Implications of Tumor Characteristics and Treatment Modality on Local Recurrence and Functional Outcomes in Children With Chest Wall Sarcoma

Abstract

Objective: To determine the impact of tumor characteristics and treatment approach on (1) local recurrence, (2) scoliosis development, and (3) patient-reported quality of life in children with sarcoma of the chest wall. Summary of Background Data: Children with chest wall sarcoma require multimodal therapy including chemotherapy, surgery, and/or radiation. Despite aggressive therapy which places them at risk for functional impairment and scoliosis, these patients are also at significant risk for local recurrence. Methods: A multi-institutional review of 175 children (median age 13 years) with chest wall sarcoma treated at seventeen Pediatric Surgical Oncology Research Collaborative institutions between 2008 and 2017 was performed. Patient-reported quality of life was assessed prospectively using PROMIS surveys. Results: The most common diagnoses were Ewing sarcoma (67%) and osteosarcoma (9%). Surgical resection was performed in 85% and radiation in 55%. A median of 2 ribs were resected (interquartile range = 1-3), and number of ribs resected did not correlate with margin status (P = 0.36). Local recurrence occurred in 23% and margin status was the only predictive factor (HR 2.24, P = 0.039). With a median follow-up of 5 years, 13% developed scoliosis (median Cobb angle 26) and 5% required corrective spine surgery. Scoliosis was associated with posteriorrib resection (HR 8.43; P= 0.003) and increased number of ribs

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

“…Surgical resection can precede other treatment modalities only if it achieves negative margins and if disfigurement and loss of function are avoided. This concept must be emphasised, primary massive resections are absolutely contraindicated, leading to important long-term effects for the patient (including rib wall defects, scoliosis [ 36 , 37 ] or pulmonary impairment).…”

“…Patients who undergo chest wall resection during periods of rapid growth have posterior chest wall tumours, and who undergo resection of two or more ribs are the most likely to develop scoliosis and should be monitored for this long-term effect of surgery [ 15 ]. A multi-institutional long-term follow-up study of 175 patients who underwent surgery for chest wall sarcoma showed that 13% developed scoliosis and 5% required corrective spine surgery [ 16 ].…”

Can We Compare the Health-Related Quality of Life of Childhood Cancer Survivors Following Photon and Proton Radiation Therapy? A Systematic Review

“…A total of 14 studies are cross-sectional, 15 studies are longitudinal, and 1 study uses both longitudinal and cross-sectional data collection. Articles are included from the United States [ 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 ], Switzerland [ 30 , 31 , 32 , 33 ], South Korea [ 34 , 35 ], Canada [ 36 , 37 ], Japan [ 38 , 39 ], Netherlands [ 40 , 41 ], India [ 42 ], United Kingdom [ 43 , 44 ], Germany [ 45 ], Brazil [ 46 ], Italy [ 47 ], Poland [ 48 ], North American collaboration [ 49 ], European collaboration, [ 50 ] and an international collaborative [ 51 ].…”

Chirurgische Therapie maligner Lungen- und Brustwandtumoren bei Kindern

“…Eine umfangreiche Analyse der Langzeitergebnisse nach Therapie von Brustwandsarkomen im Kindesalter wurde von Harris et al 2020 publiziert [3]. Die retrospektive Studie umfasst 175 Kinder mit Brustwandsarkomen, die in 17 Zentren über einen Zeitraum von 10 Jahren behandelt wurden.…”

“…Der überwiegende Anteil der bösartigen Brustwandtumoren ist mesenchymalen Ursprungs, wobei in 45 % Weichteilsarkome vorliegen und in 55 % der Tumor auf einem kartilaginären oder knöchernen Ursprung basiert. Das Ewing-Sarkom ist die häufigste Tumorentität gefolgt von Osteosarkomen und Rhabdomyosarkomen [3] Im Anschluss an die Operation erfolgt in Abhängigkeit des mikroskopischen Resektionsstatus sowie des Ansprechens des Tumors auf die Induktionschemotherapie eine zusätzliche Radiotherapie sowie die risikoadaptierte Konsolidierungschemotherapie. Die anschließenden Nachsorgeuntersuchungen sollten durch den Kinderonkologen gemäß aktueller Leitlinien erfolgen (AWMF-Register-Nr.…”