Survival and Scoliosis Following Resection of Chest Wall Tumors in Children and Adolescents: A Single-center Retrospective Analysis.

OBJECTIVE: We reviewed our experience with pediatric chest wall tumors (CWTs) to identify variables associated with survival, scoliosis development, and need for corrective scoliosis surgery. BACKGROUND: Chest wall neoplasms in children or adolescents are rare. Consequently, there are few large series that detail survival or quality of life indicators, like scoliosis. METHODS: Medical records were reviewed for all chest wall resections for primary and metastatic CWT performed from October 1, 1986 to September 30, 2016 on patients 21 years or younger at diagnosis. Kaplan-Meier distributions were compared using the log-rank test. Variables correlated with survival, scoliosis development, or need for corrective surgeries were analyzed using competing-risk analysis. RESULTS: Seventy-six cases [57 (75%) primary, 19 (25%) metastatic] were identified. Median age at diagnosis was 15.6 years (range: 0.5-21 years). Tumor types were Ewing sarcoma family tumors (54%), other soft tissue sarcomas (21%), osteosarcoma (11%), rhabdomyosarcoma (7%), and other (8%). A median of 3 (range: 1-5) contiguous ribs were resected. Surgical reconstruction included composite Marlex mesh and methyl-methacrylate, Gore-Tex, or primary closure in 57%, 28%, and 14% of procedures, respectively. Overall 5-year survival was 61% (95% confidence interval: 50%-75%). Scoliosis developed in 19 (25%) patients; 6 patients required corrective surgery. Variables associated with overall survival were the presence of metastatic disease at diagnosis, and whether the chest tumor itself was a primary or metastatic lesion. Younger age at chest wall resection was associated with the need for corrective surgery in patients who developed scoliosis. CONCLUSIONS: Among pediatric and adolescent patients with CWTs, survival depends primarily on the presence of metastases. Age, type of chest wall reconstruction, and tumor size are not associated with scoliosis development. Among patients who develop scoliosis, younger patients are more likely to require corrective surgery.

Pediatric Hodgkin Lymphoma, Version 3.2021.

Hodgkin lymphoma (HL) is a highly curable form of cancer, and current treatment regimens are focused on improving treatment efficacy while decreasing the risk of late effects of treatment. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for pediatric HL provide recommendations on the workup, diagnostic evaluation, and treatment of classic HL, including principles of pathology, imaging, staging, systemic therapy, and radiation therapy. This portion of the NCCN Guidelines focuses on the management of pediatric classic HL in the upfront and relapsed/refractory settings.

Bevacizumab-associated Bowel Microperforation in a Patient With Neuroblastoma.

The antivascular endothelial growth factor antibody, bevacizumab, is effective against several malignancies in adults but unproven in pediatric oncology. In early phase pediatric studies toxicities were similar to those in adults. Bowel perforation in adults is a rare but serious toxicity, but has not been hitherto reported in children. A 5-year-old boy with chemoresistant neuroblastoma treated with bevacizumab plus radioimmunotherapy developed acute abdominal pain. Computed tomography scan showed free abdominal air and pneumatosis coli. Emergency laparotomy and bowel diversion were performed leading to complete recovery and timely continuation of antineuroblastoma therapy. Early recognition and rapid intervention can prevent a catastrophic outcome in bevacizumab-related bowel perforation.

Intracardiac Low-grade Sarcoma Following Treatment for Ewing Sarcoma.

A 16-year-old male was diagnosed with Ewing sarcoma of the ribcage with pulmonary metastases. Six months after completion of scheduled therapy, he was found to have a new intracardiac mass, presumed recurrent Ewing sarcoma. EWSR1 fusion was not detected by droplet digital polymerase chain reaction from blood plasma. After no improvement with salvage chemotherapy, he underwent surgical resection that identified a low-grade spindle cell sarcoma. Despite the near-synchronous presentation of 2 unrelated sarcomas, extensive genomic analyses did not reveal any unifying somatic or germline mutations nor any apparent cancer predisposition. This case also highlights the potential role of utilizing plasma cell-free DNA for diagnosing tumors in locations where biopsy confers high morbidity.

Pancreatic involvement in neuroblastoma with radiologic-pathologic correlation: a single-institution experience.

