Quantitative diffusion-weighted MRI response assessment in rhabdomyosarcoma: an international retrospective study on behalf of the European paediatric Soft tissue sarcoma Study Group Imaging Committee.

OBJECTIVE: To investigate the feasibility of diffusion-weighted magnetic resonance imaging (DW-MRI) as a predictive imaging marker after neoadjuvant chemotherapy in patients with rhabdomyosarcoma. MATERIAL AND METHODS: We performed a multicenter retrospective study including pediatric, adolescent and young adult patients with rhabdomyosarcoma, Intergroup Rhabdomyosarcoma Study group III/IV, treated according to the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS2005 or MTS2008 studies. DW-MRI was performed according to institutional protocols. We performed two-dimensional single-slice tumor delineation. Areas of necrosis or hemorrhage were delineated to be excluded in the primary analysis. Mean, median and 5th and 95th apparent diffusion coefficient (ADC) were extracted. RESULTS: Of 134 included patients, 82 had measurable tumor at diagnosis and response and DW-MRI scans of adequate quality and were included in the analysis. Technical heterogeneity in scan acquisition protocols and scanners was observed. Mean ADC at diagnosis was 1.1 (95% confidence interval [CI]: 1.1-1.2) (all ADC expressed in * 10-3 mm2/s), versus 1.6 (1.5-1.6) at response assessment. The 5th percentile ADC was 0.8 (0.7-0.9) at diagnosis and 1.1 (1.0-1.2) at response. Absolute change in mean ADC after neoadjuvant chemotherapy was 0.4 (0.3-0.5). Exploratory analyses for association between ADC and clinical parameters showed a significant difference in mean ADC at diagnosis for alveolar versus embryonal histology. Landmark analysis at nine weeks after the date of diagnosis showed no significant association (hazard ratio 1.3 [0.6-3.2]) between the mean ADC change and event-free survival. CONCLUSION: A significant change in the 5th percentile and the mean ADC after chemotherapy was observed. Strong heterogeneity was identified in DW-MRI acquisition protocols between centers and in individual patients.

Abdominal US in Pediatric Inflammatory Multisystem Syndrome Associated with SARS-CoV-2 (PIMS-TS).

Background Children with pediatric inflammatory syndrome temporally associated with SARS-CoV-2 (PIMS-TS), also known as multisystem inflammatory syndrome in children, present with abdominal pain among other nonspecific symptoms. Although initial imaging features of PIMS-TS have been reported, the duration of sonographic features remains unknown. Purpose To describe the abdominal US features of PIMS-TS at initial presentation and follow-up. Materials and Methods A retrospective review of children and young adults presenting with clinical features suspicious for PIMS-TS between April 2020 and June 2021 was carried out. US features were documented and reviewed at initial presentation and follow-up. Descriptive statistics were used and interobserver variability was calculated. Results Of 140 children and young adults presenting with suspected PIMS-TS, 120 had confirmed PIMS-TS (median age, 9 years; interquartile range, 7-12 years; 65 male patients) and 102 underwent abdominal US at presentation. PIMS-TS was present as a single abnormality in 109 of the 120 patients (91%) and abdominal symptoms were present in 104 of the 109 (95%). US examinations were abnormal in 86 of 102 patients (84%), with ascites being the most common abnormality in 65 (64%; 95% CI: 54, 73). Bowel wall thickening was present at US in 14 of the 102 patients (14%; 95% CI: 7, 20) and mesenteric inflammation was present in 16 (16%; 95% CI: 9, 23); all of these patients presented with abdominal symptoms. Among the patients with bowel wall thickening, the distal and terminal ileum were most involved (eight of 14 patients, 57%). Abdominal symptoms decreased to seven of 56 patients (13%) in those followed up at 6 months. Thirty-eight patients underwent follow-up US, and the presence of bowel inflammation had decreased to three of 27 patients (11%; 95% CI: -1, 23) in those followed up for less than 2 months and 0 of 17 (0%) in those followed up for more than 2 months. Conclusion Of 102 patients with pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 who underwent US at presentation, 14 (14%) had abdominal US findings of bowel inflammation and 16 (16%) had mesenteric edema. All US abnormalities resolved after 2 months. © RSNA, 2022 Online supplemental material is available for this article. See also the editorial by van Rijn and Pajkrt in this issue.

