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Purpose While pancreaticoduodenectomy (PD) has been extensively studied in adults, there are few data pertaining specifically to pediatric patients. We retrospectively analyzed PD-associated morbidity and mortality in pediatric patients. Methods Our analytic cohort included all consecutive patients ≤18years of age treated at our institution from 1993 to 2015 who underwent PD. Patient data (demographics, disease characteristics, surgical and adjuvant treatment, length of hospital stay, and postoperative course) were extracted from the medical records. Results We identified 12 children with a median age of 9years (7 female, 5 male). Final diagnoses were pancreatoblastoma (n=3), solid pseudopapillary tumor (n=3), neuroblastoma (n=2), rhabdomyosarcoma (n=2), and neuroendocrine carcinoma (n=2). Four patients underwent PD for resection of recurrent disease. 75% (9/12 patients) received neoadjuvant therapy. The median operative time was approximately 7hours with a mean blood loss of 590cm 3 . The distal pancreas was invaginated into the posterior stomach (n=3) or into the jejunum (n=5) or was directly sewn to the jejunal mucosa (n=4). There were no operative deaths. There were 4 patients (34%) with grade II complications, 1 with a grade IIIb complication (chest tube), and 1 with a grade IV complication (reexploration). The most common long-term morbidity was pancreas exocrine supplementation (n=10; 83%). Five patients (42%) diagnosed with either solid pseudopapillary tumor or rhabdomyosarcoma are currently alive with a mean survival of 77.4months. Conclusion Pancreaticoduodenectomy is a feasible management strategy for pediatric pancreatic malignancies and is associated with acceptable morbidity and overall survival. Long-term outcome is mostly dependent on histology of the tumor. Level of evidence Level IV; retrospective study with no comparison group.

Background Although nephrectomy rates are higher in children with neuroblastoma who have image-defined risk factors and/or high-risk disease who undergo resection prior to chemotherapy, no published data outline the key radiographic and clinical characteristics associated with nephrectomy. Methods With IRB approval, imaging studies of children undergoing primary resection of intraabdominal neuroblastoma between 2000 and 2014 were retrospectively reviewed. Fisher's exact and Wilcoxon rank-sum tests were used to compare categorical and continuous variables, respectively, with p-values adjusted for multiple testing using the false discovery rate approach. Results Twenty-seven of 380 consecutive patients with CT imaging obtained prior to primary neuroblastoma resection underwent partial or total nephrectomy. On preoperative imaging, renal vessel narrowing and encasement and tumor invasion of the renal hilum, pelvis, and/or parenchyma were present significantly more frequently among patients undergoing nephrectomy. Delayed renal excretion of contrast, hydronephrosis, and tumors with MYCN amplification were also more prevalent in the nephrectomy group. Conclusion Encasement and narrowing of renal vessels, delayed excretion, and tumor invasion into the kidney, particularly pelvis and capsule invasion, are significantly associated with partial or total nephrectomy at initial neuroblastoma resection. These observations provide valuable information for surgical planning as well as presurgical discussions with families prior to neuroblastoma resection.

