The use of 3D printing in shared decision making for a juvenile aggressive ossifying fibroma in a pediatric patient.

Juvenile aggressive ossifying fibromas (JAOF) are rare, typically benign pediatric tumors that are locally aggressive and have high recurrence rates. A 7-year old male presented with a palatal mass and a 3D printed model was created and used as a visual aide to highlight the importance of management in terms of functional, cosmetic, and disease-free outcomes with the family. The patient ultimately underwent successful enucleation with final pathology consistent with JAOF. To our knowledge, this is the first description of the use of 3D printing to help in the shared decision-making process for the treatment of this aggressive tumor.

Straight to the Operating Room: An Emergent Surgery Track for Acute Testicular Torsion Transfers.

OBJECTIVE: To assess the effect of implementing an emergency surgery track for testicular torsion transfers. We hypothesized that transferring children from other facilities diagnosed with torsion straight to the operating room (STOR) would decrease ischemia time, lower costs, and reduce testicular loss. STUDY DESIGN: Demographics, arrival to incision time, hospital cost in dollars, and testicular outcome (determined by testicular ultrasound) at follow-up were retrospectively compared in all patients transferred to our tertiary care children's hospital with a diagnosis of testicular torsion from 2012 to 2016. Clinical data for STOR and non-STOR patients were compared by Wilcoxon rank-sum, 2-tailed t test, or Fisher exact test as appropriate. RESULTS: Sixty-eight patients met inclusion criteria: 35 STOR and 33 non-STOR. Children taken STOR had a shorter median arrival to incision time (STOR: 54 minutes vs non-STOR: 94 minutes, P < .0001) and lower median total hospital costs (STOR: $3882 vs non-STOR: $4419, P < .0001). However, only 46.8% of STOR patients and 48.4% of non-STOR patients achieved surgery within 6 hours of symptom onset. Testicular salvage rates in STOR and non-STOR patients were not significantly different (STOR: 68.4% vs non-STOR: 36.8%, P = .1), but follow-up was poor. CONCLUSIONS: STOR decreased arrival to incision time and hospital cost but did not affect testicular loss. The bulk of ischemia time in torsion transfers occurred before arrival at our tertiary care center. Further interventions addressing delays in diagnosis and transfer are needed to truly improve testicular salvage rates in these patients.

Craniofacial Flash: Minimizing Radiation Dose in Pediatric Craniofacial Computed Tomography.

BACKGROUND AND PURPOSE: The routine use of computed tomography (CT) in evaluation of pediatric craniofacial abnormalities is controversial. While there are benefits to preoperative imaging, there are risks related to ionizing radiation and sedation/anesthesia. In this study, the authors describe their experience with a new craniofacial CT protocol (Craniofacial-Flash) that provides bone detail equivalent to conventional CT, while delivering a substantially lower radiation dose and reducing the scan time to obviate the requirement of sedation/anesthesia. METHODS: Following IRB approval, dose identification and image analysis were conducted retrospectively on all patients who underwent craniofacial CT using either the conventional protocol or Craniofacial-Flash protocol between November 2013 and September 2015. Image analysis consisted of automated segmentation of bone and soft tissue, followed by noise interpretation of each study segment. RESULTS: The conventional CT group included 175 patients (mean age 6.38 years) with a median dose length product of 243.00 mGy·cm. The Craniofacial-Flash CT group included 208 patients (mean age 3.31 years) with median dose length product of 27.60 mGy·cm. Image quality was equivalent for bone, with coefficient of variation values of 0.20 for the conventional CT group and 0.21 for the Craniofacial-Flash group. Soft tissues coefficients of variation were disparate at 0.07 for conventional CT and 0.14 for Craniofacial-Flash protocol. CONCLUSION: The Craniofacial-Flash protocol reliably generates craniofacial bone images adequate for routine use in craniofacial practice, while reducing the radiation dose by 88.87% compared with a conventional craniofacial CT scan, and eliminating the need for sedation.

Targeted MAPK Pathway Inhibitors in Patients With Disseminated Pilocytic Astrocytomas.