Importance of Magnetic Resonance Imaging With Diffusion-weighted Imaging in Guiding Biopsy of Nodular Ganglioneuroblastoma: A Case Report.

BACKGROUND: Nodular ganglioneuroblastoma is a rare peripheral neuroblastic tumor of variable prognosis. Accurate diagnosis, staging, and risk categorization can be particularly challenging in patients with nodular ganglioneuroblastoma due to the inherent heterogeneity of these lesions. CASE PRESENTATION: We illustrate the use of diffusion-weighted magnetic resonance imaging to identify tumor nodules and guide tumor biopsy in an almost 5-year-old boy with a large abdominal tumor. CONCLUSIONS: Diffusion-weighted magnetic resonance imaging was successful in detecting and guiding biopsy of a poorly differentiated neuroblastoma nodule within the context of a well-differentiated ganglioneuroma, allowing the diagnosis and characterization of a ganglioneuroblastoma nodular, thus influencing the child's prognosis and treatment.

European guideline for imaging in paediatric and adolescent rhabdomyosarcoma - joint statement by the European Paediatric Soft Tissue Sarcoma Study Group, the Cooperative Weichteilsarkom Studiengruppe and the Oncology Task Force of the European Society of Paediatric Radiology.

Appropriate imaging is essential in the treatment of children and adolescents with rhabdomyosarcoma. For adequate stratification and optimal individualised local treatment utilising surgery and radiotherapy, high-quality imaging is crucial. The paediatric radiologist, therefore, is an essential member of the multi-disciplinary team providing clinical care and research. This manuscript presents the European rhabdomyosarcoma imaging guideline, based on the recently developed guideline of the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) Imaging Committee. This guideline was developed in collaboration between the EpSSG Imaging Committee, the Cooperative Weichteilsarkom Studiengruppe (CWS) Imaging Group, and the Oncology Task Force of the European Society of Paediatric Radiology (ESPR). MRI is recommended, at diagnosis and follow-up, for the evaluation of the primary tumour and its relationship to surrounding tissues, including assessment of neurovascular structures and loco-regional lymphadenopathy. Chest CT along with [F-18]2-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET)/CT or PET/MRI are recommended for the detection and evaluation of loco-regional and distant metastatic disease. Guidance on the estimation of treatment response, optimal long-term follow-up, technical imaging settings and standardised reporting are described. This European imaging guideline outlines the recommendations for imaging in children and adolescents with rhabdomyosarcoma, with the aim to harmonise imaging and to advance patient care.

Is surveillance imaging in pediatric patients treated for localized rhabdomyosarcoma useful? The European experience.

BACKGROUND: After the completion of therapy, patients with localized rhabdomyosarcoma (RMS) are subjected to intensive radiological tumor surveillance. However, the clinical benefit of this surveillance is unclear. This study retrospectively analyzed the value of off-therapy surveillance by comparing the survival of patients in whom relapse was detected by routine imaging (the imaging group) and patients in whom relapse was first suspected by symptoms (the symptom group). METHODS: This study included patients with relapsed RMS after the completion of therapy for localized RMS who were treated in large pediatric oncology hospitals in France, the United Kingdom, Italy, and the Netherlands and who were enrolled in the International Society of Paediatric Oncology Malignant Mesenchymal Tumor 95 (1995-2004) study, the Italian Paediatric Soft Tissue Sarcoma Committee Rhabdomyosarcoma 96 (1996-2004) study, or the European Paediatric Soft Tissue Sarcoma Study Group Rhabdomyosarcoma 2005 (2005-2013) study. The survival times after relapse were compared with a log-rank test between patients in the imaging group and patients in the symptom group. RESULTS: In total, 199 patients with relapsed RMS were included: 78 patients (39.2%) in the imaging group and 121 patients (60.8%) in the symptom group. The median follow-up time after relapse was 7.4 years (interquartile range, 3.9-11.5 years) for survivors (n = 86); the 3-year postrelapse survival rate was 50% (95% confidence interval [CI], 38%-61%) for the imaging group and 46% (95% CI, 37%-55%) for the symptom group (P = .7). CONCLUSIONS: Although systematic routine imaging is the standard of care after RMS therapy, the majority of relapses were detected as a result of clinical symptoms. This study found no survival advantage for patients whose relapse was detected before the emergence of clinical symptoms. These results show that the value of off-therapy surveillance is controversial, particularly because repeated imaging may also entail potential harm.

