Final Pr. ID: Paper #: 147
Despite significant advances in delivering dose-intensive and myeloablative therapy with hematopoietic stem cell support, the survival for patients presenting with metastatic neuroblastoma remains poor, with a 3 year event free survival (EFS) of about 60%. Modern treatment protocols are based on risk stratification which incorporates age of diagnosis, tumor stage, tumor histology, and molecular and cytogenetics including MYCN amplification. 18F-FDG PET/CT can play a role in disease staging and follow up. The purpose of this study was to report FDG PET findings in a cohort of children with neuroblastoma and assess for predictive associations with MYCN amplification status. Read More
Final Pr. ID: Poster #: EDU-085
With research pushing ever onward, it is often difficult to keep pace with the dynamic landscape of pediatric abdominal tumors and their classification systems. However, it is imperative that we, as radiologists, remain vigilant of these changes, as our initial and follow-up imaging assessments often have the potential to drive clinical intervention in widely differing directions.
In this educational poster, we will review the most up-to-date risk stratification and staging criteria for neuroblastoma, hepatoblastoma, and Wilms tumor in order to:
1. Educate about the most recent criteria for categorizing pediatric abdominal tumors such as neuroblastoma, hepatoblastoma, and Wilms tumor.
2. Provide imaging examples of these pediatric abdominal tumors and describe how the above-mentioned criteria might change radiology reports and patient management.
3. Encourage accurate risk stratification of these tumors so that radiologists are better equipped to assist in directing appropriate patient care. Read More
Final Pr. ID: Poster #: EDU-086
Congenital Central Hypoventilation Syndrome (CCHS) is a rare disorder that can cause respiratory arrest during sleep. It is sometimes referred to as "Ondine's Curse" in reference to a fictional character who had to remember to breathe based on a spell cast by a jilted lover. The number of cases has been reported to be near 1,000. The purpose of this educational exhibit is to describe CCHS and emphasize its implications for pediatric radiology. Read More
Final Pr. ID: Poster #: SCI-006
The purpose of this case report is to alert the reader to a rare differential diagnosis for infra- as well as intradiaphragmatic lung sequestration. A 38-year-old G2P0010 pregnant patient was referred for fetal magnetic resonance imaging (MRI) due to a 14.2 x 20.2 x 18 mm left paraspinal hyperechogenic mass with no internal vascularity and no convincing systemic arterial feeding vessel concerning for neuroblastoma as seen by ultrasound (US) performed at 34 weeks. Fetal MRI performed the same week showed a homogeneous T2-hyperintense left paraspinal mass in close apposition to and associated with a small area of loss of continuity in the diaphragm. This has been previously reported by postnatal computerized tomography as the "split diaphragm" sign in a case of intradiaphragmatic lung sequestration (Meier AH, Eggli KD, Cillei RE. Intradiaphragmatic extralobar sequestration: a rare pulmonary anomaly. Pediatr Surg 200;44:e27-29). Thus, the differential diagnosis provided at the time was congenital intradiaphragmatic sequestration and neuroblastoma. The fetus delivered via uncomplicated spontaneous vaginal delivery at term. Postnatal US performed at the age of 15 days showed a left hyperechogenic mass extending from the left lower chest to the ipsilateral retroperitoneum through a small defect in the diaphragm, favored to represent an extrapulmonary lung sequestration. The left adrenal gland was normal. A follow-up CT performed at 7 months of age showed a 2.6 cm left paraspinal mass with no systemic arterial blood supply to support the diagnosis of sequestration. The differential diagnosis at the time included neurogenic tumor or a myofibroma arising from the diaphragm. Follow-up CT at 13 months of age showed similar findings. The patient underwent uneventful laparoscopic removal of the mass with a final pathological diagnosis of congenital adrenal rest. Congenital adrenal rest presenting as a diaphragmatic mass is rare and as been reported once in an adult patient with an adenoma in heterotopic adrenal tissue located in the left diaphragm, diganosed because of mass effect in the gastric fundus during an uper gastrointestinal series (Keirns MM. Two unusual tumors of the diaphragm. Radiology 1952; 52:542-547). We hope this report raises awareness of this entity as a potential differential diagnosis for prenatal masses seen in close relationship with the adrenal gland and/or diaphragm. Read More
Final Pr. ID: Poster #: CR-009
Extramedullary hematopoiesis (EH) is defined as hematopoiesis occurring in organs outside of the bone marrow. It occurs in diverse conditions, including fetal development, normal immune responses, and pathological circumstances. These sites of extramedullary hematopoiesis may present as masses mimicking malignancy or produce symptoms due to pressure effects. In the setting of an existing malignancy they may appear as metastatic deposits signifying progression of disease. It is essential to confirm this due to its prognostic and treatment implications.
