Search

Peritoneal sarcomatosis (PSC) is defined as peritoneal involvement of multiple sarcomatous tumors. Desmoplastic small round cell tumors (DSRCT) and rhabdomyosarcomas are the most common pediatric PSC cases. PSC has been treated with chemotherapy and mainly palliative surgery, but long-term outcome has been poor. New imaging technologies have improved the evaluation of disease extent and patterns of peritoneal dissemination, and cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (HIPEC) is being evaluated as a treatment option to prolong remission in pediatric patients. We will review the clinical characteristics, potential biologic mechanisms, radiographic characteristics, and potential therapies for pediatric PSC patients.

Sarcomas of the head and neck region are a rare group of tumors in children and present challenges with regard to evaluation and treatment. Rhabdomyosarcomas are the most common sarcomas of the head and neck in children. Presence of metastases and complete surgical resectability continue to be the most relevant clinical prognostic factors in patients with sarcomas. However, many patients present with unresectable tumors; these require radiation therapy, which is associated with concerns about immediate and long-term side effects. New technologies, including proton beam therapy (PBT), appear very promising in terms of reducing acute and long-term toxic effects. A multi-disciplinary approach is required for best long-term outcomes in children with head and neck sarcomas.

The purpose of this study was to determine, first, the accuracy with which radiologists reading posteroanterior chest radiographs differentiate whether a central venous line is in the superior vena cava or the azygos vein and, second, the circumstances in which radiologists may omit the lateral view to determine the position of a central venous line.Twenty-four radiologists evaluated 60 posteroanterior chest radiographs to determine the position of a central venous line in the superior vena cava or azygos vein. Investigators evaluated the appearance of the central venous lines to refine rules for determining central venous line position on a frontal radiograph and omitting the lateral view.The accuracy of posteroanterior radiography for determining central venous line position was 90% at one study location and 85.5% at the other. No central venous line in the azygos vein extended more than 10.9 mm caudal to the cephalic edge of the right main bronchus. No central venous line in the superior vena cava had a down-the-barrel or curved appearance at the caudal edge.For central venous lines extending at least 15 mm caudal to the cephalic edge of the right main bronchus and having no down-the-barrel or curved caudal appearance, categorization was nearly 100% accurate. Therefore, if desired to save radiation exposure and cost, it may be feasible to omit lateral views in radiography of patients with central venous lines extending at least 15 mm caudal to the cephalic edge of the right main bronchus in whom the caudal edge does not have a down-the-barrel or curved appearance.

Background Less than 200 cases have been reported in the world literature since desmoplastic small round cell tumor (DSRCT) was first described in 1989. To date, chemotherapy, radiation therapy, and surgery have resulted in a poor survival of 30% to 55%. We used hyperthermic intraperitoneal chemotherapy (HIPEC) at the time of complete tumor resection as an adjunct to treatment of pediatric and adolescent patients with DSRCT. Purpose The aim of this study was to assess survival as a function of disease burden and response to HIPEC in patients with DSRCT. Methods Twenty-four patients with DSRCT from 1995 to 2008 were evaluated. Eight patients undergoing cytoreductive surgery and HIPEC were compared with 16 historical controls that had chemotherapy ± radiation therapy or surgery alone. Results Median age was 12 years in 8 patients who underwent HIPEC. Significant morbidity after HIPEC included renal insufficiency and gastroparesis. There were no operative mortalities. The estimated median overall 3-year survival for patients not undergoing surgery or HIPEC was 26% compared with 71% in patients who underwent HIPEC. Extraabdominal metastasis correlated with poor survival ( P = .021). Conclusion Hyperthermic intraperitoneal chemotherapy is safe in children with DSRCT. It may prolong disease-free survival in selected cases of DSRCT. It may have a limited role as an adjunct to local control in patients with DSRCT.

The aim of our study was to retrospectively evaluate whether maximum standardized uptake value (SUVmax), total lesion gylcolysis (TLG), or change therein using 18F-FDG PET/CT performed before and after initial chemotherapy were indicators of patient outcome. Methods: Thirty-one consecutive patients who underwent 18F-FDG PET/CT before and after chemotherapy, followed by tumor resection, were retrospectively reviewed. Univariate Cox regression was used to analyze for relationships between covariates of interest (SUVmax before and after chemotherapy, change in SUVmax, TLG before and after chemotherapy, change in TLG, and tumor necrosis) and progression-free and overall survival. Logistic regression was used to evaluate tumor necrosis. Results: High SUVmax before and after chemotherapy (P = 0.008 and P = 0.009, respectively) was associated with worse progression-free survival. The cut point for SUVmax before chemotherapy was greater than 15 g/mL* (P = 0.015), and after chemotherapy it was greater than 5 g/mL* (P = 0.006), as measured at our institution and using lean body mass. Increase in TLG after chemotherapy was associated with worse progression-free survival (P = 0.016). High SUVmax after chemotherapy was associated with poor overall survival (P = 0.035). The cut point was above the median of 3.3 g/mL* (P = 0.043). High TLG before chemotherapy was associated with poor overall survival (P = 0.021). Good overall and progression-free survival was associated with a tumor necrosis greater than 90% (P = 0.018 and 0.08, respectively). A tumor necrosis greater than 90% was most strongly associated with a decrease in SUVmax (P = 0.015). Conclusion:18F-FDG PET/CT can be used as a prognostic indicator for progression-free survival, overall survival, and tumor necrosis in osteosarcoma.

