Sarcoma (Ewing/Rhabdo)
Image-guided percutaneous biopsy should be strongly considered the diagnostic modality of choice for pediatric bone and soft tissue tumors. — Sophia Schermerhorn, Pediatric and Young Adult Image-Guided Percutaneous Bone Biopsy-A New Standard of Care? · 0:56
Extensive workup should be reserved for patients who are hemodynamically unstable or have more overt signs of infection — Sophia Schermerhorn, Early Postoperative Fever in Pediatric Oncology Patients Undergoing Solid Tumor Resection · 0:45
Yama would perform bronchoscopy to identify the involved segment, then thoracotomy (not thoracoscopy) in conjunction with intraoperative bronchoscopy for a patient with multiple prior operations. — Marco, Difficult Cases of Lung Lesions: Pediatric Thoracic Surgery Part 1-Lung... · 8:36
Patients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease. — Peter Ehrlich, Wilms Tumor: Audio Chapter · 55:07
Patients requiring surgical intervention for circumcision include infants over 3 months of age or weighing more than 13 pounds, who cannot safely undergo office circumcision. Additionally, patients with isolated chordee without hypospadias benefit from operative circumcision to release adhesions and straighten the penis. Conversely, elective circumcision in infants under 3 months should not require general anesthesia due to anesthetic safety concerns.
Diagnosis of soft tissue sarcomas (rhabdo and non-rhabdo) involves imaging and tissue sampling. The workup includes MRI and either incisional or core biopsy. Histological grade is critical in non-rhabdomyosarcoma soft tissue sarcomas for treatment decisions. For regional disease assessment, sentinel node biopsy is performed because clinically negative nodes may harbor occult disease; 40–50% of biopsied nodes are positive, and positive nodes carry worse prognosis and require radiation therapy.
Todd Ponsky discussed surgical decision-making for non-rhabdomyosarcoma soft tissue sarcomas. He presented a study comparing outcomes between patients who had unplanned excision of lesions later found to be non-rhabdomyosarcoma followed by re-excision versus those with planned workup and excision. The study involved 40 patients referred after innocuous lesion removal that returned as non-rhabdomyosarcoma. Ponsky concluded that since there is no outcome difference between planned and unplanned excision, it may be acceptable with low index of suspicion to excise the lesion and perform re-excision if it returns as non-rhabdomyosarcoma.
- Sentinel node biopsy is required for trunk/extremity rhabdo; 40–50% are positive, managed with radiation not completion dissection.
- Unplanned sarcoma excision yields 45.7% residual tumor but no survival difference versus planned resection; re-excise rather than accept primary morbidity.
- Solitary pulmonary Ewing relapse: 73% three-year survival with metastasectomy ± whole-lung RT versus 40% for multiple nodules—aggressive local control warranted.
