p53 and Ki-67 Proliferating Cell Nuclear Antigen in Benign and Malignant Peripheral Nerve Sheath Tumors in Children

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon soft tissue tumors. In children with neurofibromatosis 1 (NF1), a MPNST often arises in a pre-existing neurofibroma, or may represent an initial manifestation without other obvious stigmata of the disease. The development of MPNSTs may be associated with instability of the p53 tumor suppressor gene since it is the most frequent genetic abnormality in soft tissue sarcomas. To assess the presence of p53 accumulation in MPNSTs and its correlation with clinical and pathologic features, we studied 12 neurofibromas (NFs), including 4 tumors with cellular features (one congenital) and 10 MPNSTs. Six MPNSTs were associated with NF1, all of which developed within a plexiform neurofibroma. Cell proliferation evaluated with an antibody to Ki-67 and nuclear p53 staining were both detected by immunohistochemistry. We found p53 positivity in 60% of MPNSTs. All NFs except the congenital tumor were p53 immunonegative (P < 0.01). Rare p53-positive nuclei were detected in the transitional zone in two of six MPNSTs arising in plexiform NFs. Ki-67 distinguished the NFs from MPNSTs (P < 0.005). Half of the NF1 patients with p53-positive MPNSTs developed recurrence or metastases or developed a second malignancy within 2 years of diagnosis, whereas patients with p53-positive sporadic MPNSTs were free of disease 1 to 7 years later. We found p53 accumulation more frequently in NF1-associated MPNSTs. p53 mutations may be an additional biologic factor to account for the poor prognosis in these tumors. Received May 11, 1998; accepted August 31, 1998.     

MRI diagnosis of suspected atlanto-occipital dissociation in childhood

Objective. To demonstrate the utility of magnetic resonance (MR) imaging in the diagnosis of complete and partial ligamentous injuries in patients with suspected atlanto-occipital dissociation (AOD). Materials and methods. Five patients with suspected AOD had MR imaging performed within an average of 4 days after injury. MR scans were reviewed with specific analysis of craniocervical ligamentous structures. Charts were reviewed to obtain clinical information regarding presentation, treatment, hospital course, and outcome. Results. Two patients demonstrated MR evidence of complete AOD. One had disruption of all visualized major ligamentous structures at the craniocervical junction with anterolisthesis and evidence of cord damage. The second had injuries to the tectorial membrane, superior band of the cruciform ligament, apical ligament, and interspinous ligament at C 1–2. The remaining three patients sustained incomplete severance of the ligamentous structures at the craniocervical junction. All patients demonstrated subtle radiographic findings suggestive of AOD, including soft tissue swelling at the craniocervical junction without fracture. The two patients with complete AOD died. The three patients with partial AOD were treated with stabilization. On follow-up, these three children were asymptomatic following their craniocervical injury. Conclusion. MR imaging of acute AOD provides accurate identification of the craniocervical ligaments injured, classification of full versus partial ligamentous disruption, and analysis of accompanying spinal cord injury. This information is important for early appropriate neurosurgical management and preservation of neurologic function in survivors. Received: 11 February 1998 Accepted: 20 November 1998     

Giant malignant nerve sheath tumor of lumbosacral plexus with intraspinal extension in a child with neurofibromatosis type 1

Malignant peripheral nerve sheath tumors (MPNSTs) are the leading cause of death in young adults and are one of the most frequent non-rhabdomyosarcomatous soft tissue tumors in pediatric age. These tumors usually occur in young adults from a previously recognized neurofibroma, neurofibromatosis type 1 (NF1), with a noted change in size and pain. This child reached the age of 10 without the presence of the more commonly seen manifestations of NF1. Pseudoarthrosis in children has a high rate of association with NF1, and in this case diagnosis of NF1 was supported by development of MPNST in a pre-existing plexiform neurofibroma.     