This report presents a series of 5 pediatric patients with disseminated pilocytic astrocytomas and frequent nonfusion activating mutations. Genetic variants in these patients' tumors include BRAF p.Val600Glu, BRAF p.Val600Asp, and KRAS p.Gly60_Gln62ins7. The 2 patients with BRAF-mutated tumors were treated with dabrafenib or a combination of dabrafenib plus trametinib. The patients had either near complete resolution of the primary tumor (BRAF p.Val600Glu) or a stable primary tumor (BRAF p.Val600Asp). Both patients showed improvement in leptomeningeal dissemination without significant toxicity. Genomic testing of disseminated pilocytic astrocytomas, particularly those arising at extracerebellar locations, may result in the identification of mutations associated with ERK/MAPK activation. Patients with these activating mutations may benefit from targeted therapies.

Ionizing Radiation in Craniofacial Surgery: A Primer on Dose and Risks.

An understanding of radiation dose and the anticipated risk to the patient is an important aspect of ordering radiological imaging studies responsibly. It is especially true for the pediatric practitioner because children are more vulnerable to the biological effects of radiation, such as radiosensitivity, longer lifetime years, and higher cellular mitotic activity. The use of fluoroscopy and computed tomography is commonplace in the practice of craniofacial surgery, but often dose reports from varied investigations are not directly comparable, and the risk of patient harm from the investigation is unclear. This article presents the fundamentals of radiation, dose, and risk as it applies to radiological imaging and also introduces our low dose craniofacial computed tomography protocol.

Postoperative surveillance of pediatric cerebellar pilocytic astrocytoma.

The purpose of this study was to identify the optimal frequency and duration of magnetic resonance imaging follow-up in children who had gross totally resected cerebellar pilocytic astrocytomas (CPAs). Our hypothesis was that following two MR examinations, separated by at least 3 months, showing no evidence of tumor, gross totally resected CPAs did not recur and no further imaging follow-up was necessary. Retrospective review of Neuro-Oncology database from 1/2000 to 7/2013 yielded 53 patients with CPAs that had preoperative imaging and >2 years post-operative imaging follow-up available. Pilocytic astrocytomas with brainstem involvement and patients with neurofibromatosis type I were excluded. Preoperative tumor volumes were calculated. The dates and reports of the examinations were tabulated. The median number of follow-up examinations was 9 over a median follow-up time of 6.05 years (2.07-12.28 years). Two consecutive MR examinations over at least a 3 month span demonstrated the smallest negative likelihood ratio of future recurrence (0.15). There was no association of recurrence with preoperative tumor volume. Among the 35 patients with gross total resection of their tumor and greater than two negative follow-up examinations, one recurrence (2.9 %) was identified, occurring 6.4 years after initial resection. Gross totally resected pediatric CPAs can recur, but this is exceedingly rare. Frequent surveillance (every 3-6 months) is suggested in patients with CPAs until absence of tumor is concluded on imaging and documented on two consecutive studies spaced at least 3 months apart. The likelihood of recurrence thereafter is low.

Pediatric Sarcomas Are Targetable by MR-Guided High Intensity Focused Ultrasound (MR-HIFU): Anatomical Distribution and Radiological Characteristics.

BACKGROUND: Despite intensive therapy, children with metastatic and recurrent sarcoma or neuroblastoma have a poor prognosis. Magnetic resonance guided high intensity focused ultrasound (MR-HIFU) is a noninvasive technique allowing the delivery of targeted ultrasound energy under MR imaging guidance. MR-HIFU may be used to ablate tumors without ionizing radiation or target chemotherapy using hyperthermia. Here, we evaluated the anatomic locations of tumors to assess the technical feasibility of MR-HIFU therapy for children with solid tumors. PROCEDURE: Patients with sarcoma or neuroblastoma with available cross-sectional imaging were studied. Tumors were classified based on the location and surrounding structures within the ultrasound beam path as (i) not targetable, (ii) completely or partially targetable with the currently available MR-HIFU system, and (iii) potentially targetable if a respiratory motion compensation technique was used. RESULTS: Of the 121 patients with sarcoma and 61 patients with neuroblastoma, 64% and 25% of primary tumors were targetable at diagnosis, respectively. Less than 20% of metastases at diagnosis or relapse were targetable for both sarcoma and neuroblastoma. Most targetable lesions were located in extremities or in the pelvis. Respiratory motion compensation may increase the percentage of targetable tumors by 4% for sarcomas and 10% for neuroblastoma. CONCLUSIONS: Many pediatric sarcomas are localized at diagnosis and are targetable by current MR-HIFU technology. Some children with neuroblastoma have bony tumors targetable by MR-HIFU at relapse, but few newly diagnosed children with neuroblastoma have tumors amenable to MR-HIFU therapy. Clinical trials of MR-HIFU should focus on patients with anatomically targetable tumors.