Introduction Retroperitoneal partial nephrectomy has not been studied as a surgical approach for children with bilateral Wilms tumor. There are advantages to this technique, including isolation of urine leaks to the retroperitoneum, decreased risk of bowel injury, and decreased time to resuming a diet. Presently, all bilateral Wilms tumors are treated with neoadjuvant chemotherapy and attempted nephron-sparing surgery. In this study, we compare the outcomes of the retroperitoneal and transabdominal approaches in doing partial nephrectomy for bilateral Wilms tumor. Methods With the institutional review board approval, we reviewed records of 14 pediatric patients with metachronous or synchronous bilateral Wilms tumors who underwent surgery after chemotherapy between 1994 and 2014. Only operative procedures with the intent to cure were included (n = 15) and of these, 5 procedures were retroperitoneal and 10 were transabdominal in approach. Individual kidneys operated upon (n = 26) were analyzed using the preoperative radius exophytic/endophytic nearness anterior/posterior location nephrometry score to ensure that resected tumors were comparable between the two surgical groups. Charts were retrospectively analyzed for intraoperative parameters and postoperative course. Differences between parameters were evaluated using Mann-Whitney and chi-square tests. Results Resected tumors in both surgical treatment groups had comparable sizes, nephrometry scores, and rates of anaplasia. Operative time, blood loss, and transfusion requirement were similar between the two groups. The extent of lymph node sampling and rates of R0 resection were equivalent. One adverse intraoperative event, a bowel enterotomy, was seen in the transabdominal group. Patients after retroperitoneal partial nephrectomy required half the time to return to an oral diet as compared with those after a transabdominal surgery, approaching statistical significance (p = 0.08). Rates of the postoperative urine leak were similar, though two in the transabdominal group required reoperation for drainage. There were four recurrences, all in the transabdominal group. Conclusion Our experience demonstrates that the retroperitoneal approach is equivalent to the transabdominal technique with regards to intraoperative complications, lymph node dissection, and R0 resection. Advantages include less time to resumption of oral feeding, decreased risk of bowel injury, and isolation of urine leaks to the retroperitoneum. It should be considered a viable surgical option in the treatment of bilateral Wilms tumors.

Desmoplastic small round cell tumour (DSRCT) is a rare malignant neoplasm. Its radiological features have rarely been described.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.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.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.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.

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.

PurposeTo determine if selected computed tomography (CT) characteristics of pulmonary nodules in pediatric patients with osteosarcoma can help distinguish the nodules as benign or malignant.MethodsThe institutional review board approved this HIPAA (Health Insurance Portability and Accountability Act-compliant, retrospective study of 30 pediatric osteosarcoma patients (median age 14 years, range 8-22) who underwent chest CT with resection of 117 pulmonary nodules from January 2001 to December 2006. Two pediatric radiologists and one chest radiologist independently and retrospectively reviewed the CT scans and classified nodules as benign, malignant, or indeterminate on the basis of nodule size, laterality, number, location, growth, density, margin appearance, and calcification. Generalized estimating equations were used to examine which characteristics were independent predictors of nodule malignancy.ResultsOf the 117 nodules, 80 (68%) were malignant and 37 (32%) were benign by pathologic review. The readers correctly classified 93% to 94% of the malignant nodules. For benign lesions, the results were not as accurate, with the readers correctly classifying only 11% to 30% of lesions. Most of the benign lesions were classified as indeterminate by the readers (54%-65%). Nodule size (≥5 mm) and the presence of calcifications were associated with an increased probability of malignancy (P < .05).ConclusionOn chest CT, nodule size 5 mm or greater and the presence of calcifications are associated with an increased probability of malignant nodule histology in pediatric patients with osteosarcoma. However, nodule characteristics, apart from size and calcification, at chest CT cannot reliably distinguish benign from malignant pulmonary nodules in these patients.

Background/purpose Image-defined vessel encasement is a significant risk factor for surgical complications and incomplete resection for intermediate-risk tumors. We sought to examine the impact of vessel encasement on complications or resectability in intermediate-risk or high-risk patients after neoadjuvant chemotherapy. Methods We retrospectively reviewed 207 consecutive patients with circumferential encasement of the renal vessels, celiac axis, and/or superior mesenteric artery (SMA) who underwent resection between 1991 and 2009. Specifically, we evaluated resection rates, complications, and outcome. Results Median age at diagnosis was 3.0 years, and 79% of patients had stage 4 disease. Of known MYCN status, 23.4% had MYCN amplification. Vessel encasement included renal vessels, celiac axis, or SMA alone in 107, 7, and 4 patients, respectively. Both the renal vessels and celiac axis were encased in 5 patients, renal vessels and SMA in 7 patients, and celiac axis and SMA in 14 patients. Sixty-three patients had all 3 vessels encased. The gross total resection (GTR) rate was 94%. No operative or postoperative deaths occurred. The overall complication rate was 34.8% (n = 72). Overall 5-year survival (± SEM) was 67.4% (± 7.4%). Conclusion Encasement of major visceral arteries in patients with neuroblastoma who have received chemotherapy does not preclude gross total resection.

