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Diagnosis and Management of Rhabdomyosarcoma in Children and Adolescents: ICMR Consensus Document

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Abstract

Rhabdomyosarcoma (RMS) is a highly malignant tumor which is thought to originate from the pluripotent mesenchyme. It is the most common soft-tissue sarcoma of childhood. This review article summarizes the recent and older published literature and gives an overview of management of RMS in children. RMS can arise in a wide variety of primary sites, some of which are associated with specific patterns of local invasion, regional lymph nodal spread, therapeutic response and long term outcome, hence requiring physicians to be familiar with site-specific staging and treatment details. Most common primary sites include the head and neck region, genitourinary tract, and extremities. Prognosis for children and adolescents with RMS has recently improved substantially, especially for patients with local or locally extensive disease because of the development of multi-modal therapy incorporating surgery, dose-intensive combination chemotherapy, and radiation therapy. Despite aggressive approaches the outcome for patients who present with metastatic disease remains unsatisfactory. Clinical trials are ongoing to reduce toxicity and improve outcomes of such patients; newer agents in combination are being investigated.

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Acknowledgements

This article is prepared as an outcome of Indian Council of Medical Research (ICMR) Sub-Committee on Pediatric Lymphomas and Solid Tumors coordinated by the Division of Non-Communicable Diseases, ICMR.

Contributions

GC, TK, GKR, SB, DB, SL and BA: Concept and design; SPP, GC, MP, GK and VR: Analysis and interpretation; SPP, MP, TV, DB and VR: Drafting of manuscript; GC, TV, SB, DB, SA, SL and BA: Critical revision. SB will act as guarantor for this paper.

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Authors and Affiliations

  1. Department of Pediatric Oncology, Tata Memorial Hospital, Parel, Mumbai, India

    Saroj Prasad Panda, Girish Chinnaswamy, Tushar Vora, Maya Prasad & Brijesh Arora

  2. Pediatric Hematology Oncology Unit, Department of Pediatrics, Advanced Pediatric Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India

    Deepak Bansal

  3. Department of Pediatric Hematology and Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India

    Gauri Kapoor

  4. Department of Medical Oncology and Pediatric Oncology, Cancer Institute (W.I.A), Adyar, Chennai, India

    Venkatraman Radhakrishnan

  5. Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India

    Sandeep Agarwala

  6. Department of Radiation Oncology, Tata Memorial Hospital, Parel, Mumbai, India

    Siddharth Laskar

  7. NCD Division, Indian Council of Medical Research (ICMR), New Delhi, India

    Tanvir Kaur

  8. Dr. B.R.A Institute-Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India

    G. K. Rath

  9. Department of Medical Oncology, Dr. B.R.A Institute-Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India

    Sameer Bakhshi

Authors
  1. Saroj Prasad Panda
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  2. Girish Chinnaswamy
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  3. Tushar Vora
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  4. Maya Prasad
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  7. Venkatraman Radhakrishnan
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  9. Siddharth Laskar
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  10. Brijesh Arora
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  11. Tanvir Kaur
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  12. G. K. Rath
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Corresponding author

Correspondence to Girish Chinnaswamy.

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ICMR organized the meeting and funded the travel.

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Panda, S.P., Chinnaswamy, G., Vora, T. et al. Diagnosis and Management of Rhabdomyosarcoma in Children and Adolescents: ICMR Consensus Document. Indian J Pediatr 84, 393–402 (2017). https://doi.org/10.1007/s12098-017-2315-3

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  • DOI: https://doi.org/10.1007/s12098-017-2315-3

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