Pilomatricoma of Childhood: A Common Pathologic Diagnosis Yet a Rare Radiologic One.

OBJECTIVE: The purposes of this study were to determine the accuracy of radiology reports in the diagnosis of pediatric pilomatricoma and to describe the characteristic imaging findings in a large cohort. MATERIALS AND METHODS: Query of a pathology database between January 2009 and February 2014 yielded 623 specimens from 596 patients. Seventy-four patients (41 girls, 33 boys; mean age, 8.9 years) underwent imaging of the 80 excised lesions. Ninety imaging studies were available (58 ultrasound, 11 CT, 21 MRI). Radiology reports were reviewed. The lesions were evaluated for location, size, and imaging characteristics. RESULTS: Pilomatricoma was included as an indication for two (2.2%) imaging studies. No differential diagnosis was given in 34 (37.8%) imaging reports. In the radiology reports, pilomatricoma was suggested as a possibility for 12 (13.3%) lesions. Foreign body reaction (13.3%), dermoid cyst (10.0%), and sebaceous cyst (8.9%) were other leading suggested diagnoses. In six (6.7%) reports, lesions were described as aggressive tumors. In nine (10.0%) reports additional studies were recommended. In all examinations, the lesions involved both skin and subcutaneous tissues without infiltration of the deep tissues. Hypoechoic rim (82.8%), internal reticulations (65.5%), and flow at Doppler examination (63.2%) were the most common sonographic features. MRI characteristics were enhancement (100.0%), heterogeneous T2-weighted hyperintensity with intermixed hypointensity (76.2%), T1-weighted isointensity in relation to skeletal muscle (66.7%), and absence of restricted diffusion (100.0%). CONCLUSION: Although pilomatricoma is a common tumor of the skin and subcutaneous tissues, imaging is infrequently used in the diagnosis, and therefore, many radiologists are not aware of the lesion. The imaging features of pilomatricoma are consistent in all imaging modalities.

Tectal Lesions in Children: A Long-Term Follow-Up Volumetric Tumor Growth Analysis in Surgical and Nonsurgical Cases.

BACKGROUND/AIMS: Different tectal masses have been described; most are low-grade gliomas. Only 20-30% of all lesions grow, as shown on follow-up MRIs, requiring surgical resection at some point. The aim of this study is to describe the experience of a single institution managing pediatric patients with tectal lesions. METHODS: We retrospectively studied and analyzed 40 children with tectal lesions managed from 1990 to 2006; the mean age at diagnosis was 9.4 years. A volumetric classification was used to analyze tumor growth trends. More than 1 year of imaging follow-up was available for 23 patients. RESULTS AND CONCLUSION: Medium- and large-volume-size lesions were associated with the need for surgery. About half of the nonsurgical lesions grew at least 50% over a period of 4.5 years and did not require surgical resection.

Acute silent cerebral ischemia and infarction during acute anemia in children with and without sickle cell disease.