Phase II study of temozolomide and topotecan (TOTEM) in children with relapsed or refractory extracranial and central nervous system tumors including medulloblastoma with post hoc Bayesian analysis: A European ITCC study.

AIM: To assess objective response after two cycles of temozolomide and topotecan (TOTEM) in children with refractory or relapsed miscellaneous extracranial solid and central nervous system (CNS) tumors, including medulloblastoma and primitive neuroectodermal tumors (PNET). PROCEDURE: Multicenter, nonrandomized, phase 2 basket trial including children with solid tumors, completed by a one-stage design confirmatory cohort for medulloblastoma, and an exploratory cohort for PNET. Main eligibility criteria were refractory/relapsed measurable disease and no more than two prior treatment lines. Temozolomide was administered orally at 150 mg/m2 /day followed by topotecan at 0.75 mg/m2 /day intravenously for five consecutive days every 28 days. Tumor response was assessed every two cycles according to WHO criteria and reviewed independently. RESULTS: Thirty-two patients were enrolled and treated in the miscellaneous solid tumor and 33 in the CNS strata; 20 patients with medulloblastoma and six with PNET were included in the expansion cohorts. The median age at inclusion was 10.0 years (range, 0.9-20.9). In the basket cohorts, confirmed complete and partial responses were observed in one glioma, four medulloblastoma, and one PNET, leading to the extension. The overall objective response rate (ORR) in medulloblastoma was 28% (95% CI, 12.7-47.2) with 1/29 complete and 7/29 partial responses, those for PNET 10% (95% CI, 0.3-44.5). Post hoc Bayesian analysis estimates that the true ORR in medulloblastoma is probably between 20% and 30% and below 20% in PNET. The most common treatment-related toxicities of the combination therapy were hematologic. CONCLUSIONS: Temozolomide-topotecan results in significant ORR in children with recurrent and refractory medulloblastoma with a favorable toxicity profile.

Preoperative computed tomography scanning for abdominal neuroblastomas is superior to magnetic resonance imaging for safe surgical planning.

BACKGROUND: Cross-sectional imaging is required to assess disease prior to surgery for neuroblastoma (NBL), and both magnetic resonance imaging (MRI) and computed tomography (CT) scan are considered acceptable. We had concerns that MRI was underestimating disease extent, so from early 2016 we have systematically used MRI and CT before all abdominal NBL resections. The aim of this retrospective study was to establish which imaging modality is more accurate in determining disease extent, particularly after chemotherapy. METHODS: Abdominal MRI and CT scans for all children with abdominal NBL referred for surgery from January 2016 to February 2018 were retrospectively reviewed to evaluate the extent of disease and the presence of imaging-defined risk factors (IDRFs). RESULTS: Thirty-one patients were eligible for consideration of surgery post disease reassessment with MRI/MIBG. Twenty-four of 31 children were included. CT was performed a median of 15 (range, 1-47) days after MRI. MRI underestimated IDRFs compared with CT in 13 of 24 patients (54%). Seventeen of 24 patients underwent surgery, and operative findings had 100% correlation with CT imaging. Notably, there were fundamental changes in management post CT in 6 of 24 patients (25%). CT did not underestimate disease compared with MRI in any patient. CONCLUSION: MRI underestimated the extent of the disease in half of our patients considered for NBL resection. This may be due in part to tumor fibrosis, calcification, and chemotherapy. Preoperative CT scan is the best imaging modality to identify all IDRFs after chemotherapy to ensure safe surgery.