OBJECTIVE: Pancreatic involvement in neuroblastoma is extremely rare, with few cases reported in the literature. We present imaging findings of pancreatic involvement in neuroblastoma with clinical and pathologic correlation in the largest documented series to date. SUBJECTS AND METHODS: We prospectively reported pancreatic involvement evident on multimodality imaging in neuroblastoma patients presenting to our institution from 1997 to 2011. Lesions were classified according to location within the pancreas, and imaging features were correlated with cytogenetic and surgicopathologic findings. RESULTS: Neuroblastoma involving the pancreas was evident on imaging of seven of 1031 patients (mean age, 6.6 years). One patient had pancreatic involvement at presentation, and six developed pancreatic disease at relapse or disease progression. Pancreatic lesions were most frequently initially identified on concurrent CT and (123)I-metaiodobenzylguanidine scintigraphy, and additional lesions initially were found on MRI and ultrasound. Five of seven patients had focal lesions, one had diffuse pancreatic involvement, and one had pancreatic extension from contiguous disease. The distribution of lesions favored the pancreatic body and tail. All patients had International Neuroblastoma Staging System stage 3 or 4 disease, Children's Oncology Group intermediate- or high-risk disease, and unfavorable histology at initial diagnosis. For the five patients with surgical correlation, pancreatic surgical specimens revealed neuroblastoma in three cases and ganglioneuroblastoma in two cases. CONCLUSION: Although rare, pancreatic involvement in neuroblastoma occurs. Its variable imaging appearance should be considered when evaluating the retroperitoneum in patients with known or suspected neuroblastoma, particularly because increased patient survival holds the potential for uncommon patterns of recurrence.

Positron emission tomography for response assessment in desmoplastic small round cell tumor.

BACKGROUND: Desmoplastic small round cell tumors (DSRCT) typically have a large stromal component and often are extensively disseminated in the peritoneal cavity at diagnosis. These factors contribute to difficulty in quantifying response to chemotherapy using RECIST or WHO criteria. This study compares the overall disease response to chemotherapy by fluorodeoxyglucose-positron emission tomography (FDG-PET) and computed tomography (CT) in patients with DSRCT. METHODS: We conducted a retrospective chart review of 7 patients with DSRCT who were imaged by FDG-PET and CT at diagnosis and after 3 cycles of chemotherapy. Response to chemotherapy was graded according to EORTC metabolic response guidelines and RECIST. RESULTS: All tumors demonstrated some decrease in SUVmax (51%±21%) and longest diameter (23%±8%) with chemotherapy. The best response achieved by FDG-PET was a partial response in 6 patients and by CT was a partial response in 1 patient. Measured response was concordant between the 2 modalities in 2 patients. CONCLUSIONS: In this small series response measurement by FDG-PET did not always correlate with response measurement by CT. A greater decrease in metabolic activity as compared with size was seen in all patients. Further studies are needed to define the role of FDG-PET in assessing early response of DSRCT to chemotherapy.

Characteristic imaging features of desmoplastic small round cell tumour.

BACKGROUND: Desmoplastic small round cell tumour (DSRCT) is a rare malignant neoplasm. Its radiological features have rarely been described. OBJECTIVE: To assess the CT parameters characteristic of DSRCT. We also report our experience with combined FDG PET/CT in staging and follow-up for DSRCT. MATERIALS AND METHODS: The pretreatment diagnostic CT's of 65 patients with DSRCT were evaluated. Pertinent imaging findings were catalogued, with histopathology or serial follow-up studies as reference standard. Combined FDG PET/CT examinations of 11 of these patients who underwent pretreatment imaging were also reviewed. RESULTS: Sixty-two patients presented with primary intra-abdominal disease; three had primary extra-abdominal tumours at presentation. The most common imaging finding of patients with intra-abdominal DSRCT was multiple peritoneal soft tissue masses, with a dominant mass in the retrovesical or rectouterine location in more than half of the cases. Forty percent had metastatic disease to the liver, lungs, spleen or bones at diagnosis. FDG PET/CT accurately detected 97.4% of all DSRCT lesions. CONCLUSION: DSRCT typically presents as a large abdominopelvic mass with widespread peritoneal involvement predominantly in young males. Familiarity with its radiological features can help guide diagnosis and treatment. Functional imaging with PET/CT offers advantage over anatomical imaging for accurate disease staging.

Sporadic adenocarcinoma of the colon in children: case series and review of the literature.