We hypothesized that the silent cerebral infarcts (SCI), which affect up to 40% of children with sickle cell disease (SCD), could occur in the setting of acute anemic events. In a prospective observational study of children with and without SCD hospitalized for an illness associated with acute anemia, we identified acute silent cerebral ischemic events (ASCIE) in 4 (18.2%) of 22 with SCD and in 2 (6.7%) of 30 without SCD, using diffusion-weighted magnetic resonance imaging. Children with ASCIE had lower hemoglobin concentration than those without (median 3.1 vs 4.4 g/dL, P = .003). The unique temporal features of stroke on diffusion-weighted magnetic resonance imaging permit estimation of incidence rates for ASCIE of 421 (95% confidence interval, 155-920) per 100 patient-years during acute anemic events for all patients. For children with SCD, the estimated incidence was 663 (95% confidence interval, 182-1707) which is much higher than previously reported. Acute anemic events are common in children with SCD and prevalence could partially account for the high SCI. Some ASCIE (1 of 4 in our study) may be reversible. Alterations in management may be warranted for children with severe anemia to identify unrecognized ischemic brain injury that may have permanent neurocognitive sequelae.

MRI of the petromastoid canal in children.

PURPOSE: To characterize the MRI features of the petromastoid canal in children with sensorineural hearing loss (SNHL) and in normal infants. MATERIALS AND METHODS: High resolution MRI examinations of 564 children who were evaluated for SNHL and brain MRI examinations of 112 infants who had normal studies were studied independently by two reviewers. RESULTS: In SNHL group, visibility of the PMC decreased for right and left PMC (P < 0.001). The width of the right PMC significantly decreased as age increased (P < 0.0001). There was no relation between abnormalities of membranous labyrinth and cochlear nerve and PMC visibility in children with SNHL (p > 0.05). In the normal group, the PMC visibility decreased with increasing age (right P = 0.0001, left P = 0.001). In the normal group also, as age increased, the PMC width decreased for both PMCs (right, P = 0.0006; left, P = 0.03). CONCLUSION: The PMC is more frequently visualized in young children. Its visibility and width are not associated with abnormalities of membranous labyrinth and cochlear nerves.

Applicability of apparent diffusion coefficient ratios in preoperative diagnosis of common pediatric cerebellar tumors across two institutions.

INTRODUCTION: The purpose of our study was to test the accuracy and applicability of decision rules utilizing apparent diffusion coefficient (ADC) ratios on accurate preoperative diagnosis of common pediatric cerebellar tumors across two institutions. METHODS: In this HIPAA-compliant, IRB-approved study, performed at two institutions, 140 pediatric cerebellar tumors were included. Two separate reviewers placed regions of interest on the solid components of 140 tumors (98 at site A and 42 at site B) and normal brain on the ADC maps. The third reviewer who was blinded to the histopathological diagnoses made the same measurements on 140 patients to validate the data. Tumor to normal brain ADC ratios were calculated. Receiver operator curve (ROC) analysis was performed to generate thresholds to discriminate tumors. Utility of decision rules based on these thresholds was tested. RESULTS: While ADC values of medulloblastomas were different between the sites, there was no difference among the ADC ratios of medulloblastomas, pilocytic astrocytomas, ependymomas, and atypical teratoid rhabdoid tumors between the sites. ADC ratio of ≥1.8 correctly discriminated pilocytic astrocytomas from ependymomas with a sensitivity of 0.83 and a specificity of 0.78. ADC ratio of <1.2 correctly discriminated ependymomas from embryonal tumors with a sensitivity of 0.87 and a specificity of 0.83. The proposed decision rules correctly discriminated 120 of the 140 tumors (85.71%). Age ≥2 years criterion correctly sorted medulloblastomas in 84.48% of patients and age <2 years correctly distinguished atypical teratoid rhabdoid tumors in 90.00% of patients with embryonal tumors. CONCLUSIONS: Decision rules based on ADC ratios are applicable across two institutions in the accurate preoperative diagnosis of common pediatric cerebellar tumors.

The Sensitivity of Limited-Sequence MRI in Identifying Pediatric Cervical Spine Injury: A Western Pediatric Surgery Research Consortium Multicenter Retrospective Cohort Study.

