recipients with posttransplant lymphoproliferative disorder: a report for the Israel Penn International Transplant Tumor Registry. Transplantation,78:1676-82, 2004.  PubMed ID: 15591959
Cascino GD, Buchhalter JR, Sirven JI, So EL, Drazkowski JF, Zimmerman RS, Raffel C.  Peri-ictal SPECT and surgical treatment for intractable epilepsy related to schizencephaly.  Neurology 2004 Dec 28; 63(12):2426-8.   PubMed ID: 15623720
Imai C, Ross ME, Reid G, Coustan-Smith E, Schultz KR, Pui CH, Downing JR and Campana D. Expression of the adaptor protein BLNK/SLP-65 in childhood acute lymphoblastic leukemia. Leukemia 18(5): 922-5, 2004.
Jarrar RG, Buchhalter JR, Raffel C.  Long-term outcome of epilepsy surgery in patients with tuberous sclerosis.  Neurology 2004 Feb 10; 62(3):479-81.   PubMed ID: 14872037
Jin X., Gossett D., Wang S., Yang D., Reynolds R.K., and Lin, J. Inhibition of AKT pathway in endometrial cancer cells harboring PTEN mutation by an AKT small moleculare inhibitor.  British Journal of Cancer  91:1808-1812  (2004).
Maher CO, Raffel C.  Neurosurgical treatment of brain tumors in children.  Pediatr Clin North Am 2004 Apr; 51(2):327-57.   PubMed ID: 15062674
Papaconstantinou HT, Sklow B, Hanaway MJ, Gross TG, Beebe Tm, Trofe J, Alloway RR, Woodle ES. Characteristics and survival patterns of solid organ transplant patients developing de novo colon and rectal cancer. Dis Colon Rectum, 47:1898-903, 2004.  PubMed ID: 15622583
Pavelka JC, Ben-Shachar I, Fowler JM, Ramirez NC, Copeland LJ. Morbid obesity and endometrial cancer: surgical, clinical, and pathologic outcomes in surgically managed patients. Gynecol Oncol 3:588-592, 2004  PubMed ID: 15581968
Qualman SJ, France M, Grizzle WE, LiVolsi VA, Moskaluk CA, Ramirez NC, Washington MK: Establishing a tumour bank: banking, informatics and ethics. British Journal of Cancer (2004), 1-5  PubMed ID: 15026787
Ross ME, Mahfouz R, Onciu M, Liu H-C, Zhou X, Song G, Shurtleff SA, Pounds S, Cheng C, Ma J, Ribeiro RC, Rubnitz JE, Girtman K, Williams WK, Raimondi SC, Liang D-C, Shih L-Y, Pui C-H and Downing JR. Gene Expression Profiling of Pediatric Acute Myelogenous Leukemia. Blood FirstEdition release June 2004.
Satoskar A, Ramirez NC, Bartholomew D, Davis J, Nuovo GJ: Histologic correlates of viral and bacterial infection of the placenta associated with severe morbidity and mortality in the newborn.  Human Pathology, Vol. 35, No. 5, May 2004  PubMed ID: 15138926
Song H., Jin X., and Lin J.  Stat3 up-regulates MEK5 expression in human breast cancer cells. Oncogene 23:8301-8309 (2004).
Tang H., Sondak V. and Lin J. A Modified p53 Enhances Apoptosis in Sarcoma Cell Lines Mediated by Doxorubicin. British Journal of Cancer 90: 1285-92  (2004).
Trofe J, Beebe TM, Buell JF, Hanaway MJ, First MR, Alloway RR, Gross TG, Woodle ES. Prosttransplant malignancy. Prog Transplant,14:193-200, 2004.  PubMed ID: 15495778
Chandler, D.S., Qi, J., and Mattox, W. Direct repression of splicing by transformer-2. Molecular and Cellular Biology, 23(15): 5174-85, 2003.
