Diagnostic Utility of a Modified Reticulin Algorithm in Pediatric Adrenocortical Neoplasms.

Pediatric adrenocortical neoplasms (ACNs) are extremely rare tumors in contrast to their adult counterparts. Distinguishing benign from malignant is challenging based on pure morphologic grounds. Previously, 2 scoring systems were proposed in pediatric ACN, including the Wieneke criteria (WC) and its modified version (modified WC [mWC]). In adults, the reticulin algorithm (RA) has proven inexpensive, reliable, predictive, and reproducible; however, it has been validated only recently in children in a limited number of cases. This study aims to assess the RA utility compared with other scoring systems in a series of 92 pediatric ACNs. All cases were individually scored, and mitotic rate cutoffs were recorded. Reticulin alterations were classified as quantitative and qualitative. Outcome data were available in 59/92. The median age was 5 years (0.1 to 18 y) with an M:F of 0.6. Clinical presentation included virilization (39%), Cushing syndrome (21%), other symptoms (4%), and asymptomatic (36%). The reticulin framework was intact in 27% and altered in 73% of cases, showing qualitative (22%), quantitative (73%), and both (5%) alterations. In patients with favorable outcomes, 59% showed either intact reticulin or qualitative alteration compared with the unfavorable outcome group, where 90% showed quantitative alterations. All scoring systems WC ( P < 0.0001), mWC ( P = 0.0003), and the adult/pediatric RA ( P < 0.0001) had predictive value. The RA is comparable to WC and mWC, easier to apply, and is the most sensitive histopathological approach to identifying aggressive behavior in pediatric ACN. Its integration into the WC might be helpful in ACN of uncertain malignant potential and deserves further investigation.

Standardizing the surgical management of benign ovarian tumors in children and adolescents: A best practice Delphi consensus statement.

AIM: No widely agreed consensus protocols exist for the management of benign ovarian tumors (BOT) in children. This presents a substantial risk for suboptimal management. We aimed to generate multispecialty consensus guidance to standardize surgical management and provide a clear follow-up protocol for children with BOTs. METHODS: Prospective two-round confidential e-Delphi consensus survey distributed among multispecialty expert panel; concluded by two semistructured videoconferences. MAIN RESULTS: Consensus was generated on these core outcome sets: preoperative/intraoperative management; follow-up; adolescent gynecology referral. (1) Children with BOTs should receive the same management as other patients with potentially neoplastic lesions: Preoperative discussion at a pediatric oncology multidisciplinary meeting to risk stratify tumors, and management by health professionals with expertise in ovarian-sparing surgery and laparoscopy. (2) Ovarian-sparing surgery for BOTs should be performed wherever possible to maximize fertility preservation. (3) Ovarian masses detected during emergency laparoscopy/laparotomy should be left in situ wherever feasible and investigated appropriately (imaging/tumor markers) before resection. (4) Follow-up should be undertaken for all patients after BOT resection. Patients should be offered referral to adolescent gynecology to discuss fertility implications. CONCLUSION: This best practice Delphi consensus statement emphasizes the importance of managing children with BOTs through a well-defined oncological MDT strategy, in order to optimize risk stratification and allow fertility preservation by ovarian-sparing surgery wherever possible.

Hyperthermic intraperitoneal chemotherapy (HIPEC) as another treatment modality for desmoplastic round cell tumour patients: first paediatric experience from UK.

We present the first young paediatric patient with desmoplastic small round cell tumour (DSRCT) treated in UK with hyperthermic intraperitoneal chemotherapy (HIPEC). A 7-year-old girl was diagnosed with abdominal DSRCT with peritoneal and liver metastases. After six cycles of chemotherapy she obtained a partial response, including almost complete resolution of the two liver metastases. It was decided to pursue cytoreductive surgery (CRS) combined with HIPEC, a procedure commonly performed in adults, but seldom in a child. The surgery was macroscopically complete and the HIPEC uncomplicated. She continued treatment without delays, including whole abdomino-pelvic radiotherapy and maintenance chemotherapy (cyclophosphamide/vinorelbine for 12 months). She is currently in complete remission 4 months after end of treatment and 26 months after diagnosis. HIPEC was made possible by successful collaboration between multiple teams. CRS-HIPEC proved to be safe and feasible and could be offered to other children with diagnoses of peritoneal malignancies across the UK.