Extensive peritoneal metastatic disease is rare in children. Although usually manifested as carcinomatosis in adults, sarcomatosis is more common in children. The authors began a pediatric hyperthermic intraperitoneal chemotherapy (HIPEC) program, and this report describes their initial results from the first 50 pediatric, adolescent, and young adult patients. A single-institution, retrospective study investigated the first 50 cytoreductive surgeries and HIPEC by one surgeon for patients 3–21 years of age. The HIPEC was added to chemotherapy and radiotherapy treatment. Demographics, outcome, and complications were recorded. The median follow-up period for the surviving patients was 21.9 months. The most common diagnoses were desmoplastic small round cell tumor (n = 21), rhabdomyosarcoma (n = 7), mesothelioma (n = 4), and other carcinoma (n = 17). Multivariate analysis showed that patients treated with HIPEC and an incomplete cytoreduction had a greater risk for recurrence than those who had a complete cytoreduction (p = 0.0002). The patients with a higher peritoneal cancer index (PCI) (i.e., a large tumor burden) had a median overall survival (OS) time of 19.9 months relative to the patients with a lower PCI score, who had a median OS of 34 months (p = 0.049). The patients without complete cytoreduction had a median OS of 7.1 months compared with 31.4 months for the patients with complete cytoreduction (p = 0.012). No perioperative mortalities occurred. The incidence of major complications was 28 %. Cytoreductive surgery and HIPEC with a programmatic approach for patients 3–21 years of age is unique. The best outcome was experienced by patients with desmoplastic small round cell tumor and those with complete cytoreduction. Complete cytoreduction for patients without disease outside the abdominal cavity at the time of surgery affords the best outcome.

Desmoplastic small round cell tumor (DSRCT) is a rare tumor of adolescents and young adults. Less than 100 cases per year are reported in North America. Extensive peritoneal metastases are characteristic of this disease. We performed cytoreductive surgery and hyperthermic peritoneal perfusion with chemotherapy (HIPEC) using cisplatin (CDDP) for DSRCT. A retrospective cohort study was performed on 26 pediatric and adult patients who underwent cytoreduction/HIPEC using CDDP for DSRCT at a single cancer center. Neoadjuvant chemotherapy, adjuvant chemotherapy, and postoperative enteral nutrition were given to all patients. Postoperative radiation therapy was given to most patients. Follow-up was from 6 months to 6 years. Outcome variables were evaluated for disease-free and overall survival (OS). Five patients (19 %) were less than 12 years of age at surgery. Patients who had disease outside the abdomen at surgery had a larger risk of recurrence or death than those who did not (p = 0.0158, p = 0.0393 time from surgery to death respectively). Age, liver metastasis, and peritoneal cancer index level did not significantly predict disease-free or OS. Patients who had CR0 or CR1 and HIPEC had significantly longer median survival compared with patients who had HIPEC and CR2 cytoreduction (63.4 vs. 26.7 months). HIPEC may be an effective therapy for children and young adults with DSRCT. Patients with DSRCT require complete cytoreduction before HIPEC to optimize outcome. Patients with DSRCT and disease outside the abdomen at the time of surgery do not benefit from HIPEC.

Peritoneal sarcomatosis (PSC) is defined as peritoneal involvement of multiple sarcomatous tumors. Desmoplastic small round cell tumors (DSRCT) and rhabdomyosarcomas are the most common pediatric PSC cases. PSC has been treated with chemotherapy and mainly palliative surgery, but long-term outcome has been poor. New imaging technologies have improved the evaluation of disease extent and patterns of peritoneal dissemination, and cytoreductive surgery followed by hyperthermic intraperitoneal chemotherapy (HIPEC) is being evaluated as a treatment option to prolong remission in pediatric patients. We will review the clinical characteristics, potential biologic mechanisms, radiographic characteristics, and potential therapies for pediatric PSC patients.