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. To describe the MR imaging findings of FNH in pediatric cancer patients. 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. 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. FNH has a variable MR appearance in pediatric cancer patients and should be considered in the differential diagnosis of hypervascular liver lesions.

BACKGROUND: This study evaluated the safety and feasibility of the addition of pamidronate to chemotherapy for treatment of osteosarcoma. METHODS: The authors treated 40 patients with osteosarcoma with cisplatin, doxorubicin, and methotrexate with the addition of pamidronate 2 mg/kg/dose (max dose 90 mg) monthly for 12 doses. Survival, event-free survival (EFS), and durability of orthopedic reconstruction were evaluated. RESULTS: For patients with localized disease, event-free survival (EFS) at 5 years was 72% and overall survival 93%. For patients with metastatic disease, EFS at 5 years was 45% and overall survival 64%. Toxicity was similar to patients treated with chemotherapy alone. Thirteen of 14 uncemented implants demonstrated successful osteointegration. Among allograft reconstructions, there were 2 graft failures, 4 delayed unions, and 6 successful grafts. Overall, 5 of 33 reconstructions failed. There were no stress fractures or growth disturbances. CONCLUSIONS: Pamidronate can be safely incorporated with chemotherapy for the treatment of osteosarcoma. It does not impair the efficacy of chemotherapy. Pamidronate may improve the durability of limb reconstruction. Cancer 2011. © 2010 American Cancer Society.

Survival in osteosarcoma correlates with complete resection of primary and metastatic disease. The feasibility of complete pulmonary metastasectomy using thoracoscopy has been raised. Because palpation is not possible, minimally invasive techniques require preoperative radiological enumeration and localization of metastases not presenting at the lung surface. We hypothesized that computed tomographic (CT) scanning underestimated the number of pulmonary metastases in these patients.Institutional review board approval was obtained. We determined the association between the number of lesions identified by CT scanning and the number of metastases found at thoracotomies for metastatic osteosarcoma from May 1996 to October 2004. Correlations between CT findings and pathology results were computed using the Kendall tau-b correlation coefficient. Depth, in millimeters, from the pleural surface was measured for those lesions seen on CT scan.We analyzed 54 consecutive thoracotomies performed in 28 patients for whom complete imaging was available. Computed tomographic scanning was performed a median of 20 days before thoracotomy (range, 1-85 days). Correlation between the number of lesions identified by CT and the number of metastases resected at surgery was poor, with a Kendall tau-b correlation coefficient of 0.45 (P.001). In 19 (35%) of 54 thoracotomies, CT scanning underestimated the number of pathologically proven, viable and nonviable metastases found by the surgeon. Accounting for viable metastases only, correlation between the number of lesions identified by CT and the number of metastases resected at surgery was 0.50 (P.001), and CT scanning underestimated the number of viable metastases present in 14 (26%) of 54 thoracotomies. Many lesions (32%) were pleural-based, but nearly half (47%) were 5 mm or deeper from the pleural surface of the lung.Even in the era of modern CT scanning, only a very rough correlation exists between CT findings and the number of lesions identified at thoracotomy. In more than one third of thoracotomies in our series, metastases would have been missed by any tactic besides manual palpation of the lung during open thoracotomy. Minimal access procedures should not be the approach of choice if the goal is resection of all pulmonary metastases in osteosarcoma.

