Lee Sean,  Shah Amisha

Final Pr. ID: Poster #: CR-036

Rubinstein-Taybi Syndrome (RTS) and infantile myofibromatosis (IM) are both rare genetic disorders. RTS is marked by craniofacial dysmorphism, short stature, and skeletal abnormalities, while IM usually appears as one or more soft tissue nodules, which can affect bones and visceral organs. We present the rare case of a male newborn diagnosed with both RTS and IM. At birth, the patient showed unusual facial features, small size, and polydactyly. The initial skeletal survey showed multiple lucent lesions in the metaphyseal long bones. A follow-up skeletal survey at four months showed growing lucent lesions, though they had developed benign features of a sclerotic rim and a narrow zone of transition. New bone lesions were seen in the flat bones and spine with vertebra plana deformity. Additionally, calcified soft tissue masses were found. Further characterization by MRI suggested marrow infiltration of the lesions. Due to the higher cancer risk in RTS patients, leukemia, lymphoma, or metastatic neuroblastoma were the main concerns, while Langerhans cell histiocytosis was considered less likely due to the metaphyseal pattern. A bone biopsy at two sites confirmed multicentric myofibroma, consistent with IM. Genetic testing found a PDGFRB mutation linked to IM. The patient’s mother tested positive for the same mutation and recalled skin involvement as an infant. The patient tested positive for a de novo CREBBP mutation linked to RTS. These findings suggested autosomal dominant inheritance of IM, while RTS arose sporadically. A whole-body MRI ruled out visceral involvement, a good prognostic factor for IM. The patient received chemotherapy and responded well until it was stopped due to adverse effects. At age 8, he had surgery for a tethered cord, a known complication of RTS. Shortly after, a new lucent lesion, likely a recurrent myofibroma in the right femoral neck, was incidentally found on an abdominal X-ray obtained for pain. The patient is currently under clinical and imaging surveillance. No reported case has yet shown the coexistence of RTS and IM, and there is no known link between the two. This case highlights the importance of considering IM and other rare conditions in the differential diagnosis of multiple lucent bone lesions. A coexisting syndrome can complicate the picture and delay diagnosis. Imaging, biopsy, and genetic testing were key in diagnosing and managing this case. Read More

Authors:  Lee Sean , Shah Amisha

Keywords:  Bone Tumor, Rubenstein-Taybi Syndrome, Infantile Myofibromatosis

Bowman Sean,  Trinh Austin,  Tuburan Smyrna

Final Pr. ID: Poster #: CR-070

A 4 day old female, born at term without complications and with reported proper prenatal care was referred to a tertiary care center after her parents were notified of an abnormal 17-OHP on newborn screening. Work-up for suspected congenital adrenal hyperplasia was initiated. An abdominal ultrasound showed a 2.8 x 3.6 x 3.7 cm heterogeneously hypoechoic right suprarenal mass with peripheral calcification and large feeding vessels. An MRI abdomen was then obtained, which showed a round circumscribed 3.6 x 3.5 x 3.4 cm T1 hypointense and T2 iso-hyperintense heterogeneously enhancing mass. The radiologist raised the suspicion for an adrenocortical tumor, with neuroblastoma or teratoma as less likely etiologies. An MIBG scan was then obtained, revealing absence of activity in the region of the known mass, further supporting the diagnosis of an adrenocortical neoplasm. This information, in addition to normal levels of VMA and HVA, reduced the likelihood that the mass was of neural crest origin. IR then performed an ultrasound guided biopsy of the right adrenal mass. Pathology reported features consistent with an adrenal cortical neoplasm and without features of neuroblastoma. Due to concern for potential aggressive behavior, pathology recommended complete surgical excision. There is a paucity of literature secondary to the extremely low incidence of congenital adrenal cortical neoplasms. Therefore, a multidisciplinary committee, which included pediatric surgery and radiology, was held. The consensus was that the mass would be best treated by, and is amenable to, surgical excision. At 28 days old, the patient underwent an open right adrenalectomy. Pathology confirmed an adrenal cortical neoplasm of uncertain malignant potential. The post-operative course was without complications. Per pediatric hematology-oncology recommendations, a nuclear medicine whole body bone scan was obtained and demonstrated no osteoblastic metastasis. The patient was discharged and will continue to follow up with hematology-oncology and endocrinology as an outpatient. As illustrated above, the early and correct identification of a potentially aggressive neoplasm by radiology accelerated the treatment course, allowing for potentially curative interventions. This case demonstrates the necessity for multidisciplinary management to include both diagnostic and interventional radiologists and to communicate an appropriate range of differential diagnoses, regardless of incidence. Read More

Authors:  Bowman Sean , Trinh Austin , Tuburan Smyrna

Keywords:  Adrenal, Tumor, adrenal cortical neoplasm

Sato T Shawn,  Sato Yutaka

Final Pr. ID: Poster #: EDU-084

The 2016 World Health Organization Classification of Tumors of the Central Nervous System has now incorporated molecular and genetic parameters in addition to histology to define many tumor entities. Significant restructuring has occurred for pediatric CNS tumors. For example, medulloblastomas are classified into four genetic subtypes. Other embryonal tumors such as embryonal tumor with multilayered rosettes (ETMR) and atypical teratoid/rhabdoid tumor (ATRT) are further defined by their molecular features. Also new entities have been added defined by both histology and molecular signatures including H3 K27M-mutant diffuse midline glioma, RELA fusion-positive ependymoma and diffuse leptomeningeal glioneuronal tumor (DLGNT).
These more homogeneous and narrowly defined entities are expected to facilitate better classification, prognostication and patient stratification for precision therapy. This also improves the design of clinical trials and experimental models.
In this presentation, we will review the new WHO classification scheme and review the imaging and as well as molecular/genetic features of pediatric CNS tumors.
Radiologists must keep up to date with updates to the WHO classification scheme to be able to better communicate with clinicians ensure optimal patient care and relevant research collaboration.
Read More