Outcome and prognostic factors in pediatric malignant peripheral nerve sheath tumors: An analysis of the European Pediatric Soft Tissue Sarcoma Group (EpSSG) NRSTS-2005 prospective study.

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNST) are rare tumors of childhood. The role of standard chemotherapy in unresectable MPNST is still unclear. We report the outcome and prognostic factors in the EpSSG risk-adapted prospective study for localized pediatric MPNST. METHODS: Patients were stratified into four treatment groups defined by surgical resection, tumor size, and tumor grade (G): (a) surgery-only group-resected tumors G1; (b) adjuvant radiotherapy group-R0/R1, G2 tumors; (c) adjuvant chemotherapy group-R0/R1, G3 tumors; and (d) neoadjuvant chemotherapy group-R2 resected tumors and/or nodal involvement. Chemotherapy consisted of four courses of ifosfamide-doxorubicin and two courses of ifosfamide concomitant with radiotherapy (50.4-54 Gy). RESULTS: Overall, the study included 51 patients. The 5-year event-free survival (EFS) and overall survival (OS) were 52.9% (95% confidence interval, 38.1-65.8) and 62.1% (46.7-74.3), respectively. The 5-year EFS was 92% (56.6-98.9) for treatment group 1 (N = 13), 33% (0.9-77.4) for treatment group 2 (N = 4), 29% (4.1-61.2) for treatment group 3 (N = 7), and 42% (23.1-60.1) for treatment group 4 (N = 27). Response rate to chemotherapy (partial response + complete response) in patients with measurable disease was 46%. The presence of neurofibromatosis type 1 (NF1; 51% of patients) was an independent poor prognostic factor for OS and EFS. CONCLUSION: The outcome for patients with resectable MPNST was excellent. Standard ifosfamide-doxorubicin for unresectable MPNST rendered the best reported outcome. Children with NF1 disease seem to have worse prognosis.

The new international neuroblastoma response criteria.

Over recent years significant advances in histopathology and functional imaging techniques for the diagnosis and restaging of children with neuroblastoma have led to better quantification of disease and assessment of disease response, allowing for better treatment stratification. In this review we summarise recent changes to the International Neuroblastoma Response Criteria including the use of RECIST (Response Evaluation Criteria in Solid Tumours) guidance for measurable soft-tissue disease, replacement of technetium-99 m-methylene diphosphonate (MDP) bone scans with metaiodobenzylguanidine (MIBG) scan or [F-18]2-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET)/CT scanning, and a new category of minimal residual bone marrow disease.

The clinical and radiologic features of paediatric rhabdomyosarcoma.

Rhabdomyosarcoma is the most common soft-tissue sarcoma in children. The most common sites are head and neck, genitourinary tract and extremities. In this review we outline the clinical and radiologic features of paediatric rhabdomyosarcoma, as well as imaging considerations and imaging of relapse.

Prognostic relevance of early radiologic response to induction chemotherapy in pediatric rhabdomyosarcoma: A report from the International Society of Pediatric Oncology Malignant Mesenchymal Tumor 95 study.

