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Paeds Vivashaematology-oncology-and-transfusion

Paeds Vivas · haematology-oncology-and-transfusion

Bone and soft-tissue sarcomas — branching viva

Branching viva on bone and soft-tissue sarcomas: recognising osteosarcoma from the persistent non-mechanical limb pain and the radiographic pattern, applying the never-unplanned-biopsy rule and the staging panel, naming the chemotherapy backbones, and branching to the Ewing sarcoma that mimics osteomyelitis and the rhabdomyosarcoma with its fusion-driven risk stratification.

branching clinical structured oral1 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Study tools

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
Outpatient clinic: a thirteen-year-old boy with three months of deep night pain in the distal thigh and a firm mass just above the knee, and a plain radiograph showing a mixed lytic and sclerotic distal femoral metaphyseal lesion with sunburst spicules and a Codman triangle. The examiner asks: name the diagnosis, give the staging panel and the biopsy principle, then branches to the chemotherapy backbone and the histologic response, to the same lesion re-presenting as a diaphyseal permeative lesion with systemic symptoms that reframes it as Ewing sarcoma and its EWSR1-FLI1 fusion, and finally to a head-and-neck mass that opens the rhabdomyosarcoma and its PAX-FOXO1-driven risk groups.

Opening question

A thirteen-year-old boy has three months of deep night pain in the distal thigh and a firm mass above the knee, and his radiograph shows a mixed lytic and sclerotic distal femoral metaphyseal lesion with sunburst spicules and a Codman triangle. Name the diagnosis, justify the radiographic features, and give the staging panel and the biopsy principle. [1]

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References4ShowHide
  1. [1]Isakoff MS, Bielack SS, Meltzer P, Gorlick R Osteosarcoma: Current Treatment and a Collaborative Pathway to Success. J Clin Oncol, 2015.PMID 26304877
  2. [2]Grünewald TGP, Cidre-Aranaz F, Surdez D, et al. Ewing sarcoma. Nat Rev Dis Primers, 2018.PMID 29977059
  3. [3]Malempati S, Hawkins DS Rhabdomyosarcoma: review of the Children's Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and rationale for current COG studies. Pediatr Blood Cancer, 2012.PMID 22378628
  4. [4]Gartrell J, Pappo A Recent advances in understanding and managing pediatric rhabdomyosarcoma. F1000Res, 2020.PMID 32695311
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