INTRODUCTION: Clinical clearance of a child's cervical spine after trauma is often challenging due to impaired mental status or an unreliable neurologic examination. Magnetic resonance imaging (MRI) is the gold standard for excluding ligamentous injury in children but is constrained by long image acquisition times and frequent need for anesthesia. Limited-sequence MRI (LSMRI) is used in evaluating the evolution of traumatic brain injury and may also be useful for cervical spine clearance while potentially avoiding the need for anesthesia. The purpose of this study was to assess the sensitivity and negative predictive value of LSMRI as compared to gold standard full-sequence MRI as a screening tool to rule out clinically significant ligamentous cervical spine injury. METHODS: We conducted a ten-center, five-year retrospective cohort study (2017-2021) of all children (0-18y) with a cervical spine MRI after blunt trauma. MRI images were re-reviewed by a study pediatric radiologist at each site to determine if the presence of an injury could be identified on limited sequences alone. Unstable cervical spine injury was determined by study neurosurgeon review at each site. RESULTS: We identified 2,663 children less than 18 years of age who underwent an MRI of the cervical spine with 1,008 injuries detected on full-sequence studies. The sensitivity and negative predictive value of LSMRI were both >99% for detecting any injury and 100% for detecting any unstable injury. Young children (age < 5 years) were more likely to be electively intubated or sedated for cervical spine MRI. CONCLUSION: LSMRI is reliably detects clinically significant ligamentous injury in children after blunt trauma. To decrease anesthesia use and minimize MRI time, trauma centers should develop LSMRI screening protocols for children without a reliable neurologic exam. LEVEL OF EVIDENCE: 2 (Diagnostic Tests or Criteria).

Common pediatric cerebellar tumors: correlation between cell densities and apparent diffusion coefficient metrics.

PURPOSE: To test whether there is correlation between cell densities and apparent diffusion coefficient (ADC) metrics of common pediatric cerebellar tumors. MATERIALS AND METHODS: This study was reviewed for issues of patient safety and confidentiality and was approved by the Institutional Review Board of the University of Texas Southwestern Medical Center and was compliant with HIPAA. The need for informed consent was waived. Ninety-five patients who had preoperative magnetic resonance imaging and surgical pathologic findings available between January 2003 and June 2011 were included. There were 37 pilocytic astrocytomas, 34 medulloblastomas (23 classic, eight desmoplastic-nodular, two large cell, one anaplastic), 17 ependymomas (13 World Health Organization [WHO] grade II, four WHO grade III), and seven atypical teratoid rhabdoid tumors. ADCs of solid tumor components and normal cerebellum were measured. Tumor-to-normal brain ADC ratios (hereafter, ADC ratio) were calculated. The medulloblastomas and ependymomas were subcategorized according to the latest WHO classification, and tumor cellularity was calculated. Correlation was sought between cell densities and mean tumor ADCs, minimum tumor ADCs, and ADC ratio. RESULTS: When all tumors were considered together, negative correlation was found between cellularity and mean tumor ADCs (ρ = -0.737, P < .05) and minimum tumor ADCs (ρ = -0.736, P < .05) of common pediatric cerebellar tumors. There was no correlation between cellularity and ADC ratio. Negative correlation was found between cellularity and minimum tumor ADC in atypical teratoid rhabdoid tumors (ρ = -0.786, P < .05). In atypical teratoid rhabdoid tumors, no correlation was found between cellularity and mean tumor ADC and ADC ratio. There was no correlation between the ADC metrics and cellularity of the pilocytic astrocytomas, medulloblastomas, and ependymomas. CONCLUSION: Negative correlation was found between cellularity and ADC metrics of common pediatric cerebellar tumors. Although ADC metrics are useful in the preoperative diagnosis of common pediatric cerebellar tumors and this utility is generally attributed to differences in cellularity of tumors, tumor cellularity may not be the sole determinant of the differences in diffusivity.

Juvenile xanthogranulomas of the nervous system: A report of two cases and review of the literature.