We report a 2-year-old little girl who presented initially with an acute history of ataxia, nystagmus, tremor, mydriasis and bruises on her left forehead. A solid left suprarenal mass was detected and a diagnosis of Stage 4 Neuroblastoma and Opsoclonus-Myoclonus syndrome was established. Subsequently she was on treatment which included chemotherapy, IVIG and stem cell transplant. On an MRI of the abdomen done a year later, a single lesion was detected in the right lobe of the liver. On subsequent short term follow up, innumerable scattered lesions were seen in the hepatic parenchyma and were thought to represent metastases. A liver biopsy showed that these hepatic lesions represented sites of extramedullary hematopoiesis.
Extramedullary hematopoiesis has been uncommonly seen in the cranium and sacrum in the setting of Neuroblastoma. We believe this is a unique presentation with extramedullary hematopoiesis presenting as solid liver masses masquerading as metastases in a known case of Neuroblastoma. Read More
Final Pr. ID: Poster #: EDU-096
Neuroblastoma is recognized as having a broad spectrum of clinical behavior in children diagnosed with the disease. Some tumors exhibit aggressive characteristics and portend a poor prognosis, while others that appear aggressive spontaneously regress. Accurately identifying high risk neuroblastoma is important in determining which patients will benefit most from intense chemotherapy, which unfortunately carries a risk of significant adverse effects later in life. Historically this has been difficult, as the classification schemes vary in different parts of the world, limiting the ability to pool data and improve prognostication. In recent years, efforts among experts around the globe have led to a consensus on the most evidenced based approach to staging. The aim of this educational exhibit is to describe the new standardized language for radiology reports, which will contribute to accurate staging and improve treatment for patients with neuroblastoma. Additionally, key imaging features highlighting image defined risk factors will be presented. Read More
Final Pr. ID: Poster #: SCI-016
Neuroblastoma (NB) is the most common malignancy in neonate and infancy, and, furthermore, growing use of prenatal US has led to increased detection of congenital NB. This study aims to evaluate imaging assessment and clinical features of congenital NB with a special focus on cystic NB. Read More
Final Pr. ID: Poster #: EDU-098
Neuroblastoma is the most common extracranial solid malignancy in children. It can have a variety of clinical outcomes, ranging from spontaneous resolution without therapy to fatal outcomes resistant to maximal therapy.
Historically, neuroblastoma has been staged using the International Neuroblastoma Staging System (INSS). While this staging system has been used in clinical trials since its introduction in 1989, its reliance on surgical staging is problematic. Surgical resection can vary between surgeons and between tumors and occurs at an interval from diagnosis. This method complicates the process of standardizing therapy. Additionally, some patients have a disease that spontaneously regresses and does not require surgical management and thus cannot be staged.
To combat these problems, the International Neuroblastoma Risk Group (INRG) created a new staging system for use in clinical trials in 2009. This staging system relies on preoperative imaging for up-front staging. This helps standardize neuroblastoma staging and helps to guide a more standard approach to management. The INRG staging system is comprised of twenty image-defined risk factors (IDRF), across multiple organ systems, which help predict surgical outcomes and can be combined with clinical data to provide up-front risk stratification.