Authors:  Sato T Shawn , Sato Yutaka

Keywords:  WHO, Brain tumors

Dykie Adam,  Katzman Philip,  Chaturvedi Apeksha

Final Pr. ID: Poster #: EDU-037

Wilms tumors, also known as nephroblastomas, are overwhelmingly the most common renal tumors in children. While radiologists are abundantly familiar with the imaging appearances of these tumors, their knowledge of underlying histologic features, prognostic variables, and treatment approaches may not be equally robust. Histologically typically triphasic and containing differing proportions of blastemal, epithelial, and stromal components, the prognosis of individual subtypes differs. The “teratoid” variant of Wilms tumor contains components of differentiated tissues such as muscle, bone, cartilage, and fat. Focal and diffuse anaplasia are important histological features with diffuse anaplasia recognized as the most important prognostically unfavorable feature. In addition, there is an intermediate category of “nuclear unrest” which carries some but not all features of anaplasia. Clinical approaches differ between the two major renal tumor clinical research groups: the International Society of Paediatric Oncology (SIOP) Renal Tumor Study Group and the Children's Oncology Group (COG) Renal Tumor Group. This exhibit uses a case-based template of radiologic-pathologic correlation of different Wilms subtypes encountered at our institution, outlining treatment for individual tumor subtypes. We discuss differential diagnoses which may overlap with Wilms tumor on imaging, including nephrogenic rests, mesoblastic nephroma, malignant rhabdoid tumor of the kidney, clear cell sarcoma, and pediatric cystic nephroma (as a mimic of cystic Wilms tumor). We review Wilms tumor classification with known syndromic associations. We provide information on staging through the National Wilms Tumor Study system covering imaging and post-surgical findings. The ultimate goal is to make radiologists more effective members of the interdisciplinary tumor boards through enhancing a holistic understanding of Wilms tumor. Read More

Authors:  Dykie Adam , Katzman Philip , Chaturvedi Apeksha

Keywords:  Wilm's Tumor, Kidneys, Abdominal Imaging

Beckhorn Catherine,  Lee Rachel,  Cao Joseph

Final Pr. ID: Poster #: CR-028

Background: Granular cell tumors (GCT) are rare Schwann cell-derived soft tissue tumors typically presenting as a solitary, painless nodule in adults in the 4th to 6th decades of life, rarely presenting in children. GCT most commonly originate from gastrointestinal mucosa of the tongue or oral cavity, and are 2-3 times more common in women. While generally benign, approximately 2% of cases may be malignant; therefore, GCTs are typically treated with local surgical control.
Case: A 7-year-old male with sickle cell trait presented to clinic for a painless “pea-sized lump” on his penile shaft, which had been present since infancy but had recently grown larger. Ultrasound demonstrated a 1.8 cm lenticular-shaped mass on the dorsal mid shaft of the penis superficial to the fascial borders of the cavernous cavernosum. There was mild internal vascularity, but no invasion past fascial borders, surrounding soft tissue changes. On MRI, the mass was uniformly T1 iso-intense and T2 slightly hyperintense, with slight postcontrast enhancement. The mass was well-circumscribed, wrapped around the corpus cavernosum without invasion of the tunica albuginea, and exhibited uniform restricted diffusion, calcification, or internal fat. No lymph node involvement was noted on imaging. Surgical resection 8 months later noted a 3 cm mass, which now extended into the glans, with pathology consistent with benign GCT, with positive staining for S-100, CD68, SOX10, and calretinin, Ki-67 of 1%, and negative for SM actin. Due to depth of glans penetration, R2 resection was achieved based on the pediatric urologist’s decision to spare the glans, rather than performing a more aggressive distal penectomy. Post-operative MRI 4 months later demonstrated residual tumor with interval reduction in mass size. Additional investigation of the patient’s past medical history revealed that a benign GCT had been removed from the inside of his lip the previous year which helped support the final histopathologic diagnosis. While multifocal GCTs have been described in genetic syndromes, this patient had not undergone genetic testing to date. Read More

Authors:  Beckhorn Catherine , Lee Rachel , Cao Joseph

Keywords:  MRI, Genitourinary, Soft-Tissue Tumors

Lee Rachel,  Beckhorn Catherine,  Cao Joseph

Final Pr. ID: Poster #: CR-038

Keratin-positive giant cell-rich tumor is a very rare subset of giant cell-rich tumors characterized by keratin-positive cells and HMGA2::NCOR2 gene fusion. First described in 2021, fewer than 40 cases have been reported in the English literature. Reported cases have shown a strong predilection for females in their 20s-30s and occur in both soft tissue and bone. The youngest reported case thus far has been a 13-year-old female.