BACKGROUND: Adenocarcinoma of the colon is rare in pediatric patients and thus not much is known about its clinical and imaging characteristics. OBSERVATIONS: We present 4 adolescents with an average age of 15 years who present with several month histories of significant weight loss and abdominal pain. All had an abdominal and pelvic computed tomography scan, which revealed an adenocarcinoma in the colon. One patient had metastatic disease at diagnosis. The main treatment was primary resection and chemotherapy. Two of the children had a family history of colon cancer. Our case series depicts similarities and differences in disease presentation, tumor location, pattern of metastasis, genetics, management between adults and children and conducts a review of the relevant literature concerning adenocarcinoma in the pediatric population. CONCLUSIONS: In children, this disease has more aggressive histologies and presents more frequently in an advanced stage. This is because it is not a diagnosis often considered, leading to poorer outcomes. When patients present in the correct clinical context, the possibility of colonic adenocarcinoma should be considered in the differential diagnosis, which may in turn lead to better outcomes.

Radiologic features of NUT midline carcinoma in an adolescent.

We present a case of NUT midline carcinoma (NMC) mimicking lymphoma in an adolescent boy, with attention to multidetector CT appearance and pattern of metastasis on [F-18]2-fluoro-2-deoxyglucose positron emission tomography (FDG PET/CT). Few case reports have focused on the imaging characteristics of this rare tumor in the pediatric imaging literature. A newly described but increasingly recognized disease entity, NMC should enter the differential of pediatric midline tumors displaying particularly aggressive characteristics on imaging.

Clinical and imaging experience with COVID-19 in nonvaccinated children with cancer.

Children with COVID-19 fare much better than adults but less is known about children with both COVID-19 and a cancer diagnosis in terms of clinical outcome and imaging. We describe our experience with a cohort of children with COVID-19 and cancer who have undergone medical imaging. We reviewed imaging and recorded clinical data and separated this group into two subgroups - hematologic and solid malignancies. Our observational data show that 1)children with hematologic malignancies may be at higher risk for complications, including death than, those with solid tumors, 2) that pulmonary imaging in the former group more often shows abnormalities and 3) that presence of pulmonary imaging abnormalities may portend an unfavorable outcome.

Variable MR imaging appearances of focal nodular hyperplasia in pediatric cancer patients.

BACKGROUND: Focal nodular hyperplasia (FNH) is rare in the general pediatric population but is increasingly found in treated pediatric cancer patients. FNH can be incidentally found on CT and MRI and can be misdiagnosed as liver metastasis in patients with an oncological history. OBJECTIVE: To describe the MR imaging findings of FNH in pediatric cancer patients. MATERIALS AND METHODS: Ten children who had been treated for a primary malignancy and who were diagnosed with FNH from 2003 to 2010 were identified from a search for FNH in our pathology and radiology databases. Patients were included if they were treated for a primary malignancy and had undergone MR imaging of the liver including T1-weighted, T2-weighted and dynamic contrast-enhanced T1-weighted sequences. RESULTS: FNH from all patients (n=10) demonstrated typical homogeneous arterial enhancement on MRI. The FNH was often multiple and small (7/10 patients), lacking a central scar, with variable imaging characteristics including signal hyperintensity on both T1- and T2-weighted images (4/10 patients), especially in the setting of hepatic hemosiderosis. CONCLUSION: FNH has a variable MR appearance in pediatric cancer patients and should be considered in the differential diagnosis of hypervascular liver lesions.

Skeletal imaging effects of pamidronate therapy in osteosarcoma patients.

BACKGROUND: After observing metaphyseal changes in pediatric and young adult patients with osteosarcoma who were treated with pamidronate, we initiated a retrospective study to identify bone findings on computed radiography of the chest and extremities, chest CT, extremity MRI, and radionuclide bone scans in this population. OBJECTIVE: To review the generalized skeletal imaging findings on computed radiography, CT, and MR examinations in patients with osteosarcoma (OS) receiving pamidronate. MATERIALS AND METHODS: A retrospective review of 40 patients with newly diagnosed OS (mean age, 16.5 years) receiving pamidronate with chemotherapy were identified at one institution. Computed radiography, CT, MR, and bone scans on 36 patients were reviewed for osseous changes. RESULTS: Dense metaphyseal bands at the growth plates in long bones, epiphyseal ossification centers, anterior rib ends, sternum and spine were observed. Osseous changes occurred on CT in 69% of patients, and on computed chest radiography in 53%. Bone-within-bone appearance in the spine and ossification centers was identified on computed radiography in 36%. CONCLUSION: OS patients treated with pamidronate can develop metaphyseal sclerotic bands as well as epiphyseal and vertebral endplate sclerosis progressing to a bone-within-bone appearance. Findings occur more frequently in younger patients with open epiphyses.