Even though the INRG staging system has been in use since 2009, many pediatric radiologists remain unfamiliar with its definitions and application. Additionally, MR has now become an essential imaging tool for diagnosis, staging, and follow-up of patients with neuroblastoma. The purpose of this poster is to compare the INSS and INRG staging system, describe the limitations of each system, and illustrate the definitions and IDRFs that comprise the INRG staging system. Read More
Final Pr. ID: Poster #: EDU-105
Because of issues with the reliability of the previous staging system, the International Neuroblastoma Risk Group Staging System (INRG-SS) was created in 2009. Like with the introduction of any new tool, there has been some resistance to the embracing of the INRG-SS staging system by radiologists. This educational poster offers a practical approach to learning and utilizing the INRG system, emphasizing use of the descriptive terms which determine the presence or absence of imaging defined risk factors (IDRFs). Read More
Final Pr. ID: Poster #: SCI-064
In the past, neuroblastoma patients frequently had mIBG and diagnostic CT scans performed at separate times. This practice sometimes caused issues in correlating findings from the two imaging modalities. A retrospective review of our entire experience aimed to confirm the added value of optimized co-registered contrast-enhanced diagnostic CT to I-123 mIBG SPECT/CT protocol in children with neuroblastoma. An additional objective was to identify if SPECT/CT improved Curie score assignment vs planar imaging. Read More
Final Pr. ID: Poster #: CR-005
A previously healthy 12 year old female presented with left lower back pain and left thigh numbness. MRI showed a T1 isointense, heterogeneously T2 hyperintense left retroperitoneal mass with extension into the paraspinal muscles and intraspinal extension through the L1-L4 neural foramina. CT and contrast enhanced MRI (CE-MRI) of the lumbar spine and I-123 MIBG scintigraphy were performed. CT did not show any calcifications. MRI showed mildly heterogeneous, avid gadolinium enhancement. The mass was I-123 MIBG avid, without evidence of metastatic disease. Ultrasound guided biopsy yielded ganglion cells and no neuroblasts, suggestive of ganglioneuroma (GN). Partial excision of the retroperitoneal component yielded a 7 x 6 x 3 cm aggregate of tissue, and histopathology confirmed GN.
Follow up CE- MRI at 4, 10, and 16 months after surgery showed stable residual mass. CE-MRI at post op month 23 showed numerous T2 hyperintense enhancing osseous masses in the lumbar spine and sacrum. Residual mass remained stable. A fluoroscopically guided biopsy of a right sacral lesion yielded neuroblastoma. Review of the pathology from the original excision confirmed GN. Whole body I-123 MIBG scintigraphy showed the avid mass and confirmed skeletal metastases.
Ganglioneuromas (GN) are benign tumors of neural origin that exist on a spectrum with ganglioneuroblastoma (GNB) and the frankly malignant neuroblastoma (NB). CT, MR, and nuclear scintigraphy are unreliable for the differentiation of NB/GNB from GN. The most robust imaging feature to identify NB is the presence of distant metastases. Imaging findings that have been proposed as possible distinguishing features are the morphology of the calcifications, early versus delayed gadolinium enhancement, and ADC values. It is a well-known phenomenon that NB may regress into GNB or GN either spontaneously or following treatment with chemotherapy and/or radiation. To our knowledge, only one other case of GN de-differentiating into NB has been reported. Due to the extreme rarity of this case, alternatives must be considered. It is possible that the patient had a bi-phenotypic tumor and the NB component was not sampled initially or that she developed a NB extrinsic to the GN. These alternatives seem unlikely since NB is a rare tumor in 12 year olds, the residual tumor did not change on follow-up imaging, no new primary tumor was seen on recent MIBG, and two years passed prior to the development of metastasis. Read More
Final Pr. ID: Paper #: 155
Neuroblastoma (NB) is the most common non-cranial solid tumor in childhood. The MYCN oncogene plays a crucial role in tumorigenesis and angiogenesis in NB and is a strong indicator of poor prognosis. The tyrosine hydroxylase (TH)-MYCN transgenic mouse model of NB is extensively utilized; however, little is known about disease progression in this model. In this work, we use multi-modal imaging to study tumor progression and vascular architecture in TH-MYCN transgenic, allograft, and syngeneic mouse models of NB. Read More