A 4-year-old previously healthy male presented to clinic with leg pain and a limp after falling on his right leg. Initial X-ray showed a lytic lesion on the right proximal tibia. Follow up MRI showed a 2 x 2.5 x 4.3 cm solid lesion in the proximal tibial metaphysis extending into the epiphysis. The mass was T1 and T2 hyperintense and demonstrated homogenous enhancement after contrast. No periostitis was noted on radiograph and no subperiosteal collection was present. No soft tissue component was identified. CT chest, abdomen, pelvis was ordered to rule out other sites of disease and showed multi-station lymphadenopathy throughout the right groin. Biopsy revealed tumor cells negative for H3G34W and H3K36M histone markers, specific for giant cell tumor of bone and chondroblastoma respectively. The cells were focally positive for AE1/AE3, a stain for keratin. Next-generation sequencing revealed an HMGA2::NCOR2 fusion confirming the diagnosis of keratin-positive giant cell-rich tumor. He underwent complete curettage of the lesion as well as excision of 2 inguinal lymph nodes that were negative for disease. Read More

Authors:  Lee Rachel , Beckhorn Catherine , Cao Joseph

Keywords:  Bone Tumor, Musculoskeletal

Sharma Priya,  Loubriel Daphne,  Estrin Yvonne,  Rajderkar Dhanashree

Final Pr. ID: Poster #: EDU-119

Learning objectives: This educational exhibit will review atypical ovarian neoplasms in the pediatric population. We will discuss the epidemiology, clinical presentation, multimodality imaging findings as seen on ultrasound (US), computed tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography/computed tomography (PET/CT) of these rare adnexal entities. Read More

Authors:  Sharma Priya , Loubriel Daphne , Estrin Yvonne , Rajderkar Dhanashree

Keywords:  Atypical ovarian masses, Adnexa, Ovarian, tumors

Gupta Ayushi,  Monforte Hector,  Schaaf William,  Kucera Jennifer

Final Pr. ID: Poster #: EDU-031

Pediatric patients can host a variety of benign and malignant renal tumors and tumor mimics. Their clinical presentation is nonspecific as patients may be asymptomatic or present with an abdominal mass, flank pain, or hematuria. Proper patient management requires an accurate diagnosis based on a combination of radiological and pathological findings. Read More

Authors:  Gupta Ayushi , Monforte Hector , Schaaf William , Kucera Jennifer

Keywords:  renal tumor, Wilms tumor, renal cell carcinoma

Jayapal Praveen,  Alharthi Omar,  Thakor Avnesh

Final Pr. ID: Poster #: CR-018

HISTORY AND CLINICAL COURSE:A 16-year-old male presented with worsening abdominal pain, non-bloody diarrhea, and unintentional weight loss. On exam, he had diffuse abdominal tenderness and unnoticed left testicular swelling. US demonstrated a heterogeneous left testicular mass; CT demonstrated a large heterogeneous left testicular mass with extensive tumor thrombus propagating along the left gonadal vein, into the renal vein, the IVC, right atrium, right ventricle with several metastatic pulmonary emboli. The patient underwent left orchiectomy, and pathology showed testicular mixed germ cell tumor with immature teratoma and yolk sac components. The patient was referred to our institution for further management of his intravascular and intracardiac tumor components, given the patient’s pathology fell under an intermediate category based on the “Intermediate Germ Cell Consensus classification” which supports treatment with curative intent given an 80% long-term survival rate.
PROCEDURE:An open surgical approach was used to remove tumor tissue from the right heart and supra-hepatic IVC. Percutaneous endovascular thrombectomy was then used to remove the tumor from the infra-hepatic IVC and the left renal vein 24 hours after the cardiac surgery.
Step 1: Distal Embolic Control: A single disc from an Inari Flow Triever device was deployed, via the right internal jugular vein, below the level of the hepatic veins to provide distal embolic control throughout the procedure. This is an alternative application for this device.
Step2: IVC tumor removal: An Inari Clot Triever device was deployed via the right common femoral vein. Multiple 360-degree sweeps throughout the IVC to ensure intravascular tumor removal as validated on follow-up IVC venography.
Step 3: Left renal vein tumor removal: The left renal vein thrombus was removed using a combination of the Flow Triever and aspiration catheter systems. IVUS and left renal venogram confirmed the tumor removal.
FOLLOW-UP:The hospital course was uneventful. The patient was anticoagulated with a tight INR goal of 1.5-2 to balance the need for anticoagulation and avoiding the risk of bleeding from recent cardiac surgery. Chemotherapy with Bleomycin, Cisplatin, and Etoposide was initiated upon discharge and will be reviewed by surgical oncology for retroperitoneal nodal clearance later. This case demonstrates the multi-disciplinary team approach to caring for a complex case of an extensive testicular tumor in a pediatric patient. Read More

Authors:  Jayapal Praveen , Alharthi Omar , Thakor Avnesh

Keywords:  Endovascular, thrombectomy, testicular tumor

Goncalves Fabricio,  Khrichenko Dmitry,  Martin-saavedra Juan,  Alves Cesar Augusto,  Teixeira Sara,  Andronikou Savvas,  Vossough Arastoo

Final Pr. ID: Paper #: 160

Accurate preoperative imaging diagnosis of supratentorial pediatric intraventricular tumors is not always straightforward. Conventional MRI features such as T1- and T2-weighted signal and contrast enhancement may overlap among various choroid plexus tumors and embryonal tumors. This study aimed to perform apparent diffusion coefficient (ADC) histogram analysis, assessing various metrics in different intraventricular for histological classification. Read More

Authors:  Goncalves Fabricio , Khrichenko Dmitry , Martin-saavedra Juan , Alves Cesar Augusto , Teixeira Sara , Andronikou Savvas , Vossough Arastoo

Keywords:  Intranventricular tumors, ADC, Diffusion

Harvey Carly,  Allbery Sandra,  Powers Andria

Final Pr. ID: Poster #: CR-074

Juvenile Granulosa Cell Tumor of the Testis is a rare testicular tumor that falls within the category of stromal cord neoplasms. There have been less than 50 cases described in the literature with all cases being described as having a cystic component (1, 2). We present a case of a 6 month old boy presenting with unilateral scrotal swelling. Ultrasound evaluation revealed an entirely solid, hypervascular, intratesticular mass. Alpha-fetoprotein levels were negative. At surgery, radical orchiectomy was performed and pathology revealed a Juvenille Granulosa Cell Tumor.