Is IV contrast necessary for MRI follow-up in children with abdominal neuroblastoma?

PURPOSE: The safety of multiple doses of gadolinium-based MRI IV contrast has recently been called in to question. While the long-term safety is being investigated, here, we seek to determine if there is added value to the use of IV contrast for improving detection of tumoral recurrences in children with a history of abdominal neuroblastoma. METHODS: This is a retrospective review of children who underwent abdominal MRI with gadolinium contrast. One radiologist reviewer determined presence or absence of tumor, both before and after administration of IV contrast material and documented level of confidence when a finding was encountered. Change in reader confidence after the use of contrast was measured and fraction of missed lesions on pre-contrast was calculated. Liver and spleen lesions were documented separately. RESULTS: 453 MRI scans in 110 unique patients were reviewed. 65 patients were documented to have a total of 125 lesions, excluding liver, spleen and bones. There were 23 instances of contrast altering the radiologist's confidence and one lesion was missed without the use of contrast. Among liver and spleen, several hepatic lesions were seen only after contrast, but all were benign lesions. CONCLUSION: In selected patients who are undergoing MRI for neuroblastoma, it may be reasonable to forgo the use of IV contrast.

Emergency room imaging in pediatric patients with cancer: analysis of the spectrum and frequency of imaging modalities and findings in a tertiary cancer center and their relationship with survival.

BACKGROUND: To assess the spectrum and frequency of modalities used for emergency room (ER) imaging and their findings in pediatric cancer patients and assess their relationship with survival. METHODS: Consecutive pediatric cancer patients that underwent imaging during an ER visit at our tertiary cancer center over a 5-year period were retrospectively analyzed. Imaging findings were considered positive when they were relevant to the ER presenting complaint. Imaging positivity was correlated with inpatient admission. Overall survival (OS) was assessed with Kaplan-Meier curves and uni- and multi-variate Cox proportional hazards model was used to identify significant factors associated with OS. RESULTS: Two hundred sixty-one patients (135 males and 126 females; median age 11 years [interquartile range 5-16 years] with 348 visits and a total of 406 imaging studies were included. Common chief complaints were related to the chest (100 [28.7 %]) and fever (99 [28.4 %]). ER imaging was positive in 207 visits (59.5 %), commonly revealing increased metastases (50 [14.4 %]), pneumonia (47 [13.5 %]), and other lung problems (12 [2.9 %]). Positive ER imaging was associated with inpatient admission (69.3 % [133/192] vs. 40.4 % [63/156], p < 0.01). Multivariate survival analysis showed that positive ER imaging (hazard ratio [HR] = 2.35 [95% CI 1.44-3.83, p < 0.01), admission (HR = 1.86 [95% CI 1.17-3.00], p < 0.01), number of ER visits (HR = 3.08 [95% CI 1.62-5.83], p < 0.01 for ≥ 3 visits) were associated with poorer survival. CONCLUSIONS: Imaging was able to delineate the cause for ER visits in children with cancer in over half of the cases. Positive ER imaging was associated with admission and worse survival.

A novel image-based system for risk stratification in patients with desmoplastic small round cell tumor.

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is an aggressive soft tissue sarcoma affecting children and young adults with 5-year overall survival (OS) of approximately 20%. Despite generally poor prognosis, long-term survival does occur. However, no evidence-based system exists to risk-stratify patients at diagnosis. METHODS: We retrospectively reviewed all DSRCT cases diagnosed at our institution between January 2000 and September 2016. Demographics, diagnostic imaging, and clinical data were reviewed. Univariate and multivariate Cox proportional hazard modeling was used to evaluate associations between imaging characteristics and OS. RESULTS: There were 130 patients (85% male; median age at presentation: 21.2 years) with confirmed DSRCT and sufficient imaging and clinical information for analysis. Median 5-year OS was 28% (95% CI: 19%-37%). In univariate analysis, shorter OS was associated with presence of liver lesions (hazard ratio [HR] 2.1, 95% CI: 1.28-3.45), chest lesions (HR 1.86, 95% CI: 1.11-3.1), and ascites (HR 1.69, 95% CI: 1.06-2.7). In multivariate analysis, liver involvement and ascites were predictive and were used to stratify risk (intermediate=no liver involvement or ascites; high=either liver involvement or ascites; very high=both liver involvement and ascites). Intermediate-risk patients had a 5-year survival of 61% (95% CI: 40%-76%) versus 16% (95% CI: 6%-29%) among high-risk patients and 8% (95% CI: 1%-29%) among very high risk patients. CONCLUSION: Patients with DSRCT can be risk-stratified at diagnosis based on specific imaging characteristics. TYPE OF STUDY: Retrospective study with no comparison group. LEVEL OF EVIDENCE: Level IV.