The value of 201thallium-SPECT imaging in childhood brainstem gliomas

Objective. To compare ²⁰¹thallium (T1) uptake and SPECT with MRI in children with brainstem gliomas. Materials and methods. Ten children with brainstem gliomas were prospectively evaluated by ²⁰¹Tl-SPECT and MRI. Histological verification was obtained in eight children – two died prior to surgery. Quantitative thallium uptake index (UI) was obtainable in five cases and was compared to tumour grade. In addition, two patients with known benign brainstem lesions (neurofibromatosis and tuberculoma) were similarly prospectively evaluated. Results. All children with brainstem glioma accumulated thallium. (Mean U1 3.23, 100 % sensitivity). The single patient with brainstem tuberculoma also accumulated thallium (UI 2.80, 91.7 % specificity). There was no correlation between thallium uptake and tumour grade. Uptake could not be conclusively correlated with the following MR features: gadolinium enhancement, exophytic or intrinsic gliomas, necrosis and location of glioma within the brainstem. Conclusions. ²⁰¹Tl-SPECT is a promising imaging adjunct in the assessment of childhood brainstem gliomas. Received: 23 May 1997 Accepted: 16 January 1998     

MR imaging evaluation of subacute and chronic bone abscesses in children

Objective. The aim of the present study was to assess the value of magnetic resonance (MR) imaging in subacute and chronic bone abscesses in children. ¶Materials and methods. Seventy-four patients underwent MR imaging because of suspected musculoskeletal infections between January 1996 and January 1999 in Montreal Children's Hospital. The clinical, radiographic, scintigraphic and MR imaging features of patients with a bone abscess were studied. ¶Results. Eleven patients had osteomyelitis with no bone abscess and six had osteomyelitis with a subacute or chronic bone abscess. Although the lucency was eventually seen on plain radiographs in all cases, MR imaging made a significant contribution, as it helped narrow the differential diagnosis and showed better delineated medullary involvement and extension into the epiphysis. ¶Conclusion. MR imaging is valuable in the diagnostic evaluation of children with bone infection and abscess. It reveals the extent of subperiosteal and epiphyseal involvement not seen on plain radiographs. The extent of the medullary involvement around the abscess is best visualized with MR imaging, which can also distinguish between isolated soft tissue infection adjacent to bone and true bone infection.     

Cervicomedullary astrocytomas of childhood: clinical and imaging follow-up

Background. Cervicomedullary astrocytomas are a unique subset of brainstem tumors in children because they have a good prognosis when compared to the pontine subset of brainstem gliomas. Objective. To review the clinical and imaging findings in a series of children with cervicomedullary astrocytomas as to diagnosis and management. Materials and methods. A retrospective review of eleven children (six females, five males, age range: 10 days-18 years; mean = 7 years) with cervicomedullary tumors was done including the clinical presentation, imaging studies (MR: eleven, CT and MR: four), surgical findings, pathological results, and follow-up clinical and imaging findings (range: 0.2–11 years; mean = 5.2 years). Results. Symptoms and signs were delayed and protracted, often occurring over months to years (mean = 2.3 years, range 0.5–7 years). The tumors expanded the dorsal medulla and involved the upper cervical spinal cord (mean maximum tumor diameter = 4.4 cm). Only three patients had hydrocephalus. In three of four cases the tumor was not seen on CT. On MR, the majority of the tumors were T1 hypointense and T2 hyperintense. Treatment consisted of surgery only in six patients, surgery and radiation therapy in four, and surgery, chemotherapy, and radiation in one. There was recurrent local disease in four patients and on follow-up metastatic disease in the brain in one. On follow-up the majority of the patients are alive and stable (mean = 5.2 years, range 0.2–11 years). There has been one death. The majority of tumors were pilocytic astrocytomas. Conclusion. Cervicomedullary tumors are a unique subset of brainstem gliomas in childhood that present with a long duration of symptoms and a greater long-term survival than pontine gliomas. Received: 14 January 1999 Accepted: 22 March 1999     

Medical management of neurofibromatosis

The neurofibromatoses consist of at least three autosomal dominantly inherited disorders, neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis. For many years these conditions were inextricably linked as part of generalized neurofibromatosis (von Recklinghausen disease). Since 1987 with the separate localization of NF1 to chromosome 17q and NF2 (bilateral vestibular schwannoma) to 22q it has been possible to formally separate them. More recently the SMARCB1 gene on 22q has been confirmed as causing a subset of schwannomatosis. The last 20 years has seen a considerable improvement in our knowledge of the clinical and molecular features of these conditions. Both NF1 and NF2 provide the clinician with often complex management decisions. Childhood presentation of NF2 in particular presages a usually severe disease course.     