Purpose The surgical management of osteosarcoma patients with unilateral pulmonary nodules is controversial. The authors reviewed their institutional experience to evaluate the incidence of occult contralateral metastases. Methods Data were obtained retrospectively on all consecutive osteosarcoma patients from 1980 to 2002. Eighty-four patients with pulmonary nodules were identified. Forty-one had bilateral disease, and 43 had unilateral involvement by computed tomography (CT) scan. Results All 43 patients with unilateral nodules underwent ipsilateral thoracotomies. Fifteen patients had negative exploration findings, and only 1 had pulmonary relapse. Of the 28 patients with metastases confirmed at initial thoracotomies, 14 had extensive pleural or extrapulmonary disease at initial thoracotomy followed by disease progression. The other 14 are separated into early versus late metastases, using 2 years from diagnosis as the cutoff point. Seven of the 9 (78%) patients with early metastases had or subsequently had contralateral disease; 6 were identified at staged contralateral thoracotomy and 1 had relapsed in the unexplored lung a year later. Only 1 of the 5 patients with late unilateral metastases had relapse in the contralateral side. Conclusions Our data indicate that there is a high rate of contralateral involvement in osteosarcoma patients with unilateral nodules diagnosed by CT scan. Staged bilateral thoracotomies should be considered in osteosarcoma patients presenting with unilateral pulmonary disease on imaging studies within 2 years of diagnosis.

Gross total resection of the primary tumor in treatment of high-risk neuroblastoma remains controversial. Furthermore, there are few reports of the effect of primary tumor resection on local control as opposed to overall survival. The authors reviewed their institutional experience to assess the effect of primary tumor resection on local control and overall survival.A total of 141 patients were treated on protocol between November 1, 1979 and June 25, 2002 and are the subject of this report. Gross total resection was assessed by review of operative notes, postoperative computerized axial tomograms, and postoperative meta-iodobenzyl guanidine (MIBG)1 scans when available.The median age was 3.3 years, and all patients were International Neuroblastoma Staging System (INSS) stage 4 with 79% having metastases to cortical bone. The primary site was the adrenal gland in 74%, the central abdominal compartment in 13%, the posterior mediastinum in 7%, and other sites in 6%. Gross total resection was accomplished in 103 (73%) but was more than 90% for the last 3 protocols. Five kidneys were lost overall. The probability of local progression was 50% in unresected patients compared with 10% in patients undergoing gross total resection (P.01). Overall survival rate in resected patients was 50% compared with 11% in unresected patients (P.01).Our data indicate that local control and overall survival rate are correlated with gross total resection of the primary tumor in high-risk neuroblastoma. Gross total resection should be part of the management of stage 4 neuroblastoma in patients greater than 1 year of age.

Background The N7 protocol for poor-risk neuroblastoma uses dose-intensive chemotherapy (as in N6 protocol [Kushner et al.: J Clin Oncol 12:2607–2613, 1994] but with lower dosing of vincristine) for induction, surgical resection and 2100 cGy hyperfractionated radiotherapy for local control, and for consolidation, targeted radioimmunotherapy with 131I-labeled anti-GD2 3F8 monoclonal antibody and immunotherapy with unlabeled/unmodified 3F8 (400 mg/m2). Procedure The chemotherapy consists of: cyclophosphamide 70 mg/kg/d × 2 and a 72-hr infusion of doxorubicin 75 mg/m2 plus vincristine 2 mg/m2, for courses 1, 2, 4, and 6; and cisplatin 50 mg/m2/d × 4 and etoposide 200 mg/m2/d × 3, for courses 3, 5, and 7. 131I-3F8 is dosed at 20 mCi/kg, which is myeloablative and therefore necessitates stem-cell support. Results Of the first 24 consecutive previously untreated patients more than 1 year old at diagnosis, 22 were stage 4 and two were unresectable stage 3 with MYCN amplification. Chemotherapy achieved CR/VGPR in 21 of 24 patients. Twenty patients to date have completed treatment with 131I-3F8, and 15 patients have completed all treatment. With a median follow-up of 19 months, 18 of 24 patients remain progression-free. Conclusions Major toxicities were grade 4 myelosuppression and mucositis during chemotherapy, and self-limited pain and urticaria during antibody treatment. Late effects include hearing deficits and hypothyroidism. Med. Pediatr. Oncol. 36:227–230, 2001. © 2001 Wiley-Liss, Inc.