Juvenile xanthogranulomas (JXG) are uncommon non-Langerhans cell histiocytic proliferations which arise most often in children. While most cases present as solitary cutaneous lesions, occasional cases involve extracutaneous sites. Rare examples of JXGs have been reported involving all levels of the neuroaxis. We present two cases of JXGs involving the nervous system, and review the literature. The first patient was a 14-year-old female with headaches and a mass involving the left trigeminal nerve; pathologic examination showed a JXG. At 11 months follow-up, after administration of systemic chemotherapy, the patient remained stable with residual tumor. The second patient was a 15-year-old female with leg weakness and numbness, who underwent complete surgical resection of a dural JXG. At eight months follow-up, she showed no evidence of tumor, and was able to walk without difficulty. Review of the literature revealed 38 previously published reports of JXGs involving the nervous system. The CNS was involved in the majority (75%) of cases. The clinical characteristics of JXGs arising in the CNS varied significantly from cases in the peripheral nervous system (PNS); CNS tumors occurred in younger patients, more often males, and were more likely to be associated with concurrent cutaneous and extra-nervous systemic lesions. The clinical outcomes were similar for CNS and PNS lesions, with the caveat that all three lethal JXGs occurred in the CNS. The clinical and radiologic presentation of JXGs is nonspecific, thus necessitating biopsy and pathologic examination to arrive at the diagnosis. The pathologic differential diagnosis includes a heterogeneous group of histiocytic proliferations; immunostaining for histiocytic markers CD68, factor XIIIa, and Fascin, and the absence of Birbeck granules and CD1a immunoexpression suggests the diagnosis of JXG. In many cases, total surgical resection is curative. However, some cases will require additional chemotherapy and/or radiotherapy.

Management of intratonsillar abscess in children.

BACKGROUND: The aim of this study was to assess outcomes of medical and surgical treatment of intratonsillar abscess in children. METHODS: The medical charts of children with intratonsillar abscess were reviewed to obtain information on history and physical examination, imaging, management, and follow-up assessment. RESULTS: Eleven children (six male, five female; age range, 4-18 years) were identified. The common complaints included sore throat, fever, and odynophagia. Asymmetric tonsil hypertrophy was present in nine patients and erythema of tonsils in all patients. Peritonsillar fullness was present in three patients. One patient needed emergency intubation due to respiratory compromise. Computed tomography indicated unilateral intratonsillar abscess in nine patients, bilateral intratonsillar abscess in one, and unilateral phlegmon in one. Inflammatory changes were observed in the parapharyngeal space in all patients, retropharyngeal space in one, and pyriform sinus and aryepiglottic folds in two. Antibiotic treatment included clindamycin in seven patients, ampicillin/sulbactam in one, and clindamycin plus ceftriaxone in three. The patients with respiratory compromise underwent surgery prior to antibiotic treatment. Patients with isolated intratonsillar abscess or phlegmon had resolution of their symptoms with i.v. antibiotic treatment. Patients with combination of intratonsillar and peritonsillar abscess required incision and drainage of peritonsillar abscess. CONCLUSIONS: Clinically stable children with intratonsillar abscess or phlegmon respond to i.v. antibiotic therapy. Surgical drainage can accomplish clinical resolution in the presence of a combination of intra- and peri-tonsillar abscess, airway compromise, or unresponsiveness to medical treatment.

Embryonal rhabdomyosarcoma of the auricle in a child.

We describe the diagnosis and management of a child with embryonal rhabdomyosarcoma of the auricle and emphasize both clinical and radiological findings of this rare condition. A nine-year-old boy presented for evaluation of a slowly enlarging left auricle mass. The mass was nodular, violaceous, semi-translucent, and hyperpigmented with an overlying pseudo-vesicular plaque. The mass appeared to involve the left cavum concha, root of the helix, superior aspect of the external auditory canal, the tragus and extend to a deep preauricular component. MR imaging documented a lobulated soft tissue mass surrounding the external auditory canal with superficial involvement of the pinna. Incisional biopsy of the mass suggested embryonal rhabdomyosarcoma. The tumor was completely removed by total auriculectomy and lateral temporal bone resection. The final diagnosis was embryonal rhabdomyosarcoma. Although rare, otolaryngologists, pediatricians, and radiologists need to consider rhabdomyosarcoma in the differential diagnosis of auricle mass in children.

Imaging of Central Nervous System Involvement in Pediatric Hematologic Disorders.