MR imaging of glioblastoma in children: usefulness of diffusion/perfusion-weighted MRI and MR spectroscopy

Background Glioblastoma is relatively uncommon in childhood and maybe difficult to differentiate from other brain tumors such as primitive neuroectodermal tumor, ependymoma, or benign astrocytoma. Objective To describe the characteristic MR features in children with glioblastoma and to evaluate the usefulness of diffusion and perfusion MR imaging and MR spectroscopy in pediatric glioblastoma. Materials and methods MR imaging in 11 children (12 tumors) with biopsy-proven glioblastoma was reviewed retrospectively. In one patient, there was a recurrent glioblastoma. We reviewed CT and MRI imaging for tumor location, density/signal intensity, and enhancement pattern. Routine MR imaging was performed with a 1.5-T scanner. In six patients, diffusion-weighted MR images (DWIs) were obtained with a single-shot spin echo EPI technique with two gradient steps, and apparent diffusion coefficients (ADCs) were calculated. Using the gradient EPI technique, perfusion-weighted MR images (PWIs) were obtained in four patients from the data of dynamic MR images. The maximum relative cerebral blood volume (rCBV) ratio was calculated between the tumor and contralateral white matter in two cases. In three patients, proton MR spectroscopy was performed using a single voxel technique with either STEAM or PRESS sequences. The locations of the tumor were the thalamus and basal ganglia (n=8), deep white matter (n=3), and brain stem (n=1).Results Intratumoral hemorrhage was seen in four tumors. The tumors showed high-signal intensity or DWIs, having a wide range of ADC values of 0.53–1.30 (mean ±SD=1.011±0.29). The maximum rCBV ratios of glioblastoma were 10.2 and 8.5 in two cases. MR spectroscopy showed decreased N-acetylaspartate (NAA) and increased choline in three cases. The MR findings of glioblastoma in children were: a diffusely infiltrative mass with hemorrhage involving the deep cerebral white matter, thalami, and basal ganglia. Conclusion Diffusion/perfusion MR imaging and MR spectroscopy are very helpful in diagnosing glioblastoma, determining the biopsy site, and evaluating tumor recurrence.This paper was presented as a scientific contribution at the 39th Annual Congress of the European Society of Paediatric Radiology, Bergen, Norway, June 2002     

MR imaging of abdominopelvic involvement in neurofibromatosis type 1: a review of 43 patients

Background: Plexiform neurofibromas are a frequent complication of neurofibromatosis type 1. This article discusses MR imaging findings and distribution of plexiform neurofibromas in the abdomen and pelvis. Objective: To define the most prevalent patterns of involvement and MR imaging findings in abdominopelvic neurofibromatosis type 1. Materials and methods: We reviewed the MR appearance of abdominopelvic lesions in 23 male and 20 female patients (median age: 16 years) with type 1 neurofibromatosis. The patients were part of a multi-institutional study of 300 patients. Imaging included coronal or sagittal, and axial short tau inversion recovery images. Results: The most common abdominopelvic involvement was in the abdominopelvic wall (n=28, 65%) and lumbosacral plexus (n=27, 63%). Retroperitoneal involvement was frequent (n=15, 35%). Lesions were less often intraperitoneal (21%) (P=0.001). Pelvic disease (n=27, 63%), neural canal involvement (n=18, 42%), and hydronephrosis (n=4, 9%) were also noted. Target-like appearance of plexiform lesions was noted in more than half the patients. Conclusion: Abdominopelvic involvement in neurofibromatosis type 1 is primarily extraperitoneal. Although lesions are most prevalent in the abdominopelvic wall and lumbosacral plexus, retroperitoneal and pelvic involvement is common and usually affects important organs. MR imaging added information in the initial and follow-up clinical evaluation of these patients.     

Neurofibromatosis type 1 and optic pathway glioma: Molecular interplay and therapeutic insights

Children with neurofibromatosis type 1 (NF1) are predisposed to develop central nervous system neoplasms, the most common of which are low‐grade gliomas (LGGs). The absence of human NF1 associated LGG‐derived cell lines, coupled with an inability to generate patient‐derived xenograft models, represents barriers to profile molecularly targeted therapies for these tumors. Thus, genetically engineered mouse models have been identified to evaluate the interplay between Nf1‐deficient tumor cells and nonneoplastic stromal cells to evaluate potential therapies for these neoplasms. Future treatments might also consider targeting the nonneoplastic cells in NF1–LGGs to reduce tumor growth and neurologic morbidity in affected children.     

