Purpose
Intracranial ganglioneuroblastomas are incredibly rare neuroectodermal tumors with only 8 described cases total, 5 of those having imaging findings
Methods
Here we present a 9-year-old female ...patient with 4 months progressive headaches, personality changes, and vomiting. We also present a review of the current literature of intracranial ganglioneuroblastomas.
Results
Imaging demonstrated a partially calcified suprasellar mass measuring 4.6 × 6.3 × 5 cm composed of both solid and cystic components, diagnosed to be a ganglioneuroblastoma, with mass effect on the lateral and 3rd ventricles, with a midline shift of right to left of 6-7 mm. She was treated with subtotal surgical resection, an intensive chemotherapeutic regimen, and radiation and has no residual disease on imaging 1 year and 4 months status post-surgery.
Conclusion
To our knowledge, this is the first case of a ganglioneuroblastoma to mimic a craniopharyngioma based upon imaging findings and suprasellar location. As these cases are extremely rare, an optimal therapeutic regimen has not been defined. However, a combination of surgical resection, chemotherapy, and radiation therapy can be effective, as shown here with successful treatment and no evidence of residual disease.
The WHO Classification of Tumours of the Central Nervous System (2016) classifies nonmeningothelial malignant spindle cell tumors involving the extraaxial tissues of the posterior fossa as ...melanocytic tumors and malignant mesenchymal tumors (sarcomas). The objective of this study was to conduct a review of the literature pertaining to the management strategies of posterior fossa malignant spindle cell tumors in the pediatric population.
The authors performed an institutional search of their pathology database for patients younger than 18 years of age who presented with posterior fossa malignant spindle cell tumors. A literature review was also performed using the PubMed database, with "posterior fossa" or "spindle cell tumors" or "Ewing sarcoma" or "high-grade" or "spindle cell sarcoma" or "leptomeningeal melanocytoma" as keywords. The database search was restricted to pediatric patients (age ≤ 18 years). Parameters reported from the literature review included patient age, tumor location, presenting symptoms, treatment modalities (resection, chemotherapy, and/or radiotherapy), leptomeningeal spread at or after the time of treatment, and follow-up length and resulting outcome.
The authors report 3 rare cases of posterior fossa malignant spindle cell tumors, including Ewing sarcoma in a 13-year-old male; high-grade spindle cell sarcoma, not otherwise specified in a 10-year-old male; and primary leptomeningeal melanocytoma in a 16-year-old female. All 3 patients underwent resection and radiotherapy and either chemotherapy or targeted immunotherapy. At the last follow-up, all patients were alive with either resolution or stable disease.
A review of these 3 cases and the existing literature support managing patients with intracranial malignant spindle cell tumors with multimodal therapy that can include a combination of resection, radiotherapy, and chemotherapy or immunotherapy to prolong progression-free and overall survival.
Introduction
Intracranial malignant peripheral nerve sheath tumor (MPNST) is exceedingly rare. Previously reported cases of intracranial MPNST have been associated with development within a prominent ...cranial nerve.
Methods
This is the first report of an MPNST with both nerve sheath and vascular phenotype that follows the neurovascular bundle, without arising in a major cranial nerve or in the setting of neurofibromatosis type 1 (NF1).
Results
The patient is a 14-year-old boy with a history of worsening headaches for the past several months, left-sided hearing loss, nausea, vomiting, and vertigo. MRI was performed that demonstrated a large extra-axial tumor compressing the left infratemporal posterior temporal region. The tumor was associated with significant destruction of the superior portion of the petrous bone and extension through the petrous into the upper posterior fossa, immediately below the tentorium. The patient underwent surgical debulking and adjuvant chemotherapy with doxorubicin and ifosfamide. Pathology demonstrated a variant malignant peripheral nerve sheath tumor with both nerve sheath and vascular phenotype by immunostains. The patient’s symptoms improved following treatment.
Conclusion
We present the first reported case of an intracranial MPNST variant that developed along the neurovascular bundle as a sarcoma with both nerve sheath and vascular phenotype through the petrous bone and not associated with a major cranial nerve or with stigmata of neurofibromatosis type 1 (NF1). Although this is an extremely unusual presentation due to location and lack of prominent cranial nerves in that location, it is not unusual for benign nerve sheath tumors to follow the neurovascular bundle through foramen of cortical long bone or pelvis. This case suggests that physicians should incorporate intracranial MPNST variant into their differential diagnosis in the cranium, even when tumor is not located near a prominent cranial nerve. Surgical debulking and adjuvant chemotherapy with doxorubicin and ifosfamide has led to improvement in patient symptoms.
Choroid plexus: normal size criteria on neuroimaging Madhukar, Megha; Choudhary, Arabinda K.; Boal, Danielle K. ...
Surgical and radiologic anatomy (English ed.),
12/2012, Volume:
34, Issue:
10
Journal Article
Peer reviewed
Purpose
The lack of a well-recognized normal size criterion for the choroid plexus makes small or diffuse choroid plexus pathologies difficult to recognize. The purpose of this study was to determine ...the normal size of the choroid plexus in the pediatric population utilizing magnetic resonance imaging (MR) and ultrasound (US). As volume measurement across multiple slices is a laborious process, we wanted to propose a simple clinical tