Background and Aims

Younger age has been associated with better overall survival (OS) in Ewing sarcoma (ES), especially under the age of 10. Favorable survival in younger patients underlines the need for minimizing treatment burden and late sequalae. Our study aimed at describing clinical characteristics, treatment and outcome of ES patients aged 0-10.

Methods

In this retrospective multicenter study, all consecutive ES patients aged 0-10, treated in four sarcoma centers in the Netherlands (n=33) and one in Spain (n= 27) (1982-2008), with minimum follow-up of 10 years were included. OS, local recurrence free survival (LRFS) and distant metastasis free survival (DMFS) were calculated. Potential factors of influence on OS (risk and protective factors) were analyzed.

Results

Sixty patients with median follow-up 13.03 years were included. All patients were treated with chemotherapy and local treatment, being surgery in 30 (50%) patients, radiotherapy (RT) in 12 (20%) patients or surgery plus RT in 18 (30%) patients (12 pre- and 6 post-operative). Limb salvage was achieved in 93% of patients. The 10-OS, -LRFS and -DMFS is 81% (95%CI 71-91%), 89% (95%CI 85-93%) and 81% (95%CI 71-91%), respectively. Six patients developed LR, of which 2 with subsequent DM; all had axial ES (pelvis, spine or chest wall) and these patients all died. Ten patients developed DM; 8 died due to progressive disease and two are currently in remission, both with pulmonary metastasis only. Negative or wide resection margin was significantly associated with better OS. Age <6, tumor volume <200ml, absence of metastatic disease and treatment after 2000 showed trends towards better OS. 2 patients developed secondary malignancy, both had chemotherapy combined with definitive RT for local treatment.

Conclusions

Today, overall survival of these youngest patients with ES was very good. Limb salvage surgery was achieved in >90% of patients. Wide resection margin was the only factor significantly associated with better survival.

Background and Aims

Introduction: Surgical resection of rhabdomyosarcoma (RMS) of abdomen and pelvis is associated with many challenges and RO resection is unachievable. We aim to identify the associated surgical risk factors.

Methods

Methods: A retrospective review of patients with RMS of the abdomen and pelvis from 2010-2022 was conducted. Tumors originated from bladder/prostate, genitalia and biliary tract were excluded. Clinical charts, imaging, surgery and pathology reports were analyzed.

Results

Results: Among 16 patients identified, mean age 5.2 (1.4-8.9) years with 7 boys, the tumors were located in the abdomen (n=4), pelvis (n=4) and abdomino-pelvis (n=8). Two in the latter group originated from urachus. Except for a patient with alveolar RMS, the rest had embryonal RMS. Distant metastases were present in 5 patients. All patients had large tumors at presentation and 5 ruptured before chemotherapy commenced. All received pre-operative chemotherapy but 7 showed limited or no response. At surgery, we identified surgical risk factors associated with challenges to include vascular encasement of aorta (n=4), superior mesenteric artery and celiac axis (n=3), iliac arteries (n=8), involvement of sacral/obturator nerves (n=5), hydronephrosis (n=7), ureteric encasement (n=7) with renal atrophy (n=2), and sarcomatosis peritonei (n=2). Seven tumors with peri-iliac extension were associated with ureteric encasement. Respective application of perivascular tumor excision techniques, intraoperative neuromonitoring, ureteric stenting and ureterolysis, and cytoreductive surgical technique optimized outcome. All patients received radiotherapy for local control. In mean follow-up of 4.8 (1-11.8) years, 3 died of disease, 1 died of second malignancy, 1 alive with bone relapse.

Conclusions

Conclusion: RMS in abdomen and pelvis typically present with large tumors with risk of rupture. Preoperative chemotherapy improves the feasibility of R1 resection. The identified surgical risk factors highlighted the need for new precautionary techniques for better outcome.

Background and Aims

The management of Ewing sarcoma in children has evolved over the last 30 years with surgical role and approach following the collaborative oncology group guidelines. This review of the literature aimed to assess how these surgical guidelines have been applied in the modern era.