Aneurysm of Upper Limb Arteries in Children: Report of Five Cases.

BACKGROUND: Arterial aneurysm in children is rare. AIM: To present the description of case series of successful surgical treatment of upper limb aneurysms in children. The case series included 2 boys and 3 girls, with median age 3.3 years. One of them was a newborn with a true brachial artery aneurysm. Aneurysms were in the brachial (n = 3) and radial (n = 2) arteries. Two patients had idiopathic aneurysms. Two cases were associated with connective tissue dysplasia syndrome. One patient had a history of trauma. In 4 of 5 cases, there was a true aneurysm and in one a pseudoaneurysm. Diagnosis was carried out in all cases by using ultrasound, with arteriography in one case. All 5 children were operated on. Resection of the aneurysm and restoration of arterial patency was performed in 4 of 5 cases (ligation of the radial artery near the aneurysm in 1 case, aneurysm resection with end-to-end anastomosis in 1 case, resection with PTFE graft implantation in 1 case, and resection with implantation of an autovenous graft in 2 cases. Complications. Dysfunction and thrombosis of the PTFE graft required reoperation using an autovenous graft. CONCLUSION: Despite the rarity of the disease, timely and adequate surgical treatment of aneurysms of the arteries of the upper extremities in children is possible successfully in a specialized hospital.

Evaluation of needle biopsy as a potential risk factor for local recurrence of Wilms tumour in the SIOP WT 2001 trial.

RATIONALE: The impact of biopsying Wilms tumour (WT) at diagnosis on assigning the tumour stage and recommended treatment remains controversial. To address this important question, we analysed the potential association of all types of biopsy with local recurrence in patients treated in the SIOP WT 2001 trial, where needle biopsy was permitted without 'upstaging' the tumour to stage III. Only open biopsy required treatment as stage III. METHODS: Among 2971 patients with unilateral WT (stages I-IV), 420 relapsed (139 local). Risk factors for recurrence were analysed by Cox proportional hazard methods. RESULTS: Biopsy was performed in 969 of 2971 (33%) patients (64% cutting needle, 30% fine needle aspiration [FNA] and 6% open biopsy). Biopsied patients were older, with larger tumours and a greater proportion with high-risk histology. In multivariate analysis that included all factors associated with local recurrence in univariate analysis, only high-risk histology (hazard ratio [HR] = 2.32; 95% confidence interval [CI]: 1.58-3.42, p=<0.0001), age≥2 years (HR = 2.24; 95% CI: 1.22-4.09, p = 0.01) and preoperative tumour volume (HR = 1.07 per 100 ml; 95% CI: 1.02-1.12, p = 0.01) were significant. The HR for the association of local recurrence and event-free and overall survival with biopsy was not significant (HR = 1.4; 95% CI: 0.9-2.17, p = 0.13; HR = 1.1; 95% CI: 0.85-1.42, p = 0.46 and HR = 1.13; 95% CI: 0.79-1.62, p = 0.51, respectively). These results were not materially different whether FNA or open biopsy were included in the biopsy group or not. CONCLUSIONS: This post hoc analysis provides some reassurance that needle biopsy is not an independent adverse factor for either local recurrence or survival after adjustment for all relevant risk factors. Needle biopsy should not be an automatic criterion to 'upstage' WT.

Anterior mediastinal masses - A multidisciplinary pathway for safe diagnostic procedures.

