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Long term outcomes of patients with l eiomyosarcoma of uterine vs. extra-uterine origin

Long term outcomes of patients with l eiomyosarcoma of uterine vs. extra-uterine origin. Srikanth Divi , Medical Student, Univ. of Pittsburgh Meghan Levy, Kurt Weiss MD, Mark Goodman MD, Richard McGough MD. Background of Leiomyosarcoma (LMS).

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Long term outcomes of patients with l eiomyosarcoma of uterine vs. extra-uterine origin

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  1. Long term outcomes of patients with leiomyosarcoma of uterine vs. extra-uterine origin SrikanthDivi, Medical Student, Univ. of Pittsburgh Meghan Levy, Kurt Weiss MD, Mark Goodman MD, Richard McGough MD

  2. Background of Leiomyosarcoma (LMS) • Approximately 10,000 soft tissue sarcomas are diagnosed in the US annually1 • LMS composes up to 10% (1000 cases) and is the third most frequent sarcoma after pleomorphic sarcoma NOS and liposarcoma2 • Classification: • visceral • gastrointestinal, separate from GIST • uterine • somatic • peripheral soft tissues (cutaneous, deep soft tissue) • retroperitoneal (50%) • vascular • bone (rare)

  3. Background of Leiomyosarcoma (LMS) • About 2/3 of retroperitoneal LMS occur in women, median age 60 y • Presenting symptoms nonspecific: abdominal mass, swelling, pain, weight loss, nausea, or vomiting • Presenting size often large (mean size and weight: 16 cm, 1600g) • In contrast, tumors of the extremities affect men and women equally • Significantly smaller (6 cm) than retroperitoneal tumors

  4. Background of Leiomyosarcoma (LMS) • Leiomyomas not necessarily associated with malignant transformation to LMS • Observation that significant number of tumors arise from small blood vessels • LMS also seen in the setting of hereditary retinoblastoma (abnormal Rb1 locus)

  5. Prognosis of patients with somatic soft tissue (SST) LMS • Farshid et al., 42 patients3 (2002) • 8% developed local recurrence, 45% developed mets • Age > 62, size > 4cm, extensive necrosis correlated with metastasis • Conclusion: majority are of vascular origin (39/42 pts), disruption of tumor w/ biopsy is significant RF • Mankin et al., 66 patients4 (2004) • 50% survival at 4.2 years, 62% developed mets • Tumors with greater size (>500 cm3) had 82% mortality rate • Conclusion: presence of mets, size of tumor, MSTS stage effect on survival outcomes

  6. Prognosis of patients with somatic LMS • Scandinavian Sarcoma Group, 225 pts (2006)5 • 10 year survival rate (with localized disease): 49% • Higher grade, larger tumor size, deeper location all significantly correlated with decreased survival • Overall prognosis is poor even with local control (w/ or w/o radiotherapy) • Abraham et al. 115 pts (2012)1 • 1, 5, and 10 year survival: 87%, 57%, 19% • Histological grade, tumor depth, and mets significant predictors of mortality

  7. Prognosis of patients with somatic LMS • Radkowski et al., 65 pts – 20125 • Overall 1, 2, and 5 year survival: 91%, 87%, and 68% • Pts with deep lesions, grade 3 disease, and advanced stage have poorer prognosis

  8. Uterine LMS • Uterine LMS is rare, it accounts for only 1% of all uterine cancers6, however it represents up to 1/3 of all uterine sarcomas • 30-35% of patients have evidence of extra-uterine disease at the time of presentation • 5 year survival for metastatic disease is less than 40% with current therapies • Median age: 50 years, about 10 years older than the median age of presentation for leiomyoma7 • Frequency of incidental LMS detected in women at various ages having surgery for presumed leiomyoma: • 31-40 y: 0.2% • 41-50 y: 0.9% • 51-60 y: 1.4% • 61-81 y: 1.7%

  9. Motivation for study • Does extra-uterine LMS have the same prognosis as uterine LMS? • Some patients with extra-uterine LMS had hysterectomies in the past • Difference in outcomes between those with and those without hysterectomies? • Two distinct disease entities (uterine LMS and extra-uterine LMS) may actually overlap, with patients presenting decades later with recurrences of uterine LMS.

  10. Hypothesis • Patients with uterine LMS have decreased survival compared to extra-uterine LMS • Of patients with extra-uterine LMS, those with prior hysterectomies have decreased survival

  11. Methods • Searched using MARS (Medical Archive Record System) in UPMC system from 1982-present • Surgical pathology reports with designated search terms • Retrieved lists were filtered for correct diagnoses • Diagnosis of extra-uterine LMS • 513 cases of extra-uterine LMS • Included tumors from: GI, cutaneous, peripheral soft tissue, retroperitoneal, vascular, etc. • Diagnosis of uterine LMS • 127 patients

  12. Methods • Patient characteristics identified: • age at diagnosis • tumor characteristics (size, # mitoses, % necrosis, grade, FIGO stage) • metastases • Survival data collected from: • UPMC patient charts (inpatient and outpatient visits) • National and municipal registries • Censored survival data used to construct Kaplan Meier curves

  13. Results

  14. Results

  15. Results – analysis of patients with Extra-uterine LMS • Of the 513 patients with extra-uterine LMS: • 269 were females • 177 with no documentation of hysterectomies • 75 found to have hysterectomies prior to dx of LMS • 17 had hysterectomies after dx of LMS • 75/269 = 27.9% of females with hysterectomies prior to dx of LMS • Differences in survival?

  16. Results – analysis of patients with Extra-uterine LMS

  17. Discussion • Uterine vs. extra-uterine LMS has similar long term outcomes, not statistically significant • Median survival: 4.97 y vs. 4.28 y • HR 1.042, p = 0.74 • Among extra-uterine LMS, those with prior hysterectomies had a longer survival time, not statistically significant • Median: 6.42 y vs. 4.25 y • p = 0.084

  18. Limitations • Population heterogeneity • Does not stratify for commonly used clinical parameters: • Margin status • XRT • Chemotherapy • Other treatments

  19. Conclusion • Retrospective review of 640 cases of LMS • Uterine • Extra-uterine • No difference in long term survival outcomes • Future directions: • Evaluate contribution of clinical parameters • Outcomes of patients with somatic soft tissue LMS

  20. References • Abraham et al. Outcomes and prognostic factors for a consecutive case series of 115 patients with somatic leiomyosarcoma. J Bone Joint Surg Am. 2012 Apr 18; 94(8): 736-44 • O’Sullivan et al. Radiological imaging features of non-uterine leiomyosarcoma. Br J Radiol. 2008 Jan; 81(961): 73-81 • Farshid et al. Leiomyosarcoma of Somatic Soft Tissues. Am J SurgPathol. 2002. 26(1): 14–24. • Mankin et al. Leiomyosarcoma of Somatic Soft Tissues. ClinOrthopRelat Res. 2004 Apr ; (421): 225-31 • Radkowski et al. Leiomyosarcoma of the somatic soft tissues. J SurgOrthop Adv. 2012. 21(2): 96-101 • Leitao et al. Surgical cytoreduction in patients with metastatic uterine leiomyosarcoma at the time of initial diagnosis. GynecolOncol. 2012 May. 125(2): 409-413

  21. Acknowledgments • Meghan Levy • Alma Heyl – Clinical Research Coordinator • Kurt Weiss, MD and Mark Goodman, MD • Richard McGough, MD – Project Mentor

  22. Thank you

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