There is scarcity of data regarding invasive mold infections (IMIs) in children with cancer.We retrospectively identified patients (18 years old or younger) with malignant disease who developed proven or probable IMIs (European Organization for Research on the Treatment of Cancer/Mycoses Study Group criteria) during a 10-year period (1998-2008). We reviewed their risk factors and clinical characteristics and assessed their crude mortality rates and treatment outcomes 12 weeks after IMI diagnosis.Forty-eight patients (30 males) were identified, 30 (63%) of whom had a proven IMI. The most prevalent mold were Aspergillus species (40%), followed by Mucorales (20%) and Fusarium species (11%). Acute leukemia was the most common underlying malignancy (39 patients, [81%]). Twenty-three (59%) of them had refractory leukemia. Neutropenia was present at the day of IMI diagnosis in 67% of the patients. Sixty-two percent of the patients received prior corticosteroids. The dominant site of infection was the lungs (79%), followed by skin (29%) and sinuses (10%). Seventy-one percent of patients had radiological findings suggestive of fungal pneumonia (either nodules or masses). The mainstay of antifungal therapy was a lipid formulation of amphotericin B. Antifungal therapy resulted in 54% response rate (33% complete) at 12 weeks. The crude 12-week mortality rate was 31%. Logistic regression analysis demonstrated that monocytopenia (P = .013), malnutrition (P = .012), and intensive care admission in the month prior to IMI diagnosis (P = .027) were risk factors for death within 12 weeks.Although Aspergillus spp. was the most common mold in our pediatric cancer population, the epidemiology of the IMIs was diverse. Adults and children share similar risk factors for and epidemiology of IMIs.

The risks and benefits of using computed tomography (CT) as opposed to another imaging modality to accomplish a particular clinical goal should be weighed carefully. To accurately assess radiation risks and keep radiation doses as low as reasonably achievable, radiologists must be knowledgeable about the doses delivered during various types of CT studies performed at their institutions. The authors of this article propose a process improvement approach that includes the estimation of effective radiation dose levels, formulation of dose reduction goals, modification of acquisition protocols, assessment of effects on image quality, and implementation of changes necessary to ensure quality. A first step toward developing informed radiation dose reduction goals is to become familiar with the radiation dose values and radiation-associated health risks reported in the literature. Next, to determine the baseline dose values for a CT study at a particular institution, dose data can be collected from the CT scanners, interpreted, tabulated, and graphed. CT protocols can be modified to reduce overall effective dose by using techniques such as automated exposure control and iterative reconstruction, as well as by decreasing the number of scanning phases, increasing the section thickness, and adjusting the peak voltage (kVp setting), tube current-time product (milliampere-seconds), and pitch. Last, PDSA (plan, do, study, act) cycles can be established to detect and minimize negative effects of dose reduction methods on image quality.

To determine if the location of the point of maximum standardized uptake value (SUVmax) being included in or not included in the histopathologic slab section corresponded to tumor necrosis or survival.Twenty-nine osteosarcoma patients underwent post-chemotherapy [fluorine-18]-fluoro-2-deoxy-D: -glucose (FDG) positron-emission tomography-computed tomography (PET/CT) prior to resection. PET/CT images were correlated with slab-section location as determined by photographs or knowledge of specimen processing. The location of the point of SUVmax was then assigned as being 'in' or 'out' of the slab section. Cox's proportional hazard regression was used to evaluate relationships between the location and value of SUVmax and survival. Logistic regression was employed to evaluate tumor necrosis.No correlation was found between the SUVmax location and survival or tumor necrosis. High SUVmax correlated to poor survival.High SUVmax value correlated to poor survival. Minimal viable tumor (10%) following chemotherapy is a known indicator of poor survival. No correlation was found between the location of SUVmax and survival or tumor necrosis. Therefore, the SUVmax value either does not correspond to a sufficient number of tumor cells to influence tumor necrosis measurement or it was included in the out-of-slab samples that were directed to viable-appearing areas of the gross specimen. Since high SUVmax has been previously found to correspond to poor tumor necrosis, and tumor necrosis is simply an estimate of the amount of viable tumor, SUVmax likely represents many viable tumor cells. Therefore, when not in the slab section, SUVmax was likely included in the tumor necrosis measurement through directed sampling, validating our current method of osteosarcoma specimen analysis.

