Anaplasia in Paediatric Rhabdomyosarcoma

  • Research type

    Research Study

  • Full title

    Anaplasia in Paediatric Rhabdomyosarcoma

  • IRAS ID

    248202

  • Contact name

    Anna Kelsey

  • Contact email

    anna.kelsey@mft.nhs.uk

  • Sponsor organisation

    Manchester University Hospitals NHS Foundation Trust

  • Duration of Study in the UK

    1 years, 11 months, 30 days

  • Research summary

    In children, soft tissue sarcomas (malignant tumour of connective tissue) are the 4th most common type of cancer. Rhabdomyosarcoma (RMS) is a soft tissue sarcoma that predominantly affects children and accounts for approximately half of childhood soft tissue sarcomas.

    In children, the most common histomorphological (microscopic cellular / tissue appearance) subtypes of RMS are embryonal and alveolar. In adults, a third subtype, pleomorphic RMS occurs in roughly equal proportions to embryonal and alveolar subtypes and has a poor prognosis. The pleomorphic subtype is rarely seen in children. When ‘pleomorphic’ features are seen in tumours from children it is called RMS with anaplasia. Children diagnosed with RMS with anaplasia have worse prognosis compared to RMS without anaplasia. This association with a poor prognosis suggests that paediatric RMS with anaplasia may be similar to adult pleomorphic RMS.

    We hypothesise that paediatric RMS with anaplasia and adult pleomorphic RMS are similar entities. If this hypothesis is proven it will have far reaching implications for the classification of tumours, prognostication and treatment options. To test this hypothesis, this study will describe and compare the clinical, histomorphological and molecular profiles of paediatric RMS with anaplasia and adult pleomorphic RMS. A control group of paediatric patients with RMS without anaplasia will also be studied to define the impact of the presence of anaplasia in the population from which the cohort is recruited.

    The study will use surplus FFPE tissue, previously collected for diagnostic purposes. Cases of paediatric RMS will be taken from the archives of the Paediatric Histology Department at the Royal Manchester Children’s Hospital, Manchester University NHS Foundation Trust. Cases of adult pleomorphic RMS will be taken from the archives of the Histopathology Department at the Christie Pathology Partnership, The Christie NHS Foundation Trust.

  • REC name

    West of Scotland REC 3

  • REC reference

    19/WS/0196

  • Date of REC Opinion

    12 Dec 2019

  • REC opinion

    Favourable Opinion