OBJECTIVE: The objective of this study was to report our multidisciplinary diagnostic approach for patients with anterior mediastinal masses (AMM). METHODS: A retrospective review of patients with AMM at a tertiary pediatric surgical oncology centre (January 2011-December 2016) was performed. We analyzed data on clinical presentation, mode of tissue diagnosis, anesthetic techniques, and complications. RESULTS: Of the 44 patients admitted with AMM (median age 11 years, 27 males and 17 females), 22 had respiratory symptoms. Imaging revealed tracheobronchial compression in 26 children. Twenty patients had a lymph node biopsy. Ten patients had image-guided core biopsy of the mediastinal mass, and 2 had mediastinoscopic biopsy of a paratracheal lymph node. One patient with likely recurrence of a relapsed metastatic ethmoid carcinoma did not have a biopsy. The diagnosis was made from alternative tissues, such as pleural fluid in 4 and peripheral blood in 7 patients. Twenty-five anesthetics were assessed, as 14 patients required no or only local anesthesia, and 5 had unavailable anesthetic notes. Eighteen of 25 patients were anesthetized maintaining spontaneous breathing, mostly by means of ketamine sedation. There were no major anesthetic complications. CONCLUSION: Safe tissue diagnosis of anterior mediastinal masses can be obtained by a personalized multidisciplinary approach. Use of alternative tissues, local anesthesia, and ketamine sedation help minimize the need for general anesthesia, muscle paralysis, and controlled ventilation. LEVEL OF EVIDENCE: IV (Case Series with no Comparison Group).

Systematic review and meta-analysis of appendiceal carcinoid tumors in children.

OBJECTIVE: The study's aim was to review the literature regarding past and current practices in managing incidental appendiceal carcinoid tumors and need for more procedures. METHOD: A search of MEDLINE, Embase, CINAHL, and Cochrane databases of systematic reviews was undertaken of the English language literature. The mesh terms used were "carcinoid" or "neuroendocrine," "tumour" or "tumor," "appendix," "appendicectomy," or "appendectomy," and "child," "pediatric," or "paediatric." Of the 369 articles found, 37 met the inclusion criteria. Our hospital records and pathology database identified 11 patients with confirmed histological diagnosis of appendicular carcinoids from January 1996 to December 2016. Those cases were also included in this study. RESULTS: A total of 958 cases were identified from the literature and our own experience. There were 566 females and 343 males giving us a ratio of 1.65:1. The frequency was 0.3% of appendicectomies. There was a 28-fold increase in the risk of having a positive lymph node if the tumor size was >2 cm compared with the risk of having a positive lymph node if the tumor size was ≤2 cm. There was no recurrence or mortality for those with criteria for secondary surgery, who were observed after appendicectomy compared to those that had secondary surgery. Mean follow-up was 58.6 months (4.8 years) with a range of 0-396 months (33 years). CONCLUSION: Appendicectomy alone is an adequate treatment for an appendicular carcinoid in children irrespective of size, position, lymph node, or mesenteric involvement. Post-appendicectomy investigations were found to be not helpful in this study.

Pneumoperitoneum in the Setting of Pneumatosis Intestinalis in Children: Is Surgery Always Indicated?

Aim Pneumatosis intestinalis (PI) is a condition in which multiple gas-filled cysts form within the wall of the gastrointestinal tract in either the subserosa or submucosa. The presence of pneumoperitoneum in the presence of PI can present a therapeutic dilemma. The aim of our study was to review our experience and management of this condition. Methods A single-center retrospective study of consecutive children (> 1 year) presenting with a pneumoperitoneum and evidence of PI (2009-2015). Demographics, case notes, microbiology, and imaging were reviewed. Results Seven patients were identified (four males; age range 5-14 years). Four children had global developmental delay and were percutaneous endoscopic gastrostomy or jejunostomy fed, one was immunocompromised (acute lymphoblastic leukemia). The others had encephalitis and eosinophilic gastroenteritis. One patient proceeded to an exploratory laparotomy; no perforation was identified, pneumatosis of the colon was observed, and a loop ileostomy was formed. The remaining six were managed conservatively and made nil by mouth with intravenous antibiotics commenced. Five of the six had a computed tomography (CT) scan which revealed PI and free air with no other worrying signs. One died from nongastrointestinal causes, while the remaining five had feeds reintroduced uneventfully. Conclusion Free air in the setting of PI may represent rupture of the gas cysts and not always transmural perforation. Surgery may not always be indicated and conservative management may suffice. A CT scan can be useful for excluding other intra-abdominal pathological findings and continued clinical assessment is essential.