Cohen-Gadol AA, Britton JW, Wetjen NM, Marsh WR, Meyer FB, Raffel C.  Neurostimulation therapy for epilepsy: Current modalities and future directions.  Mayo Clin Proc 2003 Feb; 78(2):238-48.   PubMed ID: 12583536
Cohen-Gadol AA, Koch CA, Raffel C, Spinner RJ, Patterson RH, Giannotta SL, Hoff JT, Stern WE, Kline DG.  Confirmation of research publications reported by neurological surgery residency applicants.  Surg Neurol 2003 Oct; 60(4):280-4.   PubMed ID: 14505831
Lo, W., L. Zhu, E. Akhmametyeva, and L.-S. Chang. 2003. Induction of Apoptosis by the p53-Related p73 and Partial Inhibition by the Baculovirus-Encoded p35 in Neuronal Cells. Brain Res. Mol. Brain Res. 113:1-12.
Maher CO, Cohen-Gadol AA, Raffel C.  Cortical resection for epilepsy in children with linear sebaceous nevus syndrome.  Pediatr Neurosurg 2003 Sep; 39(3):129-35.   PubMed ID: 12876391
Ross ME, Zhou X, Song G, Shurtleff SA, Girtman K, Williams WK, Liu H-C, Mahfouz R, Raimondi SC, Lenny N, Patel A and Downing JR. Classification of pediatric acute lymphoblastic leukemia by gene expression profiling. Blood 102(8): 2951-2959, 2003.
Taylor MD, Raffel C, Bayani J, Marrano P, Mainprize TG, Squire J.  Advanced cancer genetics in neurosurgical research.  Neurosurgery 2003 Nov; 53(5):1168-78.   PubMed ID: 14580285
Warnick RE, Raffel C, Glick RP.  Neuro-oncology research opportunities in North America: results of a survey by the AANS/CNS Section on Tumors.  J Neurooncol 2003 Feb; 61(3):197-201.   PubMed ID: 12675311
Woodruff JM, Scheithauer BW, Kurtkaya-Yapicier O, Raffel C, Amr SS, LaQuaglia MP, Antonescu CR.  Congenital and childhood plexiform (Multinodular) cellular Schwannoma - A troublesome mimic of malignant peripheral nerve sheath tumor.  Am J Surg Pathol 2003 Oct; 27(10):1321-9.   PubMed ID: 14508393
Xu, Z., S. Huang, L.-S. Chang, A.D. Agulnick, and S.J. Brandt. 2003. Identification of a TAL1 Target Gene Reveals a Positive Role for the LIM Domain-Binding Protein Ldb1 in Erythroid Gene Expression and Differentiation.  Mol. Cell. Biol. 23: 7585-7599.
Ferguson, P.J., Blanton, S., Saulsbury, F.T., McDuffie, M.J., Lemahieu, V., Gastier, J.M., Francke, U., Borowitz, S.M., Sutphen, J.L., Kelly, T.E. (2000). Manifestations and linkage analysis in X-linked Autoimmunity-immunodeficiency Syndrome. Am. J. Med. Genet. 90: 390-397.
Gastier, J.M., Berg, M.A., Vesterhus, P., Reiter, E., Francke, U. (2000). Diverse deletions in the growth hormone receptor gene cause Growth Hormone Insensitivity Syndrome. Hum Mutation 16:323-333.
Gastier, J.M., Brody, T., Pulido, J.C., Businga, T., Sunden, S., Hu, X., Maitra, S., Buetow, K.H., Murray, J.C., Sheffield, V.C., Boguski, M., Duyk, G.M., Hudson, T.J. (1996). Development of a screening set for new (CAG/CTG)n dynamic mutations. Genomics 32: 75-85.
Graef, I, Gastier, J.M., Francke, U., Crabtree, G.R. (2001). Evolutionary relationships among Rel domains indicate functional diversification by recombination. Proc Natl Acad Sci USA. 98: 5740-5745.
Lemahieu, V., Gastier, J.M., Francke, U. (1999). Novel mutations in the Wiskott-Aldrich Syndrome protein gene and their effects on transcriptional, translational, and clinical phenotypes. Hum. Mutation. 14: 54-66.