Purpose: To evaluate the efficacy of 21 Gy hyperfractionated radiotherapy for local control in conjunction with surgery and intensive systemic therapy for patients with Stage 4 neuroblastoma. Methods and Materials: After achieving a partial or complete remission, 47 children, ages 1–10 years, with Stage 4 neuroblastoma were treated on four consecutive institutional protocols (N4–N7) with dose-intensive multi-agent chemotherapy, maximal surgical debulking, and hyperfractionated radiotherapy (1.5 Gy twice a day to 21 Gy). Radiotherapy fields encompassed the initial tumor volume and regional lymph nodes plus a 3-cm margin. This was followed by consolidation with either autologous bone marrow transplantation (N4 and N5) or immunotherapy (N6 and N7). Results: Forty-five of 47 patients had a complete response to surgery and chemotherapy prior to radiotherapy. Five-year actuarial rates of local control, progression-free survival, and overall survival were 84%, 40%, and 45%, respectively. Among 26 patients who relapsed, 1 failed only at the primary site, 22 developed distant metastases exclusively, and 3 had both local and distant failures. There were no acute complications of radiotherapy. Conclusion: Hyperfractionated radiotherapy to 21 Gy, in conjunction with dose-intensive systemic therapy and aggressive surgical resection, is well tolerated and is associated with durable local control for most patients with Stage 4 neuroblastoma.

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.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.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.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.

Wilms tumor (4 %) and neuroblastoma (8 %) make up the largest group of extracranial solid tumors of childhood. Most of these patients present before 5 years of age. Mesoblastic nephroma and stage IVS neuroblastoma are seen in children less than 1 year of age. Several other primary renal tumors may be seen in children. Adrenocortical carcinoma and pheochromocytoma are rare. The imaging features of these tumors will be shown. Rhabdomyosarcoma of the bladder will also be discussed.

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.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.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.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.

We evaluated HLA-compatible donor leukocyte infusions (DLIs) and HLA-compatible or HLA-disparate EBV-specific T cells (EBV-CTLs) in 49 hematopoietic cell transplantation recipients with biopsy-proven EBV-lymphoproliferative disease (EBV-LPD). DLIs and EBV-CTLs each induced durable complete or partial remissions in 73% and 68% of treated patients including 74% and 72% of patients surviving ≥ 8 days after infusion, respectively. Reversible acute GVHD occurred in recipients of DLIs (17%) but not EBV-CTLs. The probability of complete response was significantly lower among patients with multiorgan involvement. In responders, DLIs and EBV-CTLs regularly induced exponential increases in EBV-specific CTL precursor (EBV-CTLp) frequencies within 7-14 days, with subsequent clearance of EBV viremia and resolution of disease. In nonresponders, EBV-CTLps did not increase and EBV viremia persisted. Treatment failures were correlated with impaired T-cell recognition of tumor targets. Either donor-derived EBV-CTLs that had been sensitized with autologous BLCLs transformed by EBV strain B95.8 could not lyse spontaneous donor-derived EBV-transformed BLCLs expanded from the patient's blood or biopsied tumor or they failed to lyse their targets because they were selectively restricted by HLA alleles not shared by the EBV-LPD. Therefore, either unselected DLIs or EBV-specific CTLs can eradicate both untreated and Rituxan-resistant lymphomatous EBV-LPD, with failures ascribable to impaired T-cell recognition of tumor-associated viral antigens or their presenting HLA alleles.

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.To review the generalized skeletal imaging findings on computed radiography, CT, and MR examinations in patients with osteosarcoma (OS) receiving pamidronate.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.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%.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.

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.

A previously healthy 9-year-old boy presented to an outside hospital with a history of abdominal pain and vomiting. An abdominal x-ray was unremarkable. A CT of the abdomen and pelvis performed to evaluate possible obstruction after weight loss and vomiting over a 3-week period demonstrated a large retroperitoneal mass. Laparoscopic biopsy showed diffuse large B-cell lymphoma. FDG PET/CT was performed for staging. An ileocolic intussusception was identified on the PET/CT. The intussusception was successfully managed with medical treatment. We present FDG PET/CT findings in intussusception with non-Hodgkin lymphoma as the lead point in a pediatric patient.