Methods

A systematic review was conducted in accordance with PRISMA guidelines across four major literature databases. Data regarding overall survival, rate of recurrence, role of surgery, adjuvant therapy role was extracted.

Results

17 single centre observational studies and 8 retrospective reviews of multicentre trials met criteria for final analysis. There were 1028 patients identified, with a male predominance in their adolescent years. 5-year overall survival ranged from 35% to 89%. A review in 2003 established the role for neo-adjuvant chemotherapy before surgery with improved negative margins(77% vs 50%) and reduced post-op radiotherapy requirement(48% vs 71%). There was high variation in the degree of resection of surrounding tissue to obtain free margins so the collaborative oncology group guidelines for resecting a normal rib above and below and 2-3cm margins along the rib were not really followed. Some databases found similar 5 yr survival whether partial or total rib resection was performed. If negative margins were achieved then further radiotherapy was not shown to improve survival further. However if microscopic positive margins were present then additional radiotherapy could improve survival in some studies similar to microscopic free margin resections.

Conclusions

The review suggests that surgery should be included as part of multimodality treatment for most patients, with the current collaborative oncology group guidelines for surgical margins probably being too aggressive which may limit surgery being applied for some patients. Macroscopic free margins are an absolute, but microscopic positive margins can be compensated for by radiotherapy, and neo-adjuvant chemotherapy is an absolute requirement.

Background and Aims

Pulmonary metastectomy (PM) for resectable oligometastatic Osteosarcoma (OS) is recommended as the standard of care in pediatric and adult cancer therapy protocols. Recent data from the PulMiCC trial demonstrated no survival benefit from PM in Colorectal Cancer. Paradigm shifts focusing on the quality of life costs of childhood cancer treatment mandates re-evaluation of the effects of repeated ‘re-do’ thoracotomies. The aim of this study was therefore to critically re-examine the evidence for a survival benefit of PM for OS in the pediatric population.

Methods

A comprehensive systematic review was undertaken according to PRISMA methodology guidelines. Medline and Embase were searched for ALL studies detailing pediatric OS patients (<18 years) undergoing PM with a comparison group that did not receive PM. Studies where data was not distinguishable from included adult patients, those without a ‘no PM comparison group’, and those with < 4 patients were excluded.

Results

The initial search yielded 944 abstracts from which 47 full papers were selected for screening. Twelve studies met the full inclusion criteria dating from 1984 – 2017, detailing a total of 530 patients. All studies were retrospective, the majority being medium to large case series and no report directly compared PM vs no PM in pediatric patients as its main study objectives. Overall a positive survival benefit was declared in favour of PM. Patients not undergoing PM were usually those with unresectable disease and/or considered to have a poor prognosis.

Conclusions

These study findings cannot clearly demonstrate strong evidence ‘for or against’ a survival benefit of PM for OS in pediatric patients. All studies detail outdated treatments protocols and are not designed to address the questions directly. The ostensible survival benefit(s) of PM is likely due to selection bias of ‘favourable cases’. A randomised controlled trial incorporating modern bespoke cancer therapy protocols is crucial to address the question.

Background and Aims

Thyroidectomy is rarely performed in pediatric patients. The aim of the present study was to analyze our experience of thyroidectomy in children and identify factors associated with postoperative complications.

Methods

Between January 2009 and April 2022, 27 patients (21 female, 6 male) with thyroid neoplasms were operated on at our institution. The diagnostic workup consisted of ultrasound, thyroid scintigraphy, and/or magnetic resonance imaging (MRI). All patients were treated according to the guidelines of the Society of Pediatric Hematology and Oncology (GPOH). The indication for surgery was established by a multidisciplinary tumor board. Surgery was performed using a reduced Kocher incision. A retrospective review of patient’s records was carried out.

