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Li-Fraumeni Syndrome-related Malignancies Involving the Genitourinary Tract: Review of a Single-institution Experience.

Publication ,  Journal Article
Murray, KS; Spaliviero, M; Tonorezos, ES; Lacouture, ME; Tap, WD; Oeffinger, KC; Vargas, HA; Eastham, JA
Published in: Urology
September 2018

OBJECTIVE: To report a case of pelvic angiosarcoma in a 27-year-old man with Li-Fraumeni Syndrome (LFS) and evaluate the presentation and timeline of genitourinary (GU) tract involvement in LFS patients. METHODS: We retrospectively identified 39 LFS patients treated at our institution between 2000 and 2014; 7 (18%) had experienced a GU malignancy or an LFS-related malignancy involving the GU tract. Clinical characteristics, including dates of onset of first GU tract malignancies; pathologic findings; multimodal management; and familial history of LFS were reviewed. RESULTS: Median age at first malignancy was 14.0 years (interquartile range [IQR] 5.5-24.0). There was a slight male predominance (4 of 7). Median time between first malignancy and the malignancy involving the GU tract was 10.1 years (IQR 8.0-19.5). Six of the 7 patients (86%) had a form of sarcoma involving the GU tract; 1 developed adrenocortical carcinoma. The cancer pedigree of all patients showed LFS-associated malignancies in family members. Multimodal management included surgical resection in 6 patients with adjuvant chemotherapy or radiotherapy in 1 patient each. One patient received chemotherapy only. Following diagnosis of malignancy involving the GU tract, 5 of the 7 patients developed additional primary malignancies. At a median follow-up of 4.7 years (IQR 3.0-12.1), 2 patients are alive, 3 died of disease, and 1 died of unknown cause. One patient was lost at follow-up. CONCLUSION: Continued follow-up of LFS cancer patients aimed at the determination of optimal screening, management, and surveillance protocols is recommended and may result in longer survival expectations.

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Published In

Urology

DOI

EISSN

1527-9995

Publication Date

September 2018

Volume

119

Start / End Page

55 / 61

Location

United States

Related Subject Headings

  • Urology & Nephrology
  • Urogenital Neoplasms
  • Retrospective Studies
  • Male
  • Li-Fraumeni Syndrome
  • Humans
  • Fatal Outcome
  • Adult
  • 3202 Clinical sciences
  • 1103 Clinical Sciences
 

Citation

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ICMJE
MLA
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Murray, K. S., Spaliviero, M., Tonorezos, E. S., Lacouture, M. E., Tap, W. D., Oeffinger, K. C., … Eastham, J. A. (2018). Li-Fraumeni Syndrome-related Malignancies Involving the Genitourinary Tract: Review of a Single-institution Experience. Urology, 119, 55–61. https://doi.org/10.1016/j.urology.2018.06.008
Murray, Katie S., Massimiliano Spaliviero, Emily S. Tonorezos, Mario E. Lacouture, William D. Tap, Kevin C. Oeffinger, Hebert Alberto Vargas, and James A. Eastham. “Li-Fraumeni Syndrome-related Malignancies Involving the Genitourinary Tract: Review of a Single-institution Experience.Urology 119 (September 2018): 55–61. https://doi.org/10.1016/j.urology.2018.06.008.
Murray KS, Spaliviero M, Tonorezos ES, Lacouture ME, Tap WD, Oeffinger KC, et al. Li-Fraumeni Syndrome-related Malignancies Involving the Genitourinary Tract: Review of a Single-institution Experience. Urology. 2018 Sep;119:55–61.
Murray, Katie S., et al. “Li-Fraumeni Syndrome-related Malignancies Involving the Genitourinary Tract: Review of a Single-institution Experience.Urology, vol. 119, Sept. 2018, pp. 55–61. Pubmed, doi:10.1016/j.urology.2018.06.008.
Murray KS, Spaliviero M, Tonorezos ES, Lacouture ME, Tap WD, Oeffinger KC, Vargas HA, Eastham JA. Li-Fraumeni Syndrome-related Malignancies Involving the Genitourinary Tract: Review of a Single-institution Experience. Urology. 2018 Sep;119:55–61.
Journal cover image

Published In

Urology

DOI

EISSN

1527-9995

Publication Date

September 2018

Volume

119

Start / End Page

55 / 61

Location

United States

Related Subject Headings

  • Urology & Nephrology
  • Urogenital Neoplasms
  • Retrospective Studies
  • Male
  • Li-Fraumeni Syndrome
  • Humans
  • Fatal Outcome
  • Adult
  • 3202 Clinical sciences
  • 1103 Clinical Sciences