Hematologic disorders and malignancies are commonly encountered in children. The central nervous system is affected by many benign and malignant hematological diseases. Neurologic findings may occur due to central involvement of hematological disease or neurotoxic effects of treatment during the disease. At all these stages, radiological imaging and evaluation have a critical role. In this review article, we aim to describe the imaging findings of central nervous system involvement in pediatric hematologic diseases. This review was prepared based on the latest literature available in the PubMed database in the English language from inception to March 2022. The radiological images of the patients in our archive were obtained from the PACS (Picture Archiving and Communication Systems) to set an example for the diseases described in the text.

Wilms tumor: preoperative risk factors identified for intraoperative tumor spill.

PURPOSE: We identified preoperative parameters associated with increased risk of intraoperative Wilms tumor spill. MATERIALS AND METHODS: We retrospectively reviewed an institutional database of patients diagnosed with Wilms tumor between 2000 and 2008. Inclusion criteria consisted of available abdominal computerized tomogram and pathological stage I to IV disease. Patient characteristics and neoadjuvant chemotherapy use were noted. After blinding, a radiologist reviewed preoperative computerized tomogram parameters, calculating tumor volume and assigning a preoperative radiological stage. RESULTS: Of 67 patients diagnosed with Wilms tumor 41 (22 males, 19 females) met inclusion criteria, while 26 had incomplete imaging for analysis. Comparison of patients with and without intraoperative tumor spill demonstrated no significant differences in age (3.8 vs 3.6 years), sex (3 males and 3 females vs 19 males and 16 females), body weight or tumor capsule thickness. Preoperative radiological staging was unable to predict pathological stage I to III disease. Six intraoperative tumor spills (15%) were identified (left in 4, right in 2), of which 3 were stage III disease and 3 stage IV. Without neoadjuvant chemotherapy, patients with tumors greater than 1,000 cc had an increased risk of spill (2 of 2 [100%] vs 4 of 33 [12%], p = 0.03). Of 9 patients with stage IV disease 0% (0 of 4) receiving neoadjuvant chemotherapy experienced tumor spill, while lack of neoadjuvant chemotherapy was associated with a 60% (3 of 5 patients, 1 male and 2 females) risk of stage IV spill (p = 0.17). CONCLUSIONS: The sole significant tumor spill risk factor identifiable preoperatively was tumor volume greater than 1,000 cc. However, spill occurred at volumes less than 400 cc. Although not statistically significant, neoadjuvant chemotherapy for stage IV disease trended toward diminishing spill risk. Patients with Wilms tumors greater than 1,000 cc may benefit from neoadjuvant chemotherapy with less tumor spill, while stage IV tumors warrant further study in this regard.

Intermediate grade meningeal melanocytoma of cervical spine.

BACKGROUND: Meningeal melanocytoma is a rare, benign melanotic tumor of the leptomeninges, which occurs anywhere in the cranial or spinal regions but most commonly in supratentorial and thoracic spine regions. The literature on this entity consists of case reports; therefore, there is no agreement on the most effective therapy of this tumor, although total excision seems to be the best therapeutic option. CASE HISTORY: We report a 17-year-old girl with intermediate grade meningeal melanocytoma involving the C6 nerve root with spinal cord compression resulted in progressive tetraparesis. Clinical and radiological examinations suggested the possibility of an intradural extramedullary solid mass. The tumor was removed subtotally through cervical laminotomy followed by rapid improvement of most neurological deficits. This tumor was unusual because of its very hyperintense homogenous signal on T1-weighted images, invasion of the arachnoid membrane, and extension into the neural foramina. Black dots on the surface of the cord were thought to represent an organized blood clot until the frozen section suggested a melanocytic tumor. DISCUSSION: We discuss the distinction of meningeal melanocytoma from other melanocytic tumors of the leptomeninges. CONCLUSION: Melanocytic tumors should be considered in the differential diagnosis when a hyperintense lesion of the leptomeninges is identified on T1-weighted images or a very dark mass similar to charcoal or organized hematoma is found in the surgical field. The best management is complete tumor resection, but radiotherapy is reserved in cases of subtotal resection and multiple lesions. Locally aggressive nature of tumor and possibility of recurrence warrant regular follow-up.