Testicular tumors arising in the neonatal and prepubescent period are a unique set of tumors distinct from their adult counterparts. In this younger age group, germ cell tumors predominate. Of the germ cell tumors, Yolk Sac tumors are the main tumor of clinical significant, and the reason why alpha-fetoprotein is such a relevant marker (3). Stromal cell tumors account for only a small percentage of testicular tumors within this age group. When they do occur, Juvenile Granulosa Cell Tumors (JGC) predominate, particularly if the child is under 1 year of age, with this tumor type being the most common testicular tumor present at birth (3,4). JGC tumors are associated with genetic and structural anomalies of the Y chromosome, ambiguous genitalia, and contralateral undescended testis. One of their defining pathologic characteristics is positive staining with Inhibin (3).To date there are no cases of metastatic JGC tumors. In all reported cases, Sonographic evaluation demonstrates a grossly multicystic tumor (2). The adult variant of Granulosa Cell tumors can appear as a solid mass with little or no cystic component and carries the risk of metastasis of approximately 20% (2). Surgical management of testicular JGC tumors has largely been radical orchiectomy but, some studies have shown tumor sparing excision to be curative in cases with salvageable testicular parenchyma (2). This finding highlights the importance of including stromal cell tumors, particularly JGC, in the differential for solid appearing testicular masses in the neonate. With more reported cases of JGC tumors of the testes, surgical management could include a more conservative approach. Although JGC tumor of the testis is not known to be malignant, given the atypical features of this tumor and similarities with its more malignant adult counterpart, close surveillance is warranted to ensure benign course. Read More

Authors:  Harvey Carly , Allbery Sandra , Powers Andria

Keywords:  Juvenille Granulosa Cell Tumor, Testicular Tumor, Pediatric, Solid

Biyyam Deepa,  Youssfi Mostafa,  Mandell Gerald,  Taylor Steve,  Patel Mittun

Final Pr. ID: Poster #: CR-004

Calcifying nested stromal epithelial tumor (CNSET) is a very rare primary liver tumor in children. To our knowledge, about 30 cases have been reported in literature. We describe the imaging appearance and histopathologic features of this tumor detected in a 2 year old girl who presented with an incidentally detected calcified liver lesion on a chest x-ray which was obtained for cough.
Computed tomography (CT) demonstrated a 5.5 centimeter sized heterogeneous mass with large coarse calcifications. MRI better demonstrated the margins of the lesion, which was predominantly hyper-intense on T2-weighted images. Large areas of signal void were seen in the superior aspect of the lesion, corresponding to the calcifications seen on CT. The lesion demonstrated restricted diffusion. Post-contrast, the lesion demonstrated enhancement in the portal venous phase with washout on the delayed phase. Initial diagnosis based on imaging findings and patient’s age was hepatoblastoma. However, serum alpha- fetoprotein (AFP) was normal, which is unusual with hepatoblastoma. Patient underwent subsequent wedge biopsy, which was proven to represent calcifying nested stromal epithelial tumor of the liver. PET/CT, obtained to evaluate for metastatic disease, demonstrated increased FDG activity within the primary hepatic lesion, with SUV Max of 3.5, with no evidence of FDG avid metastatic disease. She then underwent right hepatectomy and cholecystectomy.

No sign of tumor recurrence has been noted to date on the follow up abdominal ultrasound examination in the past 2.5 years.

Calcifying nested stromal epithelial tumor should be considered in the differential when a large heterogeneous liver tumor with coarse/ chunky calcifications is identified at imaging in the absence of elevated serum AFP in a child. Currently the standard treatment in complete surgical excision and liver transplantation if excision is not possible. Read More

Authors:  Biyyam Deepa , Youssfi Mostafa , Mandell Gerald , Taylor Steve , Patel Mittun

Keywords:  nested stromal epithelial, tumor, liver

Lacroix Caroline,  Ahyad Rayan,  Gupta Abha,  Chavhan Govind

Final Pr. ID: Poster #: CR-065

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor, of low malignant potential. It is typically seen in adults, but it can also less frequently affect children.

Because of its multisystem involvement, it can present in various ways both clinically and on imaging. It can present with multiple hepatic lesions and/or with multinodular lung disease simulating interstitial lung disease, infectious disease and metastatic disease among others. Multifocal bone involvement is another presentation of EHE.

One of the radiologist's daily practice challenges is that rare diseases can have significant overlap of imaging features with more frequent diagnoses (that sometimes present with atypical features). It is therefore of high relevance to know the classical and unique imaging features and signature of rare entities such as epithelioid hemangioendothelioma, in order to raise clinical suspicion when needed to direct appropriate diagnostic work-up. Our goal is to present a case of EHE, discuss possible clinical presentations and illustrate classical imaging findings. An overview of clinical implications of this diagnosis and of the available treatment options will also be provided.

