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

Panda, Sudhakar; Chinnaswamy, Girish; Vora, Tushar; Prasad, Maya; Bansal, Deepak; Kapoor, Gauri; Radhakrishnan, Venkatraman; Agarwala, Sandeep; Laskar, Siddharth; Arora, Brijesh; Kaur, Tanvir; Rath, GK; Bakhshi, Sameer

doi:10.1007/s12098-017-2315-3

Cited by 41 publications

(67 citation statements)

References 25 publications

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“…Incorporating other anticancer agents into the VAC backbone such as ifosfamide, etoposide, carboplatin, irinotecan and vinorelbine in order to improve the outcome rates requires further clinical trials [2]. New molecular targets being explored include inhibiting tyrosine kinase receptors such as epidermal growth factor receptor (erlotinib, geftinib), platelet derived growth factor receptor (olaratumab, dasatinib, pazotinib, sorafenib), vascular endothelial growth factor receptor (sunitinib, apatinib) and anaplastic lymphoma kinase receptor (crizotinib, ceritinib) [6].…”

Section: Resultsmentioning

confidence: 99%

“…Rhabdomyosarcoma is a relatively rare mesodermal cancer with an estimated annual incidence of 4.3 cases per one million people younger than 20 years of age [1]. Common sites of the primary disease include the head and neck region, genitourinary tract and extremities [2]. Literature about rhabdomyosarcoma located in the gluteal region is sparse.…”

Section: Discussionmentioning

confidence: 99%

“…It is attributed to around 5% of all paediatric cancers [1]. Common affected sites include the head and neck region, genitourinary tract, retroperitonium and, to a lesser extent the extremities [2]. The most common histological variants encountered are the embryonal and alveolar subtypes.…”

Section: Introductionmentioning

confidence: 99%

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Rhabdomyosarcoma of the Gluteus Maximus – Case Report, Review of Literature and Emerging Therapeutic Targets

Meshram¹,

Kaur²,

Hura³

2019

Klin Onkol

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Background: Rhabdomyosarcoma is an uncommon mesodermal cancer, which predominantly affects the young population. Common sites of primary disease include the head and neck region, genitourinary tract and the extremities. Less than 25% of the cases of rhabdomyosarcoma are metastatic at presentation. Embryonal and alveolar are the most common histological subtypes of rhabdomyosarcoma. Literature about rhabdomyosarcoma located in the gluteal region is sparse. Case: We present a case of a 3-year-old child with alveolar rhabdomyosarcoma arising from the gluteal region. The metastatic workup was negative. We determined the tumour to be of intermediate risk and managed the patient with systemic chemotherapy consisting of cycles of vincristine, actinomycin D and cyclophosphamide, along with local treatment (wide-margin excision and radiotherapy). No recurrence was observed in the follow-up period. Conclusion: Management of alveolar rhabdomyosarcoma of the gluteus maximus requires a multipronged approach consisting of systemic chemotherapy, local surgery and radiotherapy. Long-term surveillance is imperative in children for early identification and management of relapses and treatment-related adverse effects. Several bio logical agents and small-molecule inhibitors targeting the signalling and growth pathways of rhabdomyosarcoma are in the pipeline, which hold promise for personalised therapy in the future. However, due to the rarity and molecular heterogenicity of the tumour, integrating these novel agents with the existing therapy would be a challenge.

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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Rhabdomyosarcoma of the Gluteus Maximus – Case Report, Review of Literature and Emerging Therapeutic Targets

Meshram¹,

Kaur²,

Hura³

2019

Klin Onkol

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“…9 La radioterapia ha sido descrita como un tratamiento adyuvante efectivo para los sarcomas de partes blandas, principalmente, por su potencial de reducir el riesgo de recidiva local. 10,11 Debido a los posibles efectos adversos en este grupo etario en particular, no suele utilizarse cuando los márgenes quirúrgicos son satisfactorios. 12 De los 6 pacientes incluidos en el estudio, solo 1 recibió radioterapia.…”