Results

The median age at operation was 14 years (6-16). The histologies were: 13 papillary carcinoma, 9 follicular adenoma, 3 struma nodosa, 2 cystic lesion. A hemithyroidectomy was performed in 11 cases, 16 patients underwent complete thyroidectomy (including neck-dissection in 4 cases). In 6/16 children completion thyroidectomy was performed as second-step after previous hemithyroidectomy until histological confirmation of papillary carcinoma. Intraoperative recurrent laryngeal nerve monitoring was used in all cases. The resection status was R0 in 24 cases, R1 in 2 cases, and R2 in 1 case (infiltration of recurrent laryngeal nerve). Median length of hospital stay was 4 days (3-16). Postoperative complications occurred in 3 patients (1 transient vocal cord palsy; 1 hematoma which necessitated hemostasis; 1 hypocalcemia). The overall survival was 100% after a median follow-up of 48 months (4-147).

Conclusions

Surgical management of pediatric thyroid neoplasms can be very complex. Treatment within interdisciplinary trial protocols is essential. The use of intraoperative recurrent laryngeal nerve monitoring is essential in reducing the incidence of postoperative vocal cord palsy. With sufficient surgical expertise, an excellent functional and oncological outcome can be obtained.

Background and Aims

Dynamic risk stratification(DRS) is re risk stratification of DTC based on initial treatment response into excellent response, biochemical residual disease, structural disease or indeterminate disease. This has proven to be useful in management of adult patients however it lacks evidence in pediatric population. An audit was done to evaluate application of DRS in pediatric patients treated at our centre

Methods

Retrospective single centre audit of pediatric DTC treated with total thyroidectomy followed by radioactive iodine (RAI) during 2008-2018 were included. Patients were risk stratified immediately post surgery as per ATA 2015 criteria and were restratified as per the DRS at completion of therapy. A follow up was done for outcomes.

Results

63 patients were identified and risk stratified into 13 low risk, 14 intermediate and 36 high risk as per the ATA 2015 criteria.

On DRS all low and intermediate risk patients (including those who received < 100mCi) achieved excellent response and continued to be disease free at mean follow up of 7 years

26/36 high risk showed excellent response of which only 1 had a local relapse. 3/36 showed biochemical residual disease of which 1 had local relapsed and 6/36 showed structural residual disease, of which 3 patient progressed. Rest of the patients on follow up continued to be disease free. The mean follow up was 5.6 years for high risk group.

Mean doses of RAI for achieving excellent response in high risk patients with only nodal disease and with lung metastases were 145mCi and 750mCi respectively.

Conclusions

Excellent response noted on DRS even in high risk paediatric DTC and continued disease free (DF) on follow up, favouring its use in management.

This re stratification will prompt lesser diagnostic and therapeutic interventions in children.

Patients with nodal disease can be treated with lower doses of radioactive iodine with excellent response.

Background and Aims

To report the epidemiological aspects, surgical challenges, and outcomes of children with benign retroperitoneal germ cell tumours (GCT) presenting to our centre.

Methods

A retrospective study was conducted, and all patients with retroperitoneal GCTs, managed from January 1998 to January 2022 were enrolled. Patients with increased alpha-fetoprotein (AFP) or with malignant histology were excluded. The prospectively collected data of the included patients were analysed for patient demographics, presentation, radiologic and histologic findings, surgical procedures, and the challenges faced in tumour excision, postoperative complications, and recurrence.

Results

Of the total 130 cases of extracranial GCT that presented to us, 26 (20%) had retroperitoneal tumours and formed the study group. The median age was 10.5 months (10 days – 14.8 years) at presentation. Of the total cohort, 21/26 (80%) patients presented with a palpable abdominal mass; unusual presentations, such as polymenorrhagia (n=1) and hypertensive emergency (n=1), were also noted. Bilateral hydronephrosis was present in 3/21 (14%) patients. All the patients underwent operative intervention, with 25/26 (96%) undergoing complete tumour excision. Intraoperatively, 7/26 (27%) patients had the tumour in close relationship with the aorta or inferior vena cava (4/26), duodenum (2/26) and diaphragmatic infiltration (1/26). These patients required fine intraoperative dissection, and, in a few cases, additional procedures such as bowel resection (1/26), diaphragmatic repair (1/26) and redo resection (1/26) were also required. In 4/26 (15%) of the patients, immature teratoma was present on histopathology. The median duration of follow-up was 24 months (1 month – 168 months), and one patient had a significant complication (adhesive obstruction). There were no recurrences reported in the cohort.