Combining multisystem affection and characteristic imaging features, especially MRI features of liver lesions, radiologist can help to make early diagnosis of EHE that is rarely seen in children. Read More

Authors:  Lacroix Caroline , Ahyad Rayan , Gupta Abha , Chavhan Govind

Keywords:  epithelioid, hemangioendothelioma, vascular, tumor, multisystem

Bhalla Deeksha,  Jana Manisha,  Manchanda Smita,  Bhalla Ashu,  Naranje Priyanka

Final Pr. ID: Poster #: EDU-069

Teaching points:

The spectrum of neck masses in neonates and infants (< 2 years) differs considerably from those in older children
Understand characteristic imaging appearances, particularly recognise entities that do not require sampling for diagnosis
Learn algorithmic approach to differential diagnosis based on age and lesion morphology with case based examples

Table of contents:
Introduction: Incidence, clinical considerations
Classification:
Age: Neonate
Cystic
Branchial cleft cyst
Dermoid cyst
Thyroglossal duct cyst
Solid:
Germ cell tumor
Congenital hemangioma
Neuroblastoma
Mixed
Primitive myxoid mesenchymal tumor (PMMT)
Teratoma

Age: Older infants
Cystic
Branchial cleft cyst
Dermoid cyst
Thyroglossal duct cyst

Solid:
Lymphoma
Granulocytic sarcoma
Rhabdomyosarcoma
Multinodular vacuolating tumor of infancy (MNTI)
Solitary fibrous tumor (SFT)
Fibrous tumors: Fibrous hamartoma of infancy, infantile fibrosarcoma

Vascular malformation (microcystic lymphatic, venolymphatic, arteriovenous)

Infections
Ludwig angina
Zygomycosis

Practical diagnostic algorithms based on age, location (involved neck space) and morphology
Conclusion Read More

Authors:  Bhalla Deeksha , Jana Manisha , Manchanda Smita , Bhalla Ashu , Naranje Priyanka

Keywords:  Neck tumor, congenital, vascular malformation

Maleki Nazanin,  Amiruddin Raisa,  States Lisa,  Aboian Mariam

Final Pr. ID: Poster #: EDU-075

PET/MRI plays a significant role in Pediatric Oncology, but its implementation in Pediatric Neuro-Oncology has not been well established. PET/MRI addresses a major challenge in pediatrics by providing capability for serial imaging to track disease response to therapy, while minimizing radiation exposure and sedation events. PET/MRI has become a critical imaging modality in the management of pediatric brain neoplasms and metastases, aiding in diagnosis, staging, treatment planning, and follow-up, all while reducing radiation burden, minimizing time spent in the hospital, and reducing the number of sedation events.
At our tertiary center for pediatric care, we have gained extensive experience in utilizing hybrid PET/MRI to manage complex cases referred from multiple institutions. We present a series of cases where hybrid PET/MRI provided critical information for patient management including nasopharyngeal rhabdomyosarcoma, refractory metastatic germ cell tumor, and neuroblastoma. We offer a forward-looking perspective on the current role of FDG PET/MRI and future applications of amino acid PET in improving patient outcomes and its role in distinguishing tumor progression from post-treatment changes. Amino acid PET/MRI use cases were compiled from literature review and demonstrate definitive roles of amino acid PET/MRI in decision making in brain tumor diagnosis, immediate post-surgical assessment, and delayed treatment response assessment.
18F-FDG PET/MRI representative cases from clinical practice:
Case 1.
18 year old male with refractory metastatic germ cell tumor with anterior mediastinal mass and intracranial metastasis
Clinical Problem: Whole body evaluation in addition to detailed analysis of brain metastases in time efficient manner.
Imaging Solution: PET/MRI allowed detailed evaluation of hypermetabolic metastatic disease to the mediastinum and lungs and further detailed delineation of hemorrhagic brain metastases.
Case2.
4 year old boy with history of nasopharyngeal rhabdomyosarcoma with cerebellar metastasis who underwent resection and chemoradiation.
Clincial Problem: How to monitor nasopharyngeal mass after treatment in the setting of extensive postsurgical changes on MRI
Imaging Solution: PET/MRI provides an excellent imaging method for monitoring disease recurrence in the setting of post-treatment changes in nasopharyngeal location. Recurrent tumor was identified as hypermetabolic lesion, while MRI was not definitive. Read More

Authors:  Maleki Nazanin , Amiruddin Raisa , States Lisa , Aboian Mariam

Keywords:  PET-MRI, Pediatric Imaging, Brain Tumors

Henault Kathryn,  Kanev Paul,  O'loughlin Michael

Final Pr. ID: Poster #: CR-054

Congenital glioblastoma multiforme (cGBM) is the rarest type of congenital brain tumors, constituting approximately 3.5% of cases according to the latest literature, with roughly 60 cases published worldwide. This specific presentation can be defined as ‘definitely congenital’, based on the 1964 classification of congenital tumors by Solitare and Krigman. The case report discusses the clinical presentation, radiologic and histologic findings, treatment, and prognosis of cGBM.

The tumor was first detected on an ultrasound at 31 weeks gestation, performed for a clinical diagnosis of mild oligohydramnios, revealing an unexpected large intracranial lesion. Prior to this finding, the pregnancy course was uneventful - the fetus was naturally conceived by a 30-year-old G2P1001 without history of infection, radiation, or trauma. No concerning maternal past medical history or use of drugs/alcohol during pregnancy. No family history of CNS malignancy was documented. MRI performed at 31 3/7 weeks confirmed a large complex cystic and solid mass lesion, bigger than previously measured on the aforementioned ultrasound, with mixed T1 and T2 signal within the left parietal/occipital lobe. Findings were concerning for a mass lesion, specifically a GBM given the size and complex signal characteristics. A second ultrasound preformed 12 days later showed even further growth, raising suspicion for a rapidly enlarging tumor. A pediatric neurosurgeon and maternal fetal medicine team following the patient had preemptively decided to deliver the fetus at 36 weeks due to increasing macrocephaly, with a plan for postnatal biopsy to establish pathology before a definitive treatment plan was ascertained. Unfortunately the mother presented to the clinic at 33 1/7 weeks with no fetal movement and an ultrasound confirmed intrauterine demise. After a classic Cesarean section, an autopsy confirmed a diagnosis of cGBM. No other congenital abnormalities were revealed.