Accuracy of MR imaging for detecting epiphyseal extension of osteosarcoma

Background. Too few patients are receiving epiphyseal-sparing limb salvage procedures for osteosarcoma. Objective. To determine how magnetic resonance (MR) imaging can best predict the epiphyseal extension of osteosarcoma. Materials and methods. Forty children underwent complete pretreatment static and dynamic contrast-enhanced MR imaging (DEMRI). Static MR images [T1-weighted and short tau inversion recovery (STIR)] of the epiphyses were read in three ways: (1) for suspicion of any abnormality (tumor or edema), (2) for suspicion of tumor, excluding suspected edema, and (3) validating the second method by using a scale to rate the likelihood of tumor. Presentation imaging was compared to histopathologic findings after chemotherapy and resection. The receiver operating characteristic (ROC) method was used to analyze the scaled ratings of static MR and DEMRI values. Results. At delayed resection, 20 of 40 children with osteosarcoma had confirmed epiphyseal tumor; however, 32 epiphyses were abnormal on STIR and 28 abnormal on T1. Differentiating suspected tumor from edema increased the accuracy to an A_z (area under the ROC curve) of 0.94 for both T1-weighted and STIR static sequences. T1-weighted MR had better specificity and STIR better sensitivity at any given rating. DEMRI was slightly less accurate (Az = 0.90). Conclusion. Static MR imaging most accurately detected epiphyseal extension of osteosarcoma when readers distinguished suspected tumor from edematous or normal tissue. Received: 20 August 1999 Accepted: 13 January 2000     

When do children with optic pathway tumours need treatment? An oncological perspective in 106 patients treated in a single centre

Progression patterns of optic pathway tumours (OPT) need to be precisely defined for treatment planning. In patients with neurofibromatosis type 1 (NF1), this disease is usually indolent and the available literature rarely reports progression after the age of 6 years. In patients without NF1, the disease course seems to be less favourable. We reviewed the clinical and radiological files of 106 children referred to our institution for the treatment of a symptomatic OPT since 1980. NF1 was present in 51 of them. Progression patterns in children with NF1 differed markedly from those in the other patients. A total of 83 children had tumour extension beyond the chiasm (Dodge type III). Children with NF1 had progressive tumours later during follow-up (47% after the age of 6 years), had more often proptosis and infiltrating tumours but less frequently nystagmus or increased intracranial pressure. 32 children were not treated at diagnosis because they had only mild symptoms related to the OPT. In these patients, progression occurred more often in children without than with NF1 (12/12 versus 12/20 respectively, P=0.04). A high number of patients needed treatment for progression or severe symptoms after 6 years of age. Of the patients, 33% needed treatment for progression or severe symptoms after 6 years of age. Conclusion Progression patterns of optic pathway tumours in children with neurofibromatosis type 1 differ markedly from those in other patients. This study emphasises the need for prolonged follow-up of children with optic pathway tumours, especially in neurofibromatosis type 1. Received: 1 December 1999 / Accepted: 29 March 2000     

Role of MRI in the management of children with diffuse pontine tumors: a study of 15 patients and review of the literature

Background: Pontine tumors carry the worst prognosis of all brain tumors. In most cases, the diagnosis is based solely on MR imaging, without biopsy. Objective: To describe the MR findings of pontine tumors at diagnosis and during follow-up and correlate those with prognosis and to assess the value of MR imaging in patient management compared to clinical evaluation. Materials and methods: Ninety-one MR scans of 15 children with diffuse pontine tumors were reviewed at diagnosis and during follow-up. The parameters analyzed were as follows: tumor extent, area, and volume; encasement of the basilar artery; presence of exophytic component; necrosis; cysts; hydrocephalus; and intensity and enhancement. Findings were correlated to length of progression-free and overall survival. Trends of amelioration or worsening on imaging were compared with the clinical findings. Results: Median length of progression-free survival was 10 months, and median survival was 20 months. Only hydrocephalus at presentation was associated with shorter progression-free survival (P=0.02). On the last examination of each patient, the craniocaudal diameter was significantly greater than at diagnosis (P=0.03). The concordance between the imaging and the clinical findings was good.Conclusion: MR is the mainstay for the diagnosis and management of pontine tumors. Cranial growth seems to be an ominous sign. However, the prognostic value of MR is limited. MR findings correlate well with the clinical examination.     