Yandava, C.N., Gastier, J.M., Pulido, J.C., Brody, T., Sheffield, V., Murray, J., Buetow, K., Duyk, G.M. (1997). Characterization of the Alu repeats that are associated with tri- and tetranucleotide repeat microsatellites. Genome Research 7: 716-724.
Lin J., Jin X., Rothman K., Lin H. and Burke W.M. Inhibition of Stat3 by p53 tumor suppressor in breast cancer cells. Cancer Research 62: 376-380 (2002).
Lin J., Tang T., Jin X. and Hsieh J. P53 regulates Stat3 phosphorylation and DNA binding activity in human prostate cancer cells expressing constitutively active Stat3. Oncogene 21: 3082-3088 (2002).
Chang, L.-S., E.M. Akhmametyeva, Y. Wu, L. Zhu, and D.B. Welling. 2002. Multiple Transcription Initiation Sites, Alternative Splicing, and Differential Polyadenylation Contribute to the Complexity of Human Neurofibromatosis 2 Transcripts.  Genomics 79:63-76.
Lasak, J.M., D.B. Welling, E. Akhmametyeva, M. Salloum, and L.-S. Chang. 2002. Retinoblastoma-CDK Pathway Deregulation in Vestibular Schwannomas.  Laryngoscope 112:1555-1561.
Lott, S.T.*, Chandler, D.S.*, Curley, S., Foster, C.J., El-Naggar, A., Frazier, M., Lovell, M.M., Strong, L.C., Killary, A. M. High Frequency Loss of Heterozygosity in von Hippel-Lindau (VHL)-Associated and Sporadic Pancreatic Islet Cell Tumors: Evidence for a Stepwise Mechanism for Malignant Conversion in VHL Tumorigenesis. Cancer Research, 62: 1952-1955, 2002. *designates co-first authors
Lu W., Lin J. and Chen J. Expression of p14ARF overcomes tumor resistance to p53. Cancer Research 62: 1305-1310 (2002).
Maher CO, Friedman JA, Meyer FB, Lynch JJ, Unni K, Raffel C.  Surgical treatment of fibrous dysplasia of the skull in children.  Pediatr Neurosurg 2002 Aug; 37(2):87-92.   PubMed ID: 12145517
Park K, Buchhalter J, McClelland R, Raffel C.  Frequency and significance of acute postoperative seizures following epilepsy surgery in children and adolescents.  Epilepsia 2002 Aug; 43(8):874-81.   PubMed ID: 12181006
Perry A, Scheithauer BW, Macaulay RJB, Raffel C, Roth KA, Kros JM.  Oligodendrogliomas with neurocytic differentiation. A report of 4 cases with diagnostic and histogenetic implications.  Journal of Neuropathology & Experimental Neurology 2002 Nov; 61(11):947-55.   PubMed ID: 12430711
Phuong LK, Schoeberl KA, Raffel C.  Natural history of tethered cord in patients with meningomyelocele.  Neurosurgery 2002 May; 50(5):989-93.   PubMed ID: 11950401
Podust, V.N., L.-S. Chang, R. Ott, G.L. Dianov, and E. Fanning. 2002. Reconstitution of human DNA Polymerase ? Using Recombinant Baculoviruses: The p12 Subunit Potentiates DNA Polymerizing Activity of the Four-Subunit Enzyme. J. Biol. Chem. 277:3894-3901.
