A patient-derived xenograft model of parameningeal embryonal rhabdomyosarcoma for preclinical studies

Jody E. Hooper, Emma L. Cantor, Macgregor S. Ehlen, Avirup Banerjee, Suman Malempati, Peter Stenzel, Randall (Randy) Woltjer, Regina Gandour-Edwards, Neal C. Goodwin, Yan Yang, Pali Kaur, Carol J. Bult, Susan D. Airhart, Charles Keller

Research output: Contribution to journalArticle

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Abstract

Embryonal rhabdomyosarcoma (eRMS) is one of the most common soft tissue sarcomas in children and adolescents. Parameningeal eRMS is a variant that is often more difficult to treat than eRMS occurring at other sites. A 14-year-old female with persistent headaches and rapid weight loss was diagnosed with parameningeal eRMS. She progressed and died despite chemotherapy with vincristine, actinomycin-D, and cyclophosphamide plus 50.4 Gy radiation therapy to the primary tumor site. Tumor specimens were acquired by rapid autopsy and tumor tissue was transplanted into immunodeficient mice to create a patient-derived xenograft (PDX) animal model. As autopsy specimens had an ALK R1181C mutation, PDX tumor bearing animals were treated with the pan-kinase inhibitor lestaurtinib but demonstrated no decrease in tumor growth, suggesting that single agent kinase inhibitor therapy may be insufficient in similar cases. This unique parameningeal eRMS PDX model is publicly available for preclinical study.

Original languageEnglish (US)
Article number826124
JournalSarcoma
Volume2015
DOIs
StatePublished - 2015

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Embryonal Rhabdomyosarcoma
Heterografts
Neoplasms
Autopsy
Phosphotransferases
Radiation Dosage
Dactinomycin
Vincristine
Sarcoma
Cyclophosphamide
Headache
Weight Loss
Radiotherapy
Animal Models
Drug Therapy
Mutation
Growth

ASJC Scopus subject areas

  • Oncology
  • Radiology Nuclear Medicine and imaging

Cite this

A patient-derived xenograft model of parameningeal embryonal rhabdomyosarcoma for preclinical studies. / Hooper, Jody E.; Cantor, Emma L.; Ehlen, Macgregor S.; Banerjee, Avirup; Malempati, Suman; Stenzel, Peter; Woltjer, Randall (Randy); Gandour-Edwards, Regina; Goodwin, Neal C.; Yang, Yan; Kaur, Pali; Bult, Carol J.; Airhart, Susan D.; Keller, Charles.

In: Sarcoma, Vol. 2015, 826124, 2015.

Research output: Contribution to journalArticle

Hooper, JE, Cantor, EL, Ehlen, MS, Banerjee, A, Malempati, S, Stenzel, P, Woltjer, RR, Gandour-Edwards, R, Goodwin, NC, Yang, Y, Kaur, P, Bult, CJ, Airhart, SD & Keller, C 2015, 'A patient-derived xenograft model of parameningeal embryonal rhabdomyosarcoma for preclinical studies', Sarcoma, vol. 2015, 826124. https://doi.org/10.1155/2015/826124
Hooper, Jody E. ; Cantor, Emma L. ; Ehlen, Macgregor S. ; Banerjee, Avirup ; Malempati, Suman ; Stenzel, Peter ; Woltjer, Randall (Randy) ; Gandour-Edwards, Regina ; Goodwin, Neal C. ; Yang, Yan ; Kaur, Pali ; Bult, Carol J. ; Airhart, Susan D. ; Keller, Charles. / A patient-derived xenograft model of parameningeal embryonal rhabdomyosarcoma for preclinical studies. In: Sarcoma. 2015 ; Vol. 2015.
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