Irinotecan and temozolomide for Ewing sarcoma: the Memorial Sloan-Kettering experience.

BACKGROUND: The prognosis for recurrent/progressive Ewing sarcoma (ES) remains poor. Pre-clinical, adult phase I and II trials have demonstrated the combination of irinotecan and temozolomide to have schedule-dependent synergy and significant antitumor activity. A pediatric phase I trial has shown this regimen to be safe and active in advanced ES. PROCEDURE: We conducted a retrospective chart review to identify patients with recurrent/progressive ES treated with irinotecan [20 mg/m(2)/day x 5(x2)] and temozolomide (100 mg/m(2)/day x 5) in our institution. The best response achieved, time to progression (TTP), and associated toxicities were recorded. RESULTS: Twenty patients received a total of 154 cycles of therapy. Of 19 evaluable patients, there were 5 complete and 7 partial responses (a 63% overall objective response). Median TTP for 20 evaluable patients with recurrent/progressive ES was 8.3 months; for the subset of 14 patients with recurrent ES, it was 16.2 months. Median TTP was better for patients who sustained a 2-year first remission than for those who relapsed < 24 months from diagnosis and for patients with primary localized vs. metastatic disease. Significant toxicities included grade 3 diarrhea (7 cycles), grade 3 colitis (1 cycle), grade 3 pneumonitis in one patient receiving concurrent whole-lung RT, grade 3-4 neutropenia (19 cycles), and grade 3-4 thrombocytopenia (16 cycles). CONCLUSIONS: Irinotecan and temozolomide is a well-tolerated and active regimen for recurrent/progressive ES. Prospective trials are necessary to define the role of this regimen in newly diagnosed ES.

Imaging of pediatric lymphomas.

Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) represent 10% to 15% of all malignancies occurring in children younger than 20 years of age. Advances in cross-sectional imaging and the availability of positron emission tomography (PET) and PET-CT have had a major impact on imaging and management of pediatric patients. This article reviews the clinical features of lymphoma, focusing on the spectrum of imaging findings seen in diagnosis, staging, and follow-up of HL and NHL. Pediatric NHL has four major histologic subtypes: Burkitt lymphoma, diffuse large B-cell lymphoma, anaplastic large cell lymphoma, and lymphoblastic lymphoma. The most important subtype of HL is nodular sclerosis.

Unusual abdominal metastases in osteosarcoma.

Intraabdominal metastases in the setting of osteosarcoma are very rare. We describe a case of a 17-year-old boy with high-grade right distal femur osteosarcoma who two years after diagnosis developed extensive intra abdominal metastases involving the omentum, peritoneum, bowel serosa, psoas muscles and abdominal soft tissue. Awareness of and surveillance for unusual patterns of metastasis may allow for earlier detection, intervention, and palliative care decision-making, which may affect survival and quality of life. This report underlines the need for prospective studies evaluating surveillance guidelines for patients after medical and surgical management of osteosarcoma, especially in cases complicated by pulmonary metastases.

Radiology-Pathology Conference: choledochal cyst.

We report the radiologic and pathologic findings of a newborn with a choledochal cyst and review the etiology, diagnostic considerations, and management of this entity.

Multifocal Osteosarcoma: Unusual Presentation and Imaging Findings.

Multifocal osteosarcoma is usually defined as the occurrence of a tumor at 2 or more sites in a patient without pulmonary metastases and may be synchronous with more than one lesion seen at presentation or metachronous with new tumors developing after the initial treatment. It is difficult to determine whether these represent synchronous multiple primary lesions or metastases. We present a rare case of widespread synchronous multifocal osteosarcoma and a brief review of the literature.