Ramin KD, Raffel C, Breckle RJ, Ogburn PL Jr, Friedman JD, Ramsey PS.  Chronology of neurological manifestations of prenatally diagnosed open neural tube defects.  Journal of Maternal-Fetal & Neonatal Medicine 2002 Feb; 11(2):89-92.   PubMed ID: 12375549
Taylor MD, Liu L, Raffel C, Hui CC, Mainprize TG, Zhang XY, Agatep R, Chiappa S, Gao LZ, Lowrance A, Hao AH, Goldstein AM, Stavrou T, Scherer SW, Dura WT, Wainwright B, Squire JA, Rutka JT, Hogg D.  Mutations in SUFU predispose to medulloblastoma.  Nat Genet 2002 Jul; 31(3):306-10.   PubMed ID: 12068298
Welling, D.B., J.M. Lasak, E. Akhmametyeva, B. Ghehari, and L.-S. Chang. 2002. cDNA Microarray Analysis of Vestibular Schwannomas. Otology Neurology 23:736-748.
Yeoh E-J, Ross ME, Shurtleff SA, Williams WK, Patel D, Mahfouz R, Behm FG, Raimondi SC, Relling MV, Patel A, Cheng C, Campana D, Wilkins D, Zhou X, Li J, Liu H, Pui C-H, Evans WE, Naeve C, Wong L and Downing JR. Classification, subtype discovery, and prediction of outcome in pediatric acute lymphoblastic leukemia by gene profiling. Cancer Cell 1(2): 133-143, 2002.
Chandler, D.S., McGuffin, M.E., Mattox, W. Functionally antagonistic sequences are required for normal autoregulation of Drosophila. tra-2 pre-mRNA splicing. Nucleic Acids Research, 29(14):3012-9, 2001.
Burke M.W., Jin X., Lin H., Huang M., Liu R., Reynolds Y.R.K. and Lin J. Inhibition of constitutively active Stat3 in ovarian and breast cancer cells. Oncogene 20: 7925-7934 (2001).

Lin J., Jin X., Page C., Sondak V., Jiang G., and Reynolds K. A modified p53 overcomes mdm2-mediated oncogenic transformation. Cancer Research 60:5895-5901 (2000). 

Frey M, Packianathan NB, Fehniger TA, Ross ME, Wang W-C, Stewart CC, Caligiuri MA and Evans SS. Differential expression and function of L-selectin on CD56bright and CD56dim natural killer cell subsets. J Immunol 161: 400-408, 1998.
McGuffin, M.E., Chandler, D.S., Somaiya, D., Dauwalder, B. and Mattox, W. (1998). Autoregulation of transformer-2 alternative splicing is necessary for normal male fertility in Drosophila. Genetics, 149: 1477-1486, 1998.
Zhu, L., S.B. Kahwash, and L.-S. Chang. 1998. Developmental Expression of Mouse Erythrocyte Protein 4.2 mRNA: Evidence for Specific Expression in Erythroid Cells. Blood 91: 695-705.
Chandler, D.S., McGuffin, M.E., Piskur, J., Yao, J., Baker, B.S., Mattox, W. Evolutionary conservation of germline specific autoregulation by the sex determination factor transformer-2. Molecular and Cellular Biology, 17: 2908-2919, 1997.
Ross ME and Caligiuri MA. Cytokine-induced apoptosis of human natural killer cells identifies a novel mechanism to regulate the innate immune response. Blood 89(3): 910-918, 1997.
Zhao, L. and L.-S. Chang. 1997. The Human POLD1 Gene: Identification of An Upstream Activator Sequence, Activation by Sp1 and Sp3, and Cell Cycle Regulation. J. Biol. Chem. 272: 4869-4882.
Zhu, L., L. Zhu, E. Xie, and L.-S. Chang. 1995. Differential Role of Two Tandem E2F Sites in Repression of Human p107 Promoter by Retinoblastoma and p107 proteins. Mol. Cell. Biol. 15:3552-3562.
Shi, Y., E. Seto, L.-S. Chang, and T. Shenk. 1991. Transcription Repression by a Human Kruppel-Related Protein and Relief of Repression by Adenovirus E1A.  Cell 67:377-388.
Samulski, R.J., L.-S. Chang, and T. Shenk. 1987. A Recombinant Plasmid from Which an Infectious Adeno-Associated Virus Genome Can Be Exised In Vitro and Its Use to Study Viral Replication.  J. Virol. 61:3096-3101.