Section: Discussionunclassified

Sarcomas de partes blandas en pacientes menores de 2 años. Serie de casos

et al. 2020

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Presentación de casos clínicos RESUMEN Los sarcomas de partes blandas en menores de 2 años son infrecuentes. Durante 2007-2017, 445 pacientes con sarcomas de partes blandas fueron tratados en nuestra Institución; 6 (el 0,5 %) eran menores de 2 años. Se analizaron los resultados clínicos y oncológicos en este grupo. La edad media de diagnóstico fue 15 meses. Cuatro eran varones y 2, mujeres. El seguimiento promedio fue 29 meses. El fibrosarcoma (n = 4) fue la variedad más frecuente. Cinco fueron tratados con cirugía de conservación del miembro; al restante se le realizó amputación. Todos realizaron tratamiento adyuvante con quimioterapia. La supervivencia a 24 meses fue del 100 %. Dos pacientes presentaron recidiva local; ambos casos, antes de los 24 meses. El tratamiento quirúrgico asociado a la quimioterapia impresiona ser la mejor opción terapéutica. La proporción de recurrencia local es alta para este grupo de pacientes luego de la cirugía de conservación del miembro.

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“…These recommendations are based on a detailed review of available Indian and international literature, and expert opinion has been used where adequate evidence was lacking. These guidelines cover the common pediatric tumors, including Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL), rhabdomyosarcoma, Wilms tumor, neuroblastoma, hepatoblastoma and germ cell tumor [1][2][3][4][5][6][7]. The prime focus of these guidelines includes diagnostic workup, and medical and surgical management of pediatric tumors.…”

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confidence: 99%

Editorial: Indian Guidelines for Treatment of Pediatric Malignancies

Tiwari

Bakhshi

2017

Indian J Pediatr

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Treatment strategies and protocols for pediatric tumors vary by health provider, treating center and geographical area within the country. The problem has been compounded by the rapid evolution in the world of oncology and advent of new technologies and treatment options. The cost of health care has also increased manifold in the recent times; further, all treatment options and modalities of diagnosis are not equally available in all places. This urges a need for a national guideline for management of pediatric tumors, which are made by experts in the field and with an Indian perspective in mind.These guidelines have been made with intent to bridge the gap between evidence and practice in the country, and make patient care more consistent. A multidisciplinary team of experts has contributed to the development of these guidelines. These recommendations are based on a detailed review of available Indian and international literature, and expert opinion has been used where adequate evidence was lacking. These guidelines cover the common pediatric tumors, including Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL), rhabdomyosarcoma, Wilms tumor, neuroblastoma, hepatoblastoma and germ cell tumor [1][2][3][4][5][6][7]. The prime focus of these guidelines includes diagnostic workup, and medical and surgical management of pediatric tumors. The guidelines are largely consistent with the pediatric oncology guidelines by other international bodies.The review is complete and includes the most-recent abstracts and articles. For example, the guidelines have accepted the results of the interim analysis of Inter-B-NHL Ritux 2010 trial and recommend the use of rituximab in high-risk B-NHL along with intensive chemotherapy protocol [8]; use of rituximab is also recommended in patients with relapsed B-NHL.The prime benefit of having an evidence-based guideline is that it helps patients from across the country to get standard, evidence-based uniform care. This will ensure that the quality of care is not compromised based on where and by whom the patient is being treated, and is likely to improve the outcomes of these patients and decrease the disease-related morbidity and mortality. The cost-effectiveness of management of these tumors is also likely to improve by eliminating unnecessary diagnostic evaluations and treatment that add to cost but are not based on evidence. Few examples of such practices are use of fine needle aspiration cytology (FNAC) for diagnosis of lymphomas and frequent use of imaging in the follow-up of these patients. The guidelines on HL specifically discourage use of FNAC for diagnosis and also limit imaging studies in follow-up period [1]. This will sensitize local practitioners and small centers to move away from these practices.These guidelines also address some of the ground realities of the country; for example, it stresses upon the fact that neoadjuvant chemotherapy followed by surgery is the preferred approach in children with Wilms tumors, keeping in mind the size of the tumors at presentation, and ...

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

Cited by 41 publications

References 25 publications

Rhabdomyosarcoma of the Gluteus Maximus – Case Report, Review of Literature and Emerging Therapeutic Targets

Rhabdomyosarcoma of the Gluteus Maximus – Case Report, Review of Literature and Emerging Therapeutic Targets

Sarcomas de partes blandas en pacientes menores de 2 años. Serie de casos

Editorial: Indian Guidelines for Treatment of Pediatric Malignancies

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