Li-Fraumeni Syndrome


A PACIFIST'S GUIDE TO THE WAR ON CANCER
Interview with collaborator Lara Veitch. Lara has Li-Fraumeni Syndrome, a rare inherited genetic predisposition towards cancer and she is a trustee of The George Pantziarka TP53 Trust
A Complicité Associates and National Theatre Co-production in association with HOME Manchester.
Directed by Bryony Kimmings
Book by Bryony Kimmings and Brian Lobel
Music by Tom Parkinson
Lyrics by Bryony Kimmings
www.complicite.org


12-year-old female with gradual onset of the odynophagia. There is a large, partly circumscribed, heterogeneous, T1 slightly hypointense to skeletal muscle, T2/STIR hyperintense mass centered within the right masticator space. The mass enhances solidly and extends to the skull base on the right with involvement of Meckel's cave via the foramen ovale. This is a case of rhabdomyosarcoma which is a malignant tumor with skeletal muscle cell morphology. Rhabdomyosarcomatous are the most common soft tissue tumor in children accounting for approximately 25% of all pediatric soft tissue sarcomas. The majority occur in patients under 10 years of age. There is a slight male predilection. Clinical presentation depends on the tumor location. Associations include neurofibromatosis type I, Beckwith Weidman syndrome, and Li-Fraumeni syndrome. Treatment is with a combination of surgery, chemotherapy, and radiation. 15NMR263
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