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罕见的老年患者左眼眶横纹肌肉瘤1例

http://www.cnophol.com 2009-4-8 10:23:02 中华眼科在线

  Female patients had a higher 5 year survival rate than male patients but the difference was not statistically significant, there appeared to be an association between female genders with complete response to chemotherapy. The Survival rate after treatment of RMS at all sites has improved from 25% in 1970 to 70% in 1991.

  Orbital RMS has been recognized to display better life prognosis than RMS at other site, the tumors display strong tendency for local invasion, local recurrence and hematogenous and lymphatic metastasis.

  Ferrarie evaluated the treatment outcomes for adult patients with RMS and confirm the relatively poor long term out comes.With radio imaging studies large tumors appeared as less well defined soft tissue masses with bone destruction or invasion of surrounding structures. It is still possible however to detect bonny invasion by virtue of destruction of bone marrow signal on MRI especially with administration of contrast.

  After treatment is instituted cross sectional imaging can be used to objectively monitor tumors regression or residual or recurrent disease. RMS may occur as metastasis to orbit from distant site and even after years of treatment which is extremely rare[12] . 5 year survival rate increased from 55% IRS 1 to 71% IRS 2 and IRS 4.

  Truncal and extremity tumors are more common in adults, and the relative proportion of pleomorphic tumors increases with age. The latest series from Memorial SloanKettering, the relative proportion of extremity and pleomorphic tumors increased with age, and survival decreased with increasing age in both univariate and multivariate analyses.

  CONCLUSION

  RMS is primarily a disease of children and young adults but can occur rarely in elderly people so high index of suspicion is required to establish early and correct. Diagnosis and initiation of appropriate treatment with chemotherapy, surgery and radiotherapy before metastasis make a great difference in saving lives of these patients.

【参考文献】
    1 Cripe TP, Gross S, Windle ML, Bergstrom SK, Petry PD, Coppes MJ. Rhabdomyosarcoma. emedicine2006;4:110

  2 Shields JA, Shields C. Rhabdomyosarcoma: review for the ophthalmologist. Surv Ophthalmol2003;48(1):3957

  3 Esnaola NF, Rubin BP, Baldini HE, Vasudevan N BA, Demetri GD, Fletcher CDM, Singer S. Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma. Ann Surg2001;234(2):215223

  4 Machleder DJ, Banik R, Rosenberg RB, Parikh SR. An unusual case of RMS presenting as orbital apex syndrome. Int J Pediatr Otorhinolaryngol 2005;69(2): 249254

  5 Sohaib SA, Moseley L, Wright JE. Orbital Rhabdomyosarcoma the Radiological characteristics. Clin Radiol1998;53(5): 357362

  6 Mafee M F, Pai E, Philip B. Rhabdomyosarcoma of the orbit evaluation with MR Imiging and CT. Radiol Clin North Am1998;36(6):12151227

  7 Manucha V, Castellani R, Sun CC. Alveolar Rhabdomyosarcoma of the paranasaal sinuses in a 57 year old woman with 1:16 translocation. Int J
Surg Pathol2006;14(3): 238242

  8 Maurya OPS, Patel R, Thakaur V, Singh R, Kumar M, Embryonal RMS of the orbita case report. Int Ophthalmol Clin 1990;38(4):202204

  9 Shindler KS, Liu GT, Womer RB. Long term follow up and prognosis of orbital apex syndrome resulting from nasopharyngeal rhabdomyosarcom. Am J Ophthalmol2005;140(2):236241

  10 Karavasilis V, Seddon B M, Ashley S, AL Muderis O, Fisher C , Judson I. Significant clinical benefit of firstline palliative chemotherapy in advanced softtissue sarcoma: retrospective analysis and identification of prognostic factors in 488 patients. Cancer2008;112(7):15851591

  11 Shields CL, Shields JA, Honavar SG, Demirci H. Clinical spectrum of primary ophthalmic rhabdomyosarcom. Ophthalmology2001;108(2):22842292

  12 Simpson JL, Alford MA, Carter KD, Keech RV. Metastatic RMS presenting as an isolated lateral rectus restriction. J Pediatr Ophthalmol
Strabismus1999;36(2):9091

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