"Rhabdomyosarcoma in children" by Prof Larry Hadley, Durban, South Africa. This is the recording of an invited talk as a part of the Zoom academic meetings of the Department of Paediatric Surgery in East London, South Africa.
"Rhabdomyosarcoma in children" by Prof Larry Hadley, Durban, South Africa
Summary
Join us for an insightful on-demand teaching session that unveils the complexities of Rhabdomyosarcoma, a cancer subtype commonly faced by medical professionals. Led by an experienced professional, this compelling session will delve deep into preferable and non-preferable sites and histology of RMS, its staging, groups, risk groups, and more. We'll also explore factors that affect survival rates & diagnostic procedures like genetic testing and imaging. Learn the best practices in RMS treatment, including pointers on chemotherapy, surgery, and radiotherapy. The session includes case studies and discusses special situations requiring unique approaches. Secure your seat and deepen your understanding of Rhabdomyosarcoma.
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Description
Learning objectives
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By the end of this teaching session, the participant should understand the biology and histology of Rhabdomyosarcoma, including why traditional classification systems can be confusing and the implications of favourable/unfavourable sites and histology.
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Participants should be able to identify and categorize different stages and groups of Rhabdomyosarcoma accurately, including understanding of the tumour size, node involvement and metastasis status related to each stage.
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Upon completion of the session, participants should understand the associated risk factors for Rhabdomyosarcoma and be able to classify patients into low, standard, intermediate, and high-risk categories and relate them to the overall survival.
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Participants should be able to identify and understand the necessary diagnostic tests for Rhabdomyosarcoma, including biopsy techniques, imaging options, and the importance and relevance of genetic evaluations.
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By the conclusion of the session, participants should be aware of the treatment modalities used to manage this condition and should also know the special considerations to be made under different clinical scenarios such as paratesticular tumour in boys, scrotal radiotherapy etc.
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RhabdomyosarcomaAntonio Scarpa 1752-1832 Why Rhabdomyosarcoma is confusing! Favourable/Unfavourable sites Favourable/Unfavourable histology Stages Groups RiskGroupsRMS; Sites (favourable) 1/3 Head & Neck (orbit, non-parameningeal) 1/3 Elsewhere 1/3 Genitourinary (paratesticular, female UGT) RMS Histology (myogenin positive) Embryonal 70% LOH 11p13 Alveolar t(2;13) (q35;q14) 20% Undifferentiated 10% RMS Staging Stage Site Tumour Size Nodes Mets 1 F T1 or T2 any N0,N1,Nx neg 2 UnF T1 or T2 <5 cm N0,Nx neg 3a UnF T1 or T2 <5 cm N1 neg 3b UnF T1 or T2 >5cm N0,N1,Nx neg 4 F or UnF T1 or T2 any N0,N1,Nx pos RMS Groups Group Definition I Complete resection, negative margins IIa Complete resection, micro positive margins IIb Complete resection, negative margins but N1 IIc Complete resection, positive margins and N1 III Gross residual disease IV Metastases RMS; Risk Factors Low risk; Stage 1, Group I or II, favourable site OS >85%onal Standard risk; Stage 1 or 2, Group III, favourable site , favourable histology OS>80% Intermediate risk; Majority of patients! Unfavourable site, Group III, Stages 2 or 3, Non-metastatic alveolar, Metastatic Embryonal OS +/- 50% High risk; Infants with metastatic disease, Limb tumour with N1, metastatic alveolar tumours, metastatic embryonal tumours in children >10 years. OS+/- 20% Diagnostic tests Biopsy SRBCT; striations Myogenin positive Bone marrow Lumbar puncture ( for parameningeal sites) Imaging; whatever is available; US, CXR, CT, MRI, PET Genetics; PCR; PX-FKHR fusion, IGFII, RMS treatment Biopsy ; needle incisional excisional Chemotherapy; Vincristine, Actinomycin, Cyclophosphamide Surgery; Radiotherapy ; External beam BrachytherapyBut if you don’t know…… ….Biopsy Rhabdomyosarcoma Special situations 1.Paratesticular tumour in boy >10 year s Surgery should include orchidectomy and para-aortic LN dissection 2.Pelvic radiotherapy in girls and scrotal radiotherapy Surgically move gonads out of field. 3.Limb tumours; always biopsy nodes at knee, elbow and root of limb 4.Bladder/prostate is an unfavourable site Organ preservation is not as important as life preservationSarcoma botryoides