Background Hemophagocytic lymphohistiocytosis (HLH) is a nonmalignant disorder of immune regulation, with overproduction of cytokines and diminished immune surveillance. Symptoms are nonspecific and may affect multiple organs, including the central nervous system. Neuroimaging findings have been described in case reports and small series; body imaging findings have not been described extensively. Objective To summarize findings of the most frequently performed imaging studies of the brain, chest and abdomen in patients with HLH. Materials and methods Retrospective review of chest radiographs and CT, abdominal ultrasound and CT, brain CT and MRI, skeletal surveys, and autopsy data. Results Twenty-five patients were diagnosed and treated for HLH at our institution over an 11-year period; 15 patients (60%) died. Common chest radiograph findings included alveolar-interstitial opacities with pleural effusions, often with rapid evolution and resolution. Hepatosplenomegaly, gallbladder wall thickening, hyperechoic kidneys and ascites were common abdominal findings, which resolved after therapy in some cases. Brain-imaging studies revealed nonspecific periventricular white-matter abnormalities, brain-volume loss and enlargement of extra-axial fluid spaces. Three infant cases, one with intracranial hemorrhage, one with multiple pathologic rib fractures and one with diaphyseal periosteal reaction involving multiple long bones on skeletal survey, raised suspicion of child abuse at presentation. Abuse was not substantiated in any case. Conclusions Clinicians and radiologists should be aware of the radiographic manifestations of HLH, which are nonspecific and overlap with infectious, inflammatory and neoplastic disorders. Findings in the chest (similar to acute respiratory distress syndrome) and abdomen may progress rapidly and then regress with institution of appropriate anti-HLH therapy. CNS findings may be progressive. In some infants, initial imaging findings may mimic nonaccidental trauma.

Objective. To determine the frequency of thymic involvement in Langerhans' cell histiocytosis and describe its imaging appearance before and after treatment. Materials and methods. A retrospective review of the radiology and clinical records of all patients diagnosed with Langerhans' cell histiocytosis at DeVos Children's Hospital between 1992 and 1998. Results. Of the 14 patients diagnosed with Langerhans' cell histiocytosis, 7 demonstrated multisystem involvement. The thymus was involved in 5 of 7 patients. The thymus was enlarged in 5; thymic contours were nodular/lobulated in 2; cystic changes were noted in 4; and dystrophic vascular-appearing calcifications were seen in 1. In all cases, findings regressed or resolved following chemotherapy. Conclusion. The thymus is commonly involved in Langerhans' cell histiocytosis, especially in multisystem disease. Radiologically, the thymus is enlarged and may be smooth or lobulated/nodular in contour, possibly containing cysts and calcifications.

L.S. Medina, K.E. Applegate, and C.C. Blackmore, eds. New York, NY: Springer, 2010, 665 pages. $79.95 This is a book that will appeal to an audience of radiologists, pediatricians, primary care physicians, residents and fellows, midlevel providers, and others in the fields of diagnostic radiology

Purpose: To share our experience implementing new strategies (as technology became available) to reduce Computed Tomography(CT) radiation dose to pediatric patients at a high‐volume cancer center housing 13 General Electric (GE) multidetector row CT (MDCT) scanners representing various models.Method and Materials: In October of 2008, tube current modulation (TCM) was implemented and protocols were created based on the display field of view parameter. Approximately 10 months later, the noise index for all protocols was increased by 2. Three months after increasing the noise index, the minimum and maximum tube current (mA) values were both dropped by 20% across all protocols. A chart review was used to identify eight pediatric patients (from 1 – 15 years old) that received chest CT scans before and after implementation of each of the three dose‐reduction strategies; within each patient, all technical parameters other than effective mAs were constant between scans. Results: The average effective mAs (across all eight patients) dropped from 103 mAs (49 – 171 mAs) prior to October 2008 to 75 mAs (42 – 109 mAs) after implementing all of three strategies. TCM alone reduced dose by an average of 19 %, increasing the noise index combined with TCM by 20% on average, and dropping the minimum and maximum mA values combined with the other two strategies by an average of 23%. All of the exams included in this study were of diagnostic quality with respect to the imaging task. Conclusion: To date, the combination of these efforts has led to an average reduction in dose of 23%. Additional approaches (adaptive statistical iterative reconstruction and organ shielding) are currently being evaluated and are expected to provide even greater dose‐savings to our pediatric patients.

F. Quattromani, G.A. Handal, and R. Lampe, eds. New York, NY: Thieme, 2008, 436 pages, $49.95 Pediatric Imaging: Rapid-Fire Questions and Answers has a stated purpose of providing “a comprehensive overview of common and rare problems in pediatric radiology” and a “thorough preparation for

Pediatric Radiology Review, written for an audience of nonradiologists (medical students, residents, and clinicians) involved in the medical care of children, consists of 6 sections (chest, lateral neck, abdominal, skull, and long-bone extremity radiographs and head CT). The stated purpose of the