BACKGROUND: Early response to induction chemotherapy is used in current European guidelines to evaluate the efficacy of chemotherapy and subsequently to adapt treatment in pediatric patients with rhabdomyosarcoma (RMS). However, existing literature on the prognostic value of early radiologic response on survival is contradictory; here the prognostic value is analyzed with data from the International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumor 95 (MMT-95) study. METHODS: This study examined 432 Intergroup Rhabdomyosarcoma Study Grouping III (macroscopic residue) patients enrolled in the SIOP MMT-95 study with a response assessment after 3 courses of chemotherapy (a 2-dimensional assessment). Patients with progressive disease (PD) after 3 courses of chemotherapy were excluded (n = 7). Failure-free survival (FFS) and overall survival (OS), calculated with the Kaplan-Meier method, were compared for 3 groups (complete response [CR]/partial response [PR], objective response [OR], and no response [NR]). The prognostic impact of early response was assessed through the calculation of Cox proportional hazards. RESULTS: After 3 courses of chemotherapy, 85.2% of the patients had CR/PR, 8.6% had OR, and 6.3% had NR. For all patients, the 5-year FFS and OS rates were 60% (95% confidence interval [CI], 56%-65%) and 74% (95% CI, 70%-78%), respectively. However, a Cox proportional hazards regression analysis revealed no significant difference in FFS or OS between the response groups. The adjusted hazard ratios for an OR and NR were 1.09 (95% CI, 0.63-1.88) and 0.81 (95% CI, 0.39-1.67), respectively, for FFS and 0.91 (95% CI, 0.47-1.76) and 1.27 (95% CI, 0.61-2.64), respectively, for OS. CONCLUSIONS: No evidence was found for the idea that early radiologic response to chemotherapy is prognostic for survival for patients with RMS. Treatment adaptation based on early response (except for patients with PD) should, therefore, no longer be incorporated into future studies. Cancer 2018;124:1016-24. © 2017 American Cancer Society.

Cancer incidence among children and young adults who have undergone x-ray guided cardiac catheterization procedures.

Children and young adults with heart disease appear to be at increased risk of developing cancer, although the reasons for this are unclear. A cohort of 11,270 individuals, who underwent cardiac catheterizations while aged ≤ 22 years in the UK, was established from hospital records. Radiation doses from cardiac catheterizations and CT scans were estimated. The cohort was matched with the NHS Central Register and NHS Transplant Registry to determine cancer incidence and transplantation status. Standardized incidence ratios (SIR) with associated confidence intervals (CI) were calculated. The excess relative risk (ERR) of lymphohaematopoietic  neoplasia was also calculated using Poisson regression. The SIR was raised for all malignancies (2.32, 95% CI 1.65, 3.17), lymphoma (8.34, 95% CI 5.22, 12.61) and leukaemia (2.11, 95% CI 0.82, 4.42). After censoring transplant recipients, post-transplant, the SIR was reduced to 0.90 (95% CI 0.49, 1.49) for all malignancies. All lymphomas developed post-transplant. The SIR for all malignancies developing 5 years from the first cardiac catheterization (2 years for leukaemia/lymphoma) remained raised (3.01, 95% CI 2.09, 4.19) but was again reduced after censoring transplant recipients (0.98, 95% CI 0.48, 1.77). The ERR per mGy bone marrow dose for lymphohaematopoietic neoplasia was reduced from 0.541 (95% CI 0.104, 1.807) to 0.018 (95% CI - 0.002, 0.096) where transplantation status was accounted for as a time-dependent background risk factor. In conclusion, transplantation appears to be a large contributor to elevated cancer rates in this patient group. This is likely to be mainly due to associated immunosuppression, however, radiation exposure may also be a contributing factor.

Projected cancer risks potentially related to past, current, and future practices in paediatric CT in the United Kingdom, 1990-2020.

BACKGROUND: To project risks of developing cancer and the number of cases potentially induced by past, current, and future computed tomography (CT) scans performed in the United Kingdom in individuals aged <20 years. METHODS: Organ doses were estimated from surveys of individual scan parameters and CT protocols used in the United Kingdom. Frequencies of scans were estimated from the NHS Diagnostic Imaging Dataset. Excess lifetime risks (ELRs) of radiation-related cancer were calculated as cumulative lifetime risks, accounting for survival probabilities, using the RadRAT risk assessment tool. RESULTS: In 2000-2008, ELRs ranged from 0.3 to 1 per 1000 head scans and 1 to 5 per 1000 non-head scans. ELRs per scan were reduced by 50-70% in 2000-2008 compared with 1990-1995, subsequent to dose reduction over time. The 130 750 scans performed in 2015 in the United Kingdom were projected to induce 64 (90% uncertainty interval (UI): 38-113) future cancers. Current practices would lead to about 300 (90% UI: 230-680) future cancers induced by scans performed in 2016-2020. CONCLUSIONS: Absolute excess risks from single exposures would be low compared with background risks, but even small increases in annual CT rates over the next years would substantially increase the number of potential subsequent cancers.