Nephron sparing surgery (NSS) for unilateral wilms tumor (UWT): the SIOP 2001 experience.

BACKGROUND: Total nephrectomy (TN) remains the standard treatment of unilateral Wilms tumors (uWT). The SIOP WT-2001 protocol allowed Nephron Sparing Surgery (NSS) for polar or peripherally non-infiltrating tumors. AIM: Inventory of the current SIOP NSS-experience. PROCEDURES: 2,800 patients with a unilateral, localized or metastatic and an unequivocal surgical technique recorded were included. All had neo-adjuvant chemotherapy and delayed surgery. In 91 (3%) NSS was performed and in 2709 TN. Data was retrieved from the SIOP WT 2001 database. RESULTS: NSS group contained 65% stage I tumours and the TN group 48%. Tumor volume (at diagnosis and surgery) was significantly smaller in the NSS group. Within stage III, after NSS, 7/12 (58%) had positive margins (M +), 5 with tumor negative lymph nodes (LN-). After TN, 355/712 (55%) had M + , 182 were LN-. Treatment of M+ in the NSS group resulted in two conversions to TN (one combined with radiotherapy), three patients had radiotherapy only and in two patients local therapy, if given, was not recorded. After NSS, four recurrences occurred. For localized disease the 5-year overall (OS) and event free survival (EFS) in NSS group was 100 and 94.8 (95% CI:89.9-99.9), respectively, while OS and EFS in the TN group were 94.4 (95% CI: 93.2-95.5, log-rank test P = 0.06) and 86.5 (95% CI:85.0-88.1, log-rank test P = 0.06), respectively. CONCLUSIONS: NSS was only performed in 3% of patients with uWT. Despite excellent survival with few relapses, the gain of nephrons needs to be weighed against the risk to induce stage III with intensified therapy.

UK intussusception audit: a national survey of practice and audit of reduction rates.

AIM: To define current UK reduction practice and the reductions rates achieved. MATERIALS AND METHODS: Electronic surveys were sent to radiologists at 26 UK centres. This assessed methods of reduction, equipment, personnel, and protocol usage. Standardized audit proforma were also sent to evaluate all reductions performed in 2011. RESULTS: Twenty-two of 26 centres (85%) replied. All used air enema under fluoroscopic guidance. Equipment was not standardized but could be broadly categorized into hand-pumped air-supply systems (seven centres) and pressurized air systems (15 centres). Seventeen centres followed a protocol based on British Society of Paediatric Radiologists (BSPR) guidelines. In 21 of the 22 centres a consultant paediatric radiologist led reductions and only 12 centres reported a surgeon being present. Three hundred and ten cases were reported across 22 centres. Cases per centre ranged from 0-31 (median 14). Reduction rates varied from 38-90% (median 71%). The overall perforation rate was 2.5%. Caseload did not significantly correlate with reduction rate, and there was no significant difference between the two types of equipment used. Median reduction rates were 15% higher in centres with a surgeon present at reduction (p < 0.05). CONCLUSION: Intussusception care in the UK lacks standardization of equipment and personnel involved. National reduction rates are lower than in current international literature. Improved standardization may lead to an improvement in reduction rates and a surgeon should always be present at reduction.

Tension gastrothorax in children: introducing a management algorithm.

Tension gastrothorax is a life-threatening condition and presents dramatically. It develops when the stomach herniates through a diaphragmatic defect into the thorax and distends with gas and/or fluid causing mediastinal shift. It is commonly misdiagnosed as tension pneumothorax which results in significant morbidity and mortality. In this article we report our experience of three cases with a spectrum of aetiology and a comprehensive literature review of tension gastrothorax in children. We propose an algorithm for the management of tension gastrothorax.

Air enema reduction of intussusception: a registrar-led, protocol-driven service is safe and effective.