Although fetal brain tumors are exceedingly rare, it is imperative to diagnose them in-utero due to potential prenatal and postnatal complications. Co-morbidities such as polyhydramnios, spontaneous intracranial hemorrhage, dystocia during delivery, and immediate postnatal heart failure should be continually evaluated for. Knowledge of an intracranial mass will allow providers to appropriately plan the mood of delivery and immediate postnatal course with necessary specialists available for immediate intervention. Read More

Authors:  Henault Kathryn , Kanev Paul , O'loughlin Michael

Keywords:  congential brain tumor, glioblastoma multiforme

Siu Navarro Youck Jen,  Poletto Erica,  Malik Archana,  Koenigsberg Robert

Final Pr. ID: Poster #: EDU-053

Congenital tumors represent only 1.5–2% of all pediatric tumors, with a prevalence of 1:12,500 to 1:27,500 live births. Tumors are considered congenital when detected during pregnancy or in the first 3 months of life (1). Nowadays, with more accessible prenatal care and fetal imaging, these tumors can be detected very early during fetal period. Some lesions are benign, while others carry high risk of morbidity and mortality postnatally. As a radiologist, it is important to be familiar with these tumors by recognizing their imaging features, imaging modality work up and differential diagnosis. Doing so, we contribute to a proper imaging evaluation, early diagnosis and management.

The objectives of this educational exhibit are:
-To describe the imaging features of some congenital tumors on different image modalities.
-To recognize and emphasize the key radiologic findings of each tumor and their differential diagnoses.
-To briefly review the literature, including etiology, epidemiology, cytopathology characteristics, diagnosis and treatment.

Pictorial cases from our Radiology Department will be used to describe the imaging features of the following entities:

1) Head/Neck:
● Atypical teratoid/rhabdoid tumor (ATRT)
● Cervical teratoma
● Hemangioma

2) Chest:
● Neuroblastoma
● Cardiac rhabdomyoma

3) Abdomen- Pelvis:
● Hepatic hemangioendothelioma
● Hepatic hemangioma
● Neuroblastoma
● Mesoblastic nephroma
● Sacrococcygeal teratoma

4) Soft tissues:
● Infantile fibrosarcoma

5) Systemic:
● Leukemia Read More

Authors:  Siu Navarro Youck Jen , Poletto Erica , Malik Archana , Koenigsberg Robert

Keywords:  congenital tumor, neonatal tumors

England Elizabeth,  Sarma Asha,  Thomas John,  Liang Jiancong,  Snyder Elizabeth

Final Pr. ID: Poster #: CR-011

Intrascrotal lipoblastomas are rare, benign paratesticular tumors that arise from embryologic adipose tissue and most often affect young children. Due to the rare nature of these tumors, preoperative diagnosis can be challenging, despite imaging features that are somewhat distinctive with respect to more common lesions (e.g., rhabdomyosarcoma). Prospective diagnosis, however, has potential to influence the operative management strategy (for example, minimally invasive mass excision versus radical inguinal orchidectomy). Since 2016, only two cases of preoperative imaging diagnosis of scrotal lipoblastoma have been published. This case report will describe a unique case of an incidentally found intrascrotal lipoblastoma and discuss the pertinent sonographic features of this rare entity. A 3-year-old boy initially presented for evaluation for an atrophic and undescended right testicle. During that evaluation, the patient was incidentally found to have a palpable left scrotal mass. Subsequent scrotal ultrasound showed an 2.7 x 1.9 x 2.4 cm well circumscribed ovoid mass inferior to the left testicle which was in the inguinal canal. The mass appeared predominantly hypoechoic with mild internal vascularity and also contained areas of hyperechogenicity resembling fat. The ultrasound appearance of the mass was not felt to be typical of a rhabdomyosarcoma (the most common paratesticular mass in children) and other fat-containing masses, including lipoblastoma, were suggested as diagnostic considerations. Complete excision of the left paratesticular mass was successful and subsequent scrotal orchidopexy was performed. Microscopic examination of the mass demonstrated lobules of adipocytes with occasional lipoblasts. Rearrangement of the PLAG1 (8q12.1) locus was detected, confirming the diagnosis of lipoblastoma. Although rare, prospective imaging detection of intrascrotal lipoblastoma may allow for less invasive and morbid surgical intervention and decreased risk for local recurrence. Read More

Authors:  England Elizabeth , Sarma Asha , Thomas John , Liang Jiancong , Snyder Elizabeth

Keywords:  Paratesticular tumor, Lipoblastoma

Yoon Hye-kyung,  Yoon Hee-mang

Final Pr. ID: Poster #: EDU-049

Our purpose is to show representative cases of CNS (Central Nervous System) embryonal tumors in children with discussion of the new WHO (World Health Organization) classification system recently issued in 2016. Read More