Relationship between paediatric CT scans and subsequent risk of leukaemia and brain tumours: assessment of the impact of underlying conditions.

BACKGROUND: We previously reported evidence of a dose-response relationship between ionising-radiation exposure from paediatric computed tomography (CT) scans and the risk of leukaemia and brain tumours in a large UK cohort. Underlying unreported conditions could have introduced bias into these findings. METHODS: We collected and reviewed additional clinical information from radiology information systems (RIS) databases, underlying cause of death and pathology reports. We conducted sensitivity analyses excluding participants with cancer-predisposing conditions or previous unreported cancers and compared the dose-response analyses with our original results. RESULTS: We obtained information from the RIS and death certificates for about 40% of the cohort (n∼180 000) and found cancer-predisposing conditions in 4 out of 74 leukaemia/myelodysplastic syndrome (MDS) cases and 13 out of 135 brain tumour cases. As these conditions were unrelated to CT exposure, exclusion of these participants did not alter the dose-response relationships. We found evidence of previous unreported cancers in 2 leukaemia/MDS cases, 7 brain tumour cases and 232 in non-cases. These previous cancers were related to increased number of CTs. Exclusion of these cancers reduced the excess relative risk per mGy by 15% from 0.036 to 0.033 for leukaemia/MDS (P-trend=0.02) and by 30% from 0.023 to 0.016 (P-trend<0.0001) for brain tumours. When we included pathology reports we had additional clinical information for 90% of the cases. Additional exclusions from these reports further reduced the risk estimates, but this sensitivity analysis may have underestimated risks as reports were only available for cases. CONCLUSIONS: Although there was evidence of some bias in our original risk estimates, re-analysis of the cohort with additional clinical data still showed an increased cancer risk after low-dose radiation exposure from CT scans in young patients.

Prevalence of Gallstones Compared in Children With Different Intravenous Lipids.

OBJECTIVE: The aetiology of biliary liver disease in children with intestinal failure treated with long-term parenteral nutrition (PN) is multifactorial. Risks include the lipid component of PN. The aim of the study was to compare prevalence and outcome of gallstones with different types of intravenous lipids. METHODS: Liver and biliary tract imaging and relevant clinical details were reviewed in 71 patients (37, 52% boys) treated with PN for >3 months. Types of lipid infused were compared with regard to hepatobiliary abnormalities. RESULTS: In total 369 abdominal ultrasounds were performed in 71 patients of age between 3 months and 17 years. Underlying diagnoses were short bowel syndrome in 20 (28%), small intestinal enteropathy in 34 (48%), and motility disorder in 17 (24%). A total of 67 (94%) children had 362/369 scans on lipid-containing PN. Of the total, 15 (21%) patients had gallstones, 8 (11%) had sludge, and both were detected in 7 (10%) children. The gallstones/sludge resolved in 7 patients (10%) and persisted in 10 (13%). In 6 patients, sludge progressed to form discrete gallstones, and in 9 children, gallstones led to biliary duct dilatation. Four (6%) patients underwent cholecystectomy. Fewer children had abnormalities with the newer mixed lipid emulsion (P = 0.005). There was a higher prevalence of sludge (P = 0.01) on pure soya lipid. Predictors for sludge were young age at PN (P = 0.001), lack of enteral feed (P < 0.001), and motility disorder with stoma (P = 0.002). CONCLUSIONS: Hepatobiliary pathology is common in children on PN. The use of mixed lipid was associated with less biliary complications and should be the first choice of treatment in children.