Malignant Peripheral Nerve Sheath Tumor of Bone in Children and Adolescents

Malignant peripheral nerve sheath tumor (MPNST) of bone is a rare entity. We have examined three lesions that fit standard histopathologic criteria for MPNST of soft tissues but that arose in the skeleton of three children aged 6 to 13 years. None was affected by neurofibromatosis 1 (NF1). Histologic features typical of MPNST included spindle cells with comma-shaped nuclei, tactoid bodies, nuclear palisading, hyaline bands, and schwannoma-like and curlicue foci. Epithelioid foci were seen in two cases, and heterologous differentiation in one. Immunohistochemistry revealed positivity for S-100 (1 positive/3 tested), vimentin (3/3), glial fibrillary acidic protein (2/3), CD34 (1/1), and CD68 (1/2). Studies for CD99 (0/3), epithelial membrane antigen (0/3), cytokeratin (0/3), CD57 (0/3), and HMB-45 (0/2) were negative. Ultrastructural findings in one of two cases examined included interlacing, attenuated cytoplasmic processes, microtubules, and rare dense-core granules. We conclude that MPNST may arise as a primary bone neoplasm in children without NF1. Received February 17, 1998; accepted June 19, 1998.     

Imaging following allograft reconstruction in children with malignant bone tumours

Purpose. To determine the nature of the imaging findings following reconstructive surgery using massive allografts in children with malignant bone tumours. Materials and methods. A retrospective review of the imaging studies and medical charts of 25 consecutive children who received an allograft as part of the management of a malignant bone tumour. Results. Uncomplicated allografts were sclerotic relative to native bone on radiographs and showed a typical ’tramline' appearance on bone scintigraphy. On MR, the medullary canal of the allograft showed low signal, similar to or greater than skeletal muscle, but less than subcutaneous fat, on 91 % of T1-weighted images. On short-tau inversion recovery images, the medullary canal was inhomogeneous and hyperintense to subcutaneous fat in 70 % and hyperintense to muscle in the remainder. Complications occurred in 68 % of patients and included allograft fractures (36 %), recurrent tumour (20 %), infection (8 %), and non-union or delayed union (8 %). The radiographic findings alone permitted accurate diagnosis of most serious complications. Infection and rejection were difficult to distinguish with any technique. All complications were suspected on clinical and/or radiological grounds before being shown by MR or scintigraphy. Conclusions. Allografts, whether normal or complicated, have characteristic imaging findings, except that infection and bone resorption related to rejection and revascularisation are difficult to distinguish. Routine MR and bone scintigraphy appear to contribute little to the management of these patients. Received: 6 October 1998 Accepted: 22 March 1999     

Application of a three-point method for water-fat MR imaging in children

Background. Effective fat suppression is desirable in clinical magnetic resonance imaging. Conventional frequency selective fat suppression is dependent on accurate prescan shimming and is subject to artifacts due to magnetic field inhomogeneity. Quadrature three-point water-fat imaging with direct phase encoding is an alternative technique for fat suppression that has been previously described in adult volunteers and patients. Objective. To evaluate the use of three-point water-fat imaging with direct phase encoding for fat-suppressed MR scans in children. Materials and methods. Sixty-two three-point water-fat imaging studies were performed in 55 children 2 months to 18 years old. T 1-weighted fat-suppressed (water) images from this sequence were compared with frequency selective fat-suppressed images obtained in 15 patients. The reliability and subjective quality of the sequence were assessed in the remaining 47 cases. Results. High-quality fat suppression was achieved in all anatomic sites studied, even where frequency selective fat-suppression failed due to magnetic susceptibility artifact. The three-point water-fat sequence was visually preferred to the frequency selective fat saturation technique in 15/15 cases. Conclusion. Three-point water-fat imaging has replaced the conventional frequency selective technique for fat suppression on T 1-weighted MR imaging at our institution. Received: 27 July 1998 Accepted: 23 December 1998     

Whole-body magnetic resonance imaging: a useful additional sequence in paediatric imaging