Conclusions

Retroperitoneal germ cell tumours have an excellent outcome if completely excised. The current study highlights the need for adequate preoperative evaluation and intraoperative visualization of the vital structures to achieve complete excision in these patients.

Background and Aims

Sacrococcygeal teratoma (SCT) is a rare neoplasm affecting 1:35,000 newborns. Long-term impaired bladder/bowel function has been reported by mainly small observational series. This study therefore comprehensively analyses ALL published studies to define the true long-term functional sequelae.

Methods

Medline/Embase databases were searched according to PRISMA guidelines. Data were extracted following paper selection by study authors.

Results

Final analysis yielded 36 studies involving 1,116 patients (854 female; 77%). Individual data were available in 14 studies (222 patients). SCT diagnosis was established antenatally (20%), during the neonatal period (39%), infancy (20%), and after the first year of life (20%). Mean tumour size was 8.5cm (SD 4.6). According to the Altman classification, there were 298/845 (35%) type I, 252/845 (30%) type II, 133/845 (16%) type III, and 128/845 (15%) type IV tumours. Most neoplasms were benign (640/858; 75%), 77/858 (9%) immature, and 141/858 (16%) malignant. The commonest associated congenital anomalies were anorectal malformations 42/90 (47%), spinal dysraphism 19/90 (21%), and renal tract/urogenital disorders 10/90 (11%).

Abnormal bladder function was reported in 7/39 (18%) Altman type I, 23/61 (37.7%) type II, 11/34 (32.4%) type III, and 15/25 (60%) type IV cases (p=0.007). Such findings were also more commonly recorded in immature/malignant vs benign tumours and in those patients requiring re-operation(s); p=0.002 and p=0.01, respectively. Soiling was documented in 19% and constipation in 26% patients with no apparent significant associations with individual tumour characteristics. Abnormal gait was more common in those with Altman type I-II 2/14 lesions (14.3%) vs type III-IV tumours (5/6, 83.3%), p=0.003. Additionally, 55 patients reported unsatisfactory cosmesis, and surgical scar site revision was performed in 13/55 (24%).

Conclusions

Higher Altman stage, unfavourable histology, and re-operation are associated with poorer functional outcome(s) most notably bladder dysfunction. Multidisciplinary management from 1^st diagnosis of SCT is crucial to optimise outcomes across surgical specialities.

Background and Aims

Paediatric chest wall tumours are rare. Chest wall resection and reconstruction is challenging but essential to the management. Challenges include unique characteristics related to growth in children and the variability of the post-surgical chest wall defects in size and anatomic location. Described techniques (using non-absorbable prosthetics) have limitations. We aim to review long-term outcomes when using bioabsorbable prosthetic materials.

Methods

This retrospective series includes pre-pubertal children that have undergone chest wall reconstruction at our institution. Restitution of musculoskeletal integrity and soft tissue coverage was achieved using a combination of Poly-L-lactide struts, a porcine dermal collagen patch and a combination of muscle/skin flaps where needed, allowing native tissue ingrowth and complete implant resorption. Complications, functional and aesthetic results were obtained with review of photography and axial imaging.

Results

Seven patients were included: 5 males and 2 females, median age 4 years (range 0-10), median follow-up 8 years (range 0- 9). Pathology included 4 Ewing sarcomas, 1 osteochondroma, 1 chondromesenchymal hamartoma and 1 high grade spindle-cell sarcoma. 6 patients remain disease free whilst 1 patient is deceased suffering metastatic Ewing relapse (with local control persisting). Complications included a seroma at 2 months in one patient, a sterile collection at 8 months in another (both drained and resolved) and early post-operative wound infection in one patient treated with antibiotics. The reconstruction provided good initial thoracic integrity enabling early extubation. Long term cosmetic and functional results are satisfactory with good chest wall shape and function. There were no post-operative restrictions on daily activities, including sport. Pitfalls include the low-level PET avidity of the prosthesis (when considering relapse surveillance) and the presence of mild scoliosis.