Stage IV neuroblastoma is associated with high mortality; an exception are patients whose stage IV status includes distant positive nodes, but no skeletal metastases —stage IVN neuroblastoma. We describe our experience with preoperative MRI in three patients with extensive abdominal neuroblastoma without cortical bony involvement but with unsuspected metastatic involvement to the left supraclavicular (Virchow's) node. We review findings of left supraclavicular nodal spread in five earlier cases of IVN neuroblastoma.

Background/Purpose : Although there has been a precedent of testicular-sparing surgery in some centers, the authors find it is still not general practice among pediatric surgeons. To address this and emphasize the role of testicular-sparing surgery in children, four patients with testicular masses are presented who underwent this procedure. Methods : Four patients who underwent testicular-sparing surgery between the years 1993 and 1998 were reviewed. Demographic data, histopathology, and follow-up data were obtained from office charts. The period of follow-up ranged from 1 to 5 years. Results : Four patients whose ages at diagnosis were 1, 2, 4, and 17 years presented with unilateral testicular masses. The alpha-fetoprotein and β-human chorionic gonadotropin levels were within normal limits. Testicular ultrasonography was carried out on all patients, and groin exploration with spermatic cord isolation was performed in each case. After enucleation, frozen sections to confirm benignity was carried out before repair of the testis. Follow-up of 6 months to 5 years has shown no recurrence, and on examination, testicular volume is normal in all cases. Conclusions : Testicular-sparing surgery preserves testicular volume, which is important for both cosmetic and functional purposes. It is a viable and useful method in the management of benign testicular tumors in children.

PURPOSE To improve response and survival rates in patients with high-risk rhabdomyosarcoma (RMS), extraosseous Ewing's sarcoma, and undifferentiated sarcoma, we used a short course of induction with multi-agent chemotherapy, hyperfractionated radiotherapy, and surgery when possible. Consolidation was with intensive chemotherapy and autologous bone marrow transplantation (ABMT). PATIENTS AND METHODS Twenty-six patients (21 with RMS, three with undifferentiated sarcoma, and two with extraosseous Ewing's sarcoma) were entered onto the protocol between June 1990 and March 1994. Induction consisted of ifosfamide, etoposide, doxorubicin, dactinomycin, cyclophosphomide, and vincristine, and a split course of hyperfractionated radiotherapy. Patients who attained a complete response (CR) or good partial response (GPR) received consolidation with high-dose melphalan and etoposide followed by ABMT. RESULTS Of 26 previously untreated patients 19 (73%) achieved a CR (n=13) or GPR (n=6) at the completion of induction and underwent ABMT. Two-year overall survival (OS) was 56% (95% confidence interval [CI], 36% to 76%) and progression-free survival (PFS) was 53% for the whole group (95% CI, 33% to 73%). CONCLUSION Consolidation of response by myeloablative chemotherapy was well tolerated. Split-course hyperfractionated radiotherapy did not increase the rate of local control. The results of this short-course therapy were comparable to previous therapies of 1 to 2 years' duration. Induction and consolidation chemotherapy, as well as radiation dose, could be further intensified, since no death due to toxicity occurred among these patients.

Purpose. This paper describes the clinical history and radiographic appearance of second malignancies in patients with bilateral retinoblastoma. Subjects/methods. The imaging studies and clinical data of 14 patients with a history of bilateral retinoblastoma who were treated for second malignancies were reviewed. Results. A total of 17 tumors were identified in 14 patients during the period 1978–1996. The median age of occurrence of the second malignancy was 17 years (range 10–32 years). Fourteen of the 17 malignancies occurred in the facial structures and three developed in the lower extremities. The histologies included osteosarcoma (n = 5), malignant fibrous histiocytoma (n = 3), high-grade spindle cell sarcoma (n = 3), malignant mesenchymoma (n = 1), leiomyosarcoma (n = 4) and angiosarcoma (n = 1). The tumors were locally aggressive and had a similar appearance to those found in nonretinoblastoma patients. Six of the 14 patients are alive and disease free. Discussion. Most of the adolescent and young adult retinoblastoma survivors developed second malignancies in the irradiated facial structures but some occurred in distal sites. Radiologically, these tumors do not differ in appearance from those seen in non-retinoblastoma patients with the exception of their location.