Background: Whole-body MR (WBMR) imaging allows the acquisition of images of the entire body in a matter of minutes. Its use has primarily been in the evaluation of possible metastases in the setting of a known primary tumour. Objective: To document the value of WBMR imaging in ten children in whom this was added as an additional sequence when the primary diagnosis had not yet been made. Materials and methods: Ten children, age range 4 months–15 years (mean 7 years 4 months) had WBMR imaging after initial MR showed an abnormality that raised the possibility of systemic disease. Initial scanning was of the brain (n=1), spine (n=2), retroperitoneum (n=4), hips (n=1), femur (n=1) and wrist (n=1). Results: Abnormalities were detected in eight patients. Two patients had acute lymphoblastic leukaemia, and another had an anaplastic lymphoma, unsuspected prior to the WBMR. Two patients had a previously undiagnosed neuroblastoma with bone marrow metastases. Two patients had Langerhans cell histiocytosis. Another had multiple bone lesions due to cystic angiomatosis. Conclusions: WBMR imaging may be a useful additional sequence in children in whom a systemic and especially a bone marrow abnormality is suspected.Presented at 40th annual meeting of ESPR, Genoa, June 2003     

A prospective 10 year follow up study of patients with neurofibromatosis type 1

OBJECTIVE—To establish the prevalence and incidence of symptoms and complications in children with neurofibromatosis type 1 (NF1) and to assess possible risk factors for the development of complications.
DESIGN—A 10 year prospective multidisciplinary follow up study.
PATIENTS—One hundred and fifty children diagnosed with NF1 according to criteria set by the National Institutes of Health.
RESULTS—In 62 of 150 children (41.3%) complications were present, including 42 (28.0%) children with one complication, 18 (12.0%) with two complications, and two (1.3%) with three complications (mean (SD) duration of follow up 4.9 (3.8) years). Ninety five of the 150 children presented without complications (follow up, 340.8 person-years). The incidence of complications was 2.4/100 person-years in this group. An association was found between behavioural problems and the presence of complications.
CONCLUSION—This is the largest single centre case series of NF1 affected children followed until 18 years of age. Children with NF1, including those initially presenting without complications, should have regular clinical examinations.

     

MRI evaluation of infectious and non-infectious synovitis: preliminary studies in a rabbit model

Background. Literature on magnetic resonance imaging (MR) evaluation of inflammatory joint effusions is sparse. Objective. To describe an animal model for studying infectious and non-infectious joint effusions with magnetic resonance imaging. Materials and methods. Ten rabbit knees with septic arthritis and four with talc synovitis were imaged with MR. Contralateral knees injected with saline served as controls. Fat saturation T2-weighted and gadolinium-enhanced T1-weighted images were assessed for joint effusion, and periarticular and adjacent intraosseous increased signal or enhancement. Each knee was cultured and underwent pathologic examination. Results. Both Staphylococcus aureus and talc produced effusions in all knees. The degree of periarticular signal and enhancement was greater in infected knees than talc-injected knees. No abnormal enhancement was seen within bone. Pathologic examination showed a greater degree of inflammation and joint destruction in the infected knees, but no evidence of osteomyelitis. Conclusion. A greater degree of abnormal signal and enhancement seen on MR suggests a more vigorous inflammatory process, as seen with septic arthritis. In spite of advanced septic arthritis, no enhancement was evident within bone, suggesting that enhancement within bone is not an expected finding in isolated septic arthritis and should raise concern for osteomyelitis. Received: 8 May 1998 Accepted: 27 October 1998     

Imaging features of pancreatic tumors in children: 13-year experience at a pediatric tertiary hospital

Background

Pancreatic tumors are rare in children, resulting in limited information regarding their frequency and imaging appearances.

Objective

To review all pancreatic tumors seen in children over a decade at a large tertiary pediatric institution and to describe multimodality imaging findings.

Materials and methods

We conducted a retrospective review of imaging studies performed in children with pancreatic tumors between January 2000 and December 2012, analyzing imaging features on available US, CT and MR examinations. We reviewed patient charts for clinical features, management and final diagnosis.

Results

We included 23 children in this study. Of these, 12 had solid and papillary epithelial neoplasms (SPEN), 3 had neuroendocrine tumors, 3 had lymphoma, and 1 each had hemangioendothelioma, benign congenital cysts with adipose tissue, dendritic cell sarcoma, metastatic rhabdomyosarcoma, and lipoma. All children with SPEN were teenage girls. SPEN showed characteristic imaging features including well-defined margins with surrounding capsule, solid-cystic components and hemorrhage.

Conclusion

Pancreatic tumors are uncommon in children. SPEN is the most common tumor and is seen predominantly in teenage girls and shows characteristic imaging features.