Conclusions

Poly-L-lactide struts and Permacol® offer bioabsorbability and a physiologically advantageous method for reconstruction of the chest wall allowing adaptation and growth with good functional and aesthetic long-term outcomes in the paediatric population.

Background and Aims

There are several surgical strategies for patients with synchronous pulmonary metastases undergoing resection. This includes simultaneous bilateral or staged thoracotomies, median or transverse sternotomy. There are currently only a few paediatric reports of simultaneous bilateral posterolateral thoracotomies. The aim of the study was to evaluate the safety and feasibility of simultaneous bilateral muscle-sparing posterolateral thoracotomies (BT) in paediatric patients compared with unilateral thoracotomy (UT).

Methods

We conducted a retrospective review of all patients who underwent thoracotomy for pulmonary malignancy at Children’s Hospital at Westmead between January 2010 and March 2021. Medical records were reviewed and patients’ demographics, types of pain management, underlying diseases, operative time, days with intercostal catheter (ICC), length of stay after surgery (LOS), and perioperative complications were analyzed.

Results

34 patients (24 males, 10 females, average age 11 year) underwent 44 thoracotomies during the study period. All thoracotomies were muscle-sparing posterolateral thoracotomy. Four patients underwent bilateral simultaneous thoracotomies. The most common underlying malignancy was Osteosarcoma (n= 15, 38%), followed by NRSTS (n= 6, 15%) and Ewing sarcoma (n=5, 13%). Epidural analgesia was used for pain management in 31 patients (78%) including all four patients who underwent bilateral thoracotomies. When we compared outcomes for patients undergoing UT versus BT, median length of hospital stay was 5 days in UT and 8 days in BT (p<0.01), and days with intrathoracic drain was 2 days in UT versus 3.5 days in BT (p=0.06). Postoperative complications occurred in four patients (pneumonia, prolonged air leakage, urinary retention, and Clostridium difficile colitis) in UT versus one patient (prolonged air leakage) in BT (p=0.4).

Conclusions

Simultaneous bilateral muscle-sparing thoracotomies seem safe and feasible with acceptable postoperative outcomes.

Background and Aims

Thoracic air leak syndrome (TALS) is a complication related to chronic pulmonary graft-versus-host disease (pGvHD) that affects approximately 0.83% to 3.08% patients after allogenic hematopoietic stem cell transplant. Such complication is defined as the occurrence of any form of air leak in the thorax, including spontaneous pneumomediastinum or pneumopericardium, subcutaneous emphysema, interstitial emphysema and pneumothorax and has a negative impact on post-transplant survival.

The aim of the present study is to describe a single-center experience in the surgical management of recurrent TALS in adolescents and young adults and its outcome.

Methods

The clinical notes of patients with previous allogenic hematopoietic stem cell transplant who underwent surgical procedures for recurrent TALS from January 2016 until March 2021 were retrospectively reviewed. Clinical data, number of episodes of thoracic air leak, surgical procedures and outcome were analyzed.

Results

In the examined period, four patients, aged 16 to 25 years, underwent surgical procedures for TALS, including thoracostomy tube placement, thoracoscopic pleurodesis and thoracotomy. All the patients had been diagnosed with pGvHD before the onset of TALS, with a mean time lapse of 276 days (range 42 – 513). These patients experienced on average 4.5 air leak episodes (range 3 – 6). All the patients experienced at least two episodes before surgery.

One patient underwent emergency tube thoracostomy only, three patients underwent thoracoscopic pleurodesis and two patients underwent thoracotomy.

After surgery, patients were free from air leak symptoms for a mean time of 176 days (range 25 – 477). Pulmonary function progressively deteriorated, and all the patients eventually died because of respiratory failure after a mean time of 483 days (range 127 – 1045) after the first episode of air leak.

Conclusions

Surgery provides temporary relief to symptoms related to TALS. When TALS develops, pulmonary function progressively worsens toward respiratory failure and death.