Final Pr. ID: Poster #: EDU-081
The purpose of this exhibit is explore pitfalls in our experience with SPECT-CT Iodine-123 (I-123) MIBG imaging in patients with neuroblastoma. SPECT-CT can more specifically localize areas of uptake over planar imaging and mitigate false-positive results with correlative anatomic information. We will review cases of false-positive MIBG uptake in nonmalignant sites, cases of false-negative MIBG uptake in neuroblastoma/neural crest tumors, and cases of secondary tumors/malignancies occurring in the setting of known neuroblastoma, with variable uptake on MIBG. Read More
Final Pr. ID: Paper #: 156
Neuroblastoma is a clinically heterogeneous pediatric malignancy, varying in location, histopathologic appearance, and biologic characteristics. Genetics plays an important role in the prognosis. Amplification of the MYC family member, MYCN, is found in 25% of cases and correlates with high-risk disease and poor prognosis. However, genetic information can only be obtained via surgery or biopsy with concurrent morbidity and sampling variability associated with biopsy. The ability to detect MYCN amplification from routine pre-operative imaging can stratify neuroblastoma risk groups and affect clinical decision making. The purpose of this study was to predict the patient's MYCN status based on radiomics analysis of the magnetic resonance imaging (MRI) characteristics in patients with neuroblastoma. Read More
Final Pr. ID: Paper #: 157
Radiogenomics refers to the correlation of imaging and genomic data of tumors in cancer patients. This study attempts to make correlations between biomarkers in NGS Comprehensive Solid Tumor Panel reports and MRI imaging findings in neuroblastoma patients. Read More
Keywords: Neuroblastoma, Radiogenomics
Final Pr. ID: Poster #: EDU-069
In this exhibit, we will outline the revised International Neuroblastoma Response Criteria (INRC) used to assess treatment response in children with neuroblastoma, particularly high risk patients, in the context of clinical relevance to their treatment plan. Neuroblastoma is the most common extracranial solid malignancy in children, accounting for approximately 12% of deaths in children younger than 15 years of age affected with cancer. Up to 50% of children with neuroblastoma are found to have a high-risk phenotype with poor long-term survival and risk of therapy-related toxicity. Due to a lack of consensus regarding the definition of disease response, the development of more effective therapy treatment of high-risk disease has been hindered. The revised INRC consensus integrates modern, functional imaging techniques and quantitative assessment of bone marrow disease. It is anticipated that the revised INRC will enable a more precise assessment of treatment response that can be used to inform treatment decisions. This exhibit will delve into these modalities which are more sensitive and specific for Neuroblastoma detection. This exhibit will also demonstrate how the revised response criteria are used in the clinical setting in the Children’s Oncology Group clinical trials. Read More
Final Pr. ID: Poster #: SCI-021
The diagnosis of Wilms tumor and adrenal gland neuroblastoma can be challenging, even with imaging methods such as computed tomography and magnetic resonance imaging (MRI). This study aimed to show the utility of diffusion weighted MRI (DW-MRI) in the differentiation of neuroblastoma and Wilms tumor. Read More
Final Pr. ID: Paper #: 152
Neuroblastoma (NBL) is the most common extracranial solid malignancy of childhood. Biological features are known to affect disease severity. Status of amplification of the MYCN proto-oncogene is among several factors that affect the prognosis. CT texture analysis of tumor provides a step from qualitative to quantitative assessment, with the added bonus of use of routinely acquired images without the need for further tests. The aim of the study is to ascertain the ability to differentiate between MYCN-amplified and non MYCN-amplified neuroblastoma on pre-treatment CT images. The hypothesis is that lesion microenvironment and heterogeneity may differ significantly between MYCN-amplified and non MYCN-amplified tumor. Such differences may be exhibited in first-order texture analysis techniques that could detect more subtle differences in tumor heterogeneity by quantifying both pixel attenuation. This retrospective study objective is to explore the correlation between the first –order CT texture analysis (CTTA) and MYCN amplification status of neuroblastoma. Read More