Authors:  Yoon Hye-kyung , Yoon Hee-mang

Keywords:  Pediatric, Brain tumor, Medulloblastoma

Carmona Daniela,  Erripa Jose,  Lostra Juliana,  Rizzi Ana,  Dardanelli Esteban,  Moguillanky Slvia,  Lipsich Jose

Final Pr. ID: Poster #: CR-017

Gastrointestinal stromal tumors (GIST) in children are not well characterized, different adult rare entity. It corresponds to a heterogeneous group of lesions for correct classification requires histological, immunohistochemical evaluation and in some cases also adds to the cytogenetic diagnosis. We describe the findings of pediatric GIST. Read More

Authors:  Carmona Daniela , Erripa Jose , Lostra Juliana , Rizzi Ana , Dardanelli Esteban , Moguillanky Slvia , Lipsich Jose

Keywords:  gastrointestinal stromal tumors, CD17 +, children, imaging

Infante Ignacio,  Amoretti Natalia,  Roa Cintia,  Rizzi Ana,  Lipsich Jose

Final Pr. ID: Poster #: CR-004 (S)

Liver tumors account for 1-4% of all pediatrics malignancies. Most primary liver tumours in children are malignant, but one-third are benign. Mesenchymal hamartoma of the liver, though rare is the second most frequent benign liver mass in children. Generally occurs in children less than 2 years of age with a male preponderance (male: female, 2:1). We report a case of mesenchymal hamartoma of the liver in an 11 month-old female presented at the emergency department with abdominal distension of two months of evolution associated with intermittent vomiting, diarrhea and chronic malnutrition. Abnormal labs included elevated alpha-fetoprotein levels (15006 ng/ml) and non detectable beta Human Chorionic Gonadotrophin. The liver function was normal. Imaging demonstrated an abnormal abdominal X-ray, with soft tissues mass enlargement and displacement of bowel. An ultrasound revealed a mixed mass (cystic-solid) liver in segment VII. On colour-Doppler flow imaging, there was evidence of increased vascularity. Contrast-enhanced CT scan of the abdomen in arterial and venous phases showed a large cystic mass in the left lobe of the liver, with peripheral solid tissue, very vascularized in both vascular secuences. Percutaneous biopsy was done and pathology confirmed mesenchymal hamartoma. The patient underwent left hepatectomy. The patient evolved successfully. Read More

Authors:  Infante Ignacio , Amoretti Natalia , Roa Cintia , Rizzi Ana , Lipsich Jose

Keywords:  Cystic Mass, tumor, Imaging

Lionberg Alex,  Ong Seng

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

Authors:  Lionberg Alex , Ong Seng

Keywords:  Neuroblastoma, Oncology, Tumor Staging

Chambers Greg,  Zarfati Angelo,  Malthete Cellier Cecile,  Adamsbaum Catherine,  Branchereau Sophie,  Franchi-abella Stephanie

Final Pr. ID: Poster #: SCI-009

Describe imaging features of pediatric focal nodular hyperplasia (pFNH) in a large cohort of children with clinical, radiological and surgical management with a significant follow up period. We aim to provide an algorithm to treat these patients using the evidence base we have created. Read More

Authors:  Chambers Greg , Zarfati Angelo , Malthete Cellier Cecile , Adamsbaum Catherine , Branchereau Sophie , Franchi-abella Stephanie

Keywords:  Liver, Tumor

Carrasco Rosario,  Hulett-bowling Rebecca

Final Pr. ID: Poster #: EDU-094

Pleuropulmonary blastoma (PPB) is a rare, intrathoracic, malignant tumor in the pediatric population with approximately 500 cases reported worldwide. Over 90% of these cases are in patients below the age of 6. The spectral morphology of PPB is used to classify the lesions along a continuum from the least malignant to the most malignant: type I (cystic) 14%, type II (solid and cystic) 48%, and type III (solid) 38%. Congenital lung cysts are not known to degenerate to become PPB, but the cystic type I PPB may progress to the more aggressive type II or type III PPB. In addition, PPB is associated with cystic nephroma in 30% of cases, and has been linked to the DICER1 mutation which puts these patients at risk for other tumors. For example, the genetic basis of the PPB familial syndrome (which is the heterozygous loss of function mutation of DICER1) includes PPB, cystic nephroma, ovarian Sertoli-Leydig cell tumors, ciliary body medulloepithelioma, nodular hyperplasia and differentiated carcinoma of the thyroid gland, pituitary blastoma, pineoblastoma, nasal chondromesenchymal hamartoma, and cervical embryonal rhabdomyosarcoma.
The purpose of this educational report is to demonstrate various presentations and identify distinguishing features of each type of PPB as seen on initial radiographs with correlation on subsequent CT scans. Only cases where the PPB and type were confirmed by pathology are included. At least one case of the PPB familial syndrome will also be presented. Positive DICER1 mutations will be provided when available, as this information is increasingly used to aid in the treatment decisions. Early recognition of PPB with timely investigation for cystic nephroma and DICER1 mutations can lead to improved patient outcomes. Read More

Authors:  Carrasco Rosario , Hulett-bowling Rebecca

Keywords:  pediatric, pleural, tumor

Lacroix Caroline,  Balamuth Naomi,  Miller Tamara,  Shekdar Karuna

Final Pr. ID: Poster #: SCI-044

To describe the imaging manifestations of orbit involvement in extra orbital oncological processes in the pediatric population. Read More