PURPOSE: In the UK, air enema reduction of intussusception is predominantly performed by consultant radiologists. At our institution, it is usually performed by a team involving radiology and surgical registrars. The purpose of this study was to assess who performs air enemas in the UK and the efficacy and safety of our registrar-led service. METHODS: A telephone survey of paediatric surgical centres in the UK was performed asking who performs air enema in these institutions. Following this, a retrospective review of all air enemas at our institution between January 2005 and 2011 was performed. Cases were identified from radiology databases and reviewed for grade of radiologist, perforation and outcome. RESULTS: At all 25 centres, consultant radiologists performed air enemas. At our institution 145 enemas were performed in 6 years. 141 were analysed (54 girls, 87 boys). Median age was 9 months (range 3-107 months). 82 % were performed by the registrar-only team. Over 6 years the registrar-led reduction rate was 77.5 % and in the last 3 years 84 %. The perforation rate was 0.9 %. CONCLUSION: A paediatric surgical registrar-led service for air enema reduction can be safe and effective ensuring a team approach is adopted, equipment is efficient and a strict protocol is applied.

Cervical approach to the thoracic inlet in paediatric patients with broncho-pulmonary foregut malformations.

Lesions of the thoracic inlet present a significant challenge to the surgeon due to the difficulty of access and proximity to important neurovascular structures within the region. We describe two cases of benign disease of the thoracic inlet in children, one bronchogenic cyst and an esophageal duplication, and report the cervical approach used to manage them. Both lesions extended from the neck through the thoracic inlet, but demonstrate how benign lesions in this area can be delivered up into a cervical incision, negating the need for the more invasive modified thoracotomies. A cervical approach can be safely and successfully used to approach benign pathology, such as bronchogenic cysts and oesophageal duplications of the thoracic inlet. Careful multidisciplinary planning is required for such procedures.

The impact of adult major trauma centre status on paediatric trauma activity.

OBJECTIVES: The London Trauma Network was launched in April 2010 in order to centralise trauma care in the capital city. The consultation and resourcing of the four new major trauma centres (MTC) was adult focused. The objective of this study was to assess the impact that adult MTC status has on paediatric trauma workload. METHODS: A retrospective review of paediatric major trauma calls was performed between 1 April 2009 to 31 January 2010, before MTC status, and the same time period in 2010/11 when St George's Healthcare Trust was a designated adult MTC. The following variables were assessed; number of trauma calls, admissions to hospital, radiological services usage, inpatient stay, mechanism of injury and injury severity score (ISS)--calculated from abbreviated injury score. RESULTS: There was a 200% increase in trauma calls between the two time periods and a 191% increase in admission to hospital. The usage of radiology increased 221% for CT and 161% for plain radiology. Mean inpatient stay decreased by 0.2 days. Mechanisms of injury were similar. Despite becoming a MTC the relative volume of major injuries (ISS>15) decreased between the two time periods by 1% with a relative increase in minor trauma (ISS<5) from 63% to 72%. These results may suggest at present paediatric trauma patients are being over triaged. CONCLUSIONS: Major adult trauma centre status has a significant effect on paediatric trauma workload and hence resources. When reconfiguration of trauma services are being considered it is essential to take into account the impact on paediatric services alongside those of the adult population.

Treatment of high risk Sertoli-Leydig cell tumors of the ovary using a gonadotropin releasing hormone (GnRH) analog.

Sertoli-Leydig cell tumors are rare ovarian neoplasms. We report two unusual cases with bilateral SLCTs suggesting evidence of genetic predisposition and at high risk of recurrence. To reduce this risk, we exploited the use of GnRH analog to lower gondadotropin and potentially directly inhibit the tumors through expressed GnRH receptors. We used it as maintenance antitumor therapy for 2 years after completion of chemotherapy, to cover the period of risk for recurrence. Both patients remain in complete remission at >2 years after completing leuprorelin therapy. Of note, both patients carry DICER1 mutations, frequently found in pleuropulmonary blastoma syndrome.

Gastroschisis with Hirschsprung's disease: a therapeutic dilemma.

The authors report a case of a neonate with gastroschisis, which, after repair, was further complicated by the diagnosis of Hirschsprung's disease. The authors discuss the diagnostic and management dilemmas posed by the coexistence of these two conditions for the clinician.