Epstein-Barr virus (EBV)-associated smooth muscle tumors or leiomyosarcomas (EBV LMS) are extremely rare. These tumors develop in patients with acquired or underlying immune deficiency including those after solid organ transplant (SOT) and exhibit a latency type II phenotype. EBV LMSs are typically aggressive and respond poorly to conventional therapy. As part of an on-going trial of EBV specific cytotoxic T lymphocytes for the treatment of EBV related malignancies we have treated 5 patients with EBV LMS with cytotoxic T cell lines generated from normal donors. Five patients, three after SOT (kidney N=1, small bowel N=2), one after solid organ (kidney) and hematopoetic stem cell transplant, and one with a poorly defined underlying immune deficiency syndrome developed EBV LMS. Two patients were successfully treated for EBV lymphoproliferative disease prior to developing EBV LMS. EBV LMS was diagnosed 18 – 46 months after SOT in 4 patients, at 9 years old in the fifth and were multifocal at diagnosis in all 5 patients. All four patients who had undergone SOT failed to respond to decreased immune suppression, one had failed treatment with autologous EBV CTLs and two had failed cytotoxic chemotherapy. The EBV LMS was of host origin in 4/5 patients and of mixed donor and host origin in one. Lines of EBV CTLs were generated as previously described by co-culture of donor peripheral blood T lymphocytes with autologous EBV transformed BLCLs. Lines are tested for EBV specific cytotoxicity, sterility and absence of alloreactivity. Patients with EBV LMS were treated with cell lines generated from unrelated as well as related donors. Three patients received EBV CTLs generated from related haplo-identical parent donors including one whose parental donor was also the donor of her transplanted kidney. One patient received EBV CTLs from an HLA identical related donor. All other lines were generated from 3rd parties. Lines from unrelated third party donors were matched at 2 – 4/8 HLA alleles. In all instances EBV directed cytotoxicity of infused lines was restricted by HLA alleles shared by the patient and the EBV LMS. Three patients achieved partial remissions (PR) lasting 18 – 122 months and one patient has had stable disease for >80 months. One patient had stable disease for 14 months before progression. Expansion of EBV CTLp was demonstrated in all patients after infusion of EBV CTLs lasting from 0.5 to 4 months. Based on the prediction that 3rd party partially matched EBV CTLs are unlikely to persist long term in vivo four of the patients have received multiple cycles of EBV CTLs in an effort to maintain these responses. EBV directed cytotoxic T cell therapy is a novel treatment strategy for EBV LMS, a malignancy that is typically associated with a very poor prognosis. EBV CTLs from immunologically normal, partially HLA-matched third party donors, appropriately specific and HLA restricted can induce significant and durable responses to EBV LMS. Approaches designed to selectively expand T-cells specific for EBV antigens expressed by these malignancies may further improve responses. Disclosures: No relevant conflicts of interest to declare.

The magnetic resonance bone marrow patterns in thalassemia were evaluated to determine changes produced by transfusion and chelation therapy. Thirteen patients had T1- and T2-weighted images of the spine, pelvis and femurs. Three received no therapy (age range 2.5-3 years). Three were hypertransfused (transfused to maintain a hemoglobin greater than 10 g/dl) and not chelated because of age (age range 6 months-8 years). Seven were hypertransfused and chelated (age range 12-35 years). Signal characteristics of marrow were compared with those of surrounding muscle and fat. Fatty marrow (isointense with subcutaneous fat) was compared with red marrow (hypointense to fat and slightly hyperintense to muscle). Marrow hypointense to muscle was identified as iron deposition within red marrow. The untreated group demonstrated signal consistent with red marrow throughout the central and peripheral skeleton. Hypertransfused but not chelated patients demonstrated marked iron deposition in the central and peripheral skeleton. Hypertransfused and chelated patients demonstrated iron deposition in the central skeleton and a mixed appearance of marrow in the peripheral skeleton. The MR appearance of marrow in thalassemia is a reflection of the patient s transfusion and chelation therapy. Iron deposition occurs despite chelation therapy in sites of active red marrow. As red marrow retreats centrally with age, so does the pattern of iron deposition. The long-term biological effects of this iron deposition are unknown.