Authors:  Lacroix Caroline , Balamuth Naomi , Miller Tamara , Shekdar Karuna

Keywords:  Tumor, Orbital, Neoplastic

Chitalkar Sachin,  Shet Narendra

Final Pr. ID: Poster #: EDU-055

To review the characteristic imaging findings of pediatric fibroblastic/myofibroblastic masses to aid in their diagnosis and management.
Soft tissue lesions in pediatric patients encompass a wide variety of pathologies. While a comprehensive review of all possible pathologies is beyond the scope of this poster, we aim to review one particular subset of lesions, fibroblastic lesions (including myofibroblastic neoplasms), in hopes to bring these to mind when evaluating a pediatric soft tissue mass to improve diagnosis and management of these entities.
Soft tissue masses typically present clinically on the basis of history and physical examination. US and MRI are used as first- and second-line imaging modalities, with limited roles for radiographs, CT and FDG-PET. In this poster, we will review the pertinent imaging findings of these neoplasms. Read More

Authors:  Chitalkar Sachin , Shet Narendra

Keywords:  Soft Tissue Mass, Soft-Tissue Tumors, Pediatric Radiology

Ro Esther,  Schooler Gary,  Morin Cara,  Khanna Geetika,  Towbin Alexander

Final Pr. ID: Poster #: EDU-029

The American College of Radiology Pediatric LI-RADS Working Group was formed in 2017 with the task of advancing the imaging work up, diagnosis, and management of pediatric liver tumors, particularly pediatric hepatic malignancies. This exhibit will present how the Pediatric LI-RADS Working Group is working towards this goal by means of advocacy, education, and research. In regard to advocacy, the Working Group is a strong proponent for the standardization of imaging practice for pediatric liver tumors. Based on expert interpretation of the literature, the Working Group established consensus imaging recommendations for children with a known or suspected liver neoplasm. This exhibit will highlight the key recommendations for choosing imaging modality and protocol for the work up of a pediatric liver tumor. Education is another major component of the Working Group’s mission. The group has presented at meetings and published manuscripts on various topics. Topics include age and indication-based imaging strategies for pediatric liver tumors and liver masses in children with predisposition syndromes. This exhibit will touch on the major teaching points of these papers. Lastly, this exhibit will present the research conducted by the Pediatric LI-RADS Working Group. Their research studied the utility of applying adult LI-RADS criteria to children. Another study analyzed the imaging features of hepatocellular carcinoma in children with and without an underlying predisposition. To summarize, through discussion of the major accomplishments of the Pediatric LI-RADS Working Group, this exhibit will provide insight, education, and resources for the radiologist interpreting pediatric liver tumors. Read More

Authors:  Ro Esther , Schooler Gary , Morin Cara , Khanna Geetika , Towbin Alexander

Keywords:  Liver Tumor, Malignancy, Abdominal Imaging

Luo Yu,  Crane Gabriella

Final Pr. ID: Poster #: EDU-123

Bone and soft tissue tumor in pediatric hands and feet are not infrequently encountered in pediatric patient. The lesion can be benign and malignant. Some are specific on imaging and some are nonspecific requiring biopsy. Recognizing imaging specific findings and correctly triage the patient is important in patient management. Read More

Authors:  Luo Yu , Crane Gabriella

Keywords:  Musculoskeltal, tumor, hands and feet

Lyon Jane,  Le Hau

Final Pr. ID: Poster #: CR-014

Inflammatory Myofibroblastic Tumor (IMT) is now considered a distinct entity and a true neoplasm within the heterogeneous group of inflammatory mass-forming tumors. It is now recognized as a fibroblastic/myofibroblastic neoplasm with intermediate biological potential. It occurs predominantly in children. Abnormalities on Chromosome 2p23 with a rearrangement of the ALK (anaplastic lymphoma kinase) locus causes abnormal tyrosine kinase receptor expression. Chromosomal abnormalities suggest a clonal origin and not just a reactive process or “pseudotumor,” as these masses have been categorized in the past. Up to sixty percent of inflammatory myofibroblastic tumors express ALK which may help establish the diagnosis of the inflammatory mass as an IMT. The masses can occur in a variety of locations and have non-specific imaging findings, which will be reviewed.

We present three cases, each in a different location:

Case 1: 7 year old female presents with chronic cough and persistent right middle lobe abnormality on chest x-ray with concern for pneumonia or inhaled foreign body. She was found to have a soft tissue mass in her right mainstem bronchus with extra-luminal extension. CT, MR and gross surgical photos of the endobronchial IMT will be presented.

Case 2: 20 month old male presents with hepatomegaly, jaundice and elevated bilirubin, alkaline phosphatase and liver function tests. Ultrasound and MR images of the pancreatic head IMT causing biliary obstruction will be presented.

Case 3: 13 year old male presents with back, leg and pelvic pain. MR, CT and gross surgical photos of the right posterior pelvic sidewall IMT, which had evidence of nerve entrapment at biopsy, will be presented.
The imaging, pathological and surgical findings from these patients, where available, will be presented and reviewed.

We suggest that the radiologist consider Inflammatory Myofibroblastic Tumor in the differential diagnosis for inflammatory and fibrous lesions in children. Read More

Authors:  Lyon Jane , Le Hau

Keywords:  Inflammatory Myofibroblastic Tumor, Pediatric, Neoplastic

Aldraihem Ahmed,  Abdeen Nishard

Final Pr. ID: Poster #: SCI-047

To determine the inter-observer agreement in measurement of relative cerebral blood volume (rCBV) in pediatric brain tumors, using normal grey matter as a control. Read More

Authors:  Aldraihem Ahmed , Abdeen Nishard

Keywords:  brain tumor, T2* perfusion, interobserver agreement