Pediatric oncology surveillance imaging: two recommendations. Abandon CT scanning, and randomize to imaging or solely clinical follow-up.

Radiologic assessments in children with cancer provide information crucial to patient management at diagnosis and during follow-up. Many studies have now been published, however, questioning the usefulness of off-therapy surveillance imaging. There is growing concern regarding the hazards from diagnostic irradiation to young patients, most notably from CT scanning. In this paper we advocate abandoning repeated CT surveillance in young patients with a previously treated solid malignancy not arising in the central nervous system. In addition, randomized studies of imaging surveillance versus no imaging surveillance strategies are needed to determine whether earlier detection of recurrence results in improved survival.

Duodenal haematoma following endoscopy as a marker of coagulopathy.

BACKGROUND: Intramural duodenal haematomas (IDHs) are a rare complication of endoscopic biopsy but can cause significant morbidity and mortality, including duodenal obstruction, hospitalization and needing intravenous feeding. They are extremely uncommon in those with normal haematology. OBJECTIVE: To describe the occurrence of IDHs following endoscopic biopsy in our institution. MATERIALS AND METHODS: We identified three patients who developed a post biopsy IDH during an 18-month period (2010-2012) in a retrospective search of our hospital pathology and imaging databases. RESULTS: All three children had complex medical problems and presented with gastrointestinal symptoms including severe abdominal pain, reflux, poor feeding and abnormal gut transit time. All underwent normal upper GI endoscopy with duodenal biopsy. Following endoscopy, they presented with intermittent GI obstruction with severe abdominal pain, distension and bilious vomiting or symptoms of pancreatitis, had imaging features of IDH and were managed conservatively making a full recovery. Initial haematology including platelet counts were normal, but two children were subsequently found to have platelet dysfunction and the third to have an unclassified coagulopathy. CONCLUSION: IDHs may be the presenting factor in children with unsuspected bleeding problems. We present these findings to raise awareness of the imaging features and clinical impact of these cases because better understanding of these risk factors may help to avoid these complications in children in the future.

Diagnostic radiology in paediatric palliative care.

Palliative care is an expanding specialty within paediatrics, which has attracted little attention in the paediatric radiological literature. Paediatric patients under a palliative care team will have numerous radiological tests which we traditionally categorise under organ systems rather than under the umbrella of palliative medicine. The prevalence of children with life-limiting illness is significant. It has been estimated to be one per thousand, and this may be an underestimate. In this review, we will focus on our experience at one institution, where radiology has proven to be an invaluable partner to palliative care. We will discuss examples of conditions commonly referred to our palliative care team and delineate the crucial role of diagnostic radiology in determining treatment options.

Yolk sac tumour: a rare cause of raised serum alpha-foetoprotein in a young child with a large liver mass.

BACKGROUND: Primary yolk sac tumour of the liver is extremely rare, and when it occurs in a young child it can be confused with hepatoblastoma. OBJECTIVE: To present a retrospective case series of primary hepatic yolk sac tumour from two institutions to highlight the imaging features. MATERIALS AND METHODS: We conducted a retrospective review of pathological and imaging findings in biopsy-proven pure primary yolk sac tumour of the liver. RESULTS: A total of three children (15 months to 6 years) presented between 2006 and 2012 with primary hepatic yolk sac tumour. Two children presented with acute abdomen after minor trauma caused by tumour rupture and haemoperitoneum. We identified five additional paediatric cases of pure hepatic yolk sac tumour with descriptions of imaging findings in the literature. In seven of these eight children, central necrosis was seen on cross-sectional imaging. CONCLUSION: Primary yolk sac tumour should be considered as an alternative diagnosis to hepatoblastoma in a young child with a large liver mass and significantly raised serum alpha-foetoprotein, but in view of the nonspecific imaging features histological examination is essential for diagnosis. Although there are no specific imaging findings of primary yolk sac tumour, presence of cystic or necrotic areas and a tendency for tumour rupture are suggestive features.