9533 Background: There has been little improvement in outcome for patients (pts) with intermediate (INT)- and high-risk (HR) rhabdomyosarcoma (RMS) in nearly 2 decades. Methods: From November 2003 through December 2009, we enrolled 56 newly diagnosed pts with INT- (28) and HR (28) RMS on IRB 03-099, a 7-drug regimen that commenced with 2 courses of irinotecan + carboplatin (I+C) given 3 weeks apart. I (20 mg/m2 IV over 1 hour) was given on days 1-5 and 8-12; C (560 mg/m2 IV over 1 hour) was given on day 1. Results: Median age at diagnosis was 11.5 yrs (4 months – 42 years); median tumor size was 6 cm. There were 29 males and 27 females; 30 pts (15 HR) had alveolar RMS (ARMS); 24 pts (12 HR) had embryonal RMS (ERMS); one each had sclerosing RMS and pleomorphic RMS (HR RMS). Of 28 pts with INT-RMS, 26 had invasive (T2) and/or 16 large (Tb) tumors, and 15 were N1. 11 of 28 pts with HR RMS had more than 1 site of metastatic disease. Pts were restaged after 2 cycles using RECIST and WHO response criteria. 4 pt...

Although the technique for ultrasonographic evaluation of the infant hip is difficult to learn, it is a valuable diagnostic tool. In many institutions, hip ultrasonography expands upon and complements the clinical examination and minimizes radiographs.

Despite advances and refinements in diagnostic techniques, distinguishing between cholestatic syndromes and extrahepatic biliary atresia is not always possible. Because there are inherent errors in all diagnostic studies including nuclear scanning, ultrasonography, and liver biopsy some infants presumed to have biliary atresia will come to surgery when, in fact, the bile ducts are partially or completely patent. The decision to proceed with a portoenterostomy or to terminate the procedure depends upon the appearance of the liver and histology of the biopsy.

THE ASSOCIATION of rickets with prematurity has received increased attention in the past ten years because of the improved survival rates and increasing numbers of observations in very low-birth-weight infants. '-7 Calcium, phosphorus, and vitamin D deficiency have been implicated as causative factors in these infants, many of whom have illnesses associated with prolonged periods of nutritional deficiency. We recently diagnosed rickets in six VLBW infants who had been hospitalized in the Neonatal Intensive Care Unit during an eight-month period from June, 1975, to January, 1976. Four had craniotabes, one had multiple fractures, and one had rickets discovered on a chest radiograph obtained for suspected pneumonia. Subsequently a systematic search identified 16 additional cases during the next 13 months. The present report is a review of these 22 cases.

The incidence of rickets was found to be 32% (39/125) in a retrospective review of consecutive survivors of very low birth weight in whom serial radiographic and biochemical data were obtained. A higher proportion of these infants were black, had a greater initial weight loss, and had a longer hospitalization; there was a prevalence of births in the spring. Soy formula, supplemented with calcium and vitamin D but not phosphorus, was used predominantly in both groups; cumulative calcium, phosphorus, vitamin D, and caloric intakes were the same. We believe that the etiology of rickets in VLBW infants is multifactorial; however, nutritional deficiency is of central importance. Soy isolate formula, as well as human milk and many other commercially available formulas, do not provide sufficient calcium and phosphorus to keep pace with rates of intrauterine accretion. Supplementation with calcium, phosphorus, and vitamin D, beginning as soon as possible after birth, is indicated.