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Kerrington Smith, M.D. CTOS Nov 14, 2008

Malignant peripheral nerve sheath tumors (MPNST): clinical, pathologic and molecular predictors of survival. Sarcoma Research Center UT MDACC. Kerrington Smith, M.D. CTOS Nov 14, 2008. Clinical challenges in MPNST. Diagnostic challenges. Which deep neurofibromas will

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Kerrington Smith, M.D. CTOS Nov 14, 2008

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  1. Malignant peripheral nerve sheath tumors (MPNST):clinical, pathologic and molecular predictors of survival Sarcoma Research Center UT MDACC Kerrington Smith, M.D. CTOS Nov 14, 2008

  2. Clinical challenges in MPNST Diagnostic challenges Which deep neurofibromas will undergo malignant transformation?

  3. Clinical challenges in MPNST Prognostic challenges After resection which patients will recur? What is the pattern of recurrence? NF1-MPNST vs. sporadic MPNST?

  4. Clinical challenges in MPNST Therapeutic challenges Effective systemic therapy?

  5. CASE

  6. Which factors alter clinical outcome? Patient factors….NF-1 status? Tumor factors? Treatment related factors?

  7. MPNST epidemiology Neurofibromatosis Type 1 (NF-1) History of radiation exposure 10% • Autosomal dominant • 1 in 3500 live births • Nf1 gene on Ch 17 40-50% 50-60% Sporadic MPNST Evans DG J Med Genet 2002:39;311-4

  8. How are MPNSTs staged? AJCC staging MPNST Histopathologic grade Primary tumor Size Depth Regional lymph nodes Distant metastases Most high grade Most >10 cm All deep Rare Coindre JM et al. Cancer 2001:91;1914-26

  9. There is a need to subclassify stage III MPNST

  10. No consensus in re prognostic factors World Series of MPNST Patient Presentation Reference Year No. Status Site Size Grade NF1 Ducatman et al. (Mayo) 1986 120 NR NS NS NS + Hruban et al. (MSKCC) 1990 43 NR NS + + NS Wanebo et al. (NCI) 1992 28 NR + + NS NS Doorn et al. (Dutch) 1995 22 NR NR NR NR NS Wong et al. (Mayo Clinic) 1998 134 NR NS NS + + Cashen et al. (MGH) 2004 80 NR + NR + NS Anghileri et al. (Italy) 2006 205 + + + NS NS Molecular prognostic factors unknown

  11. Purpose To identify and validate tumor, pathologic and molecular factors prognostic of MPNST clinical behavior

  12. Methods MDACC clinical database Univariate and multivariate statistical analyses MPNST tissue microarray

  13. Study population Study dates: 1985 to 2006 Median length of follow up: 91 mo (2 to 236) Total patients N=140 Without NF-1 Syndrome N= 68 With NF-1 Syndrome N= 72

  14. Which clinical factors are associated with worse MPNST survival outcomes?? n %

  15. Metastasis trumps NF1 biology n %

  16. What drives metastasis in patients with localized MPNST??

  17. Factors associated with development of distant metastases

  18. Development of distant metastases in MPNST Tumor size S100 staining NF-1 status % Metastasis Free Time (months) Time (months) Time (months) ??? ???

  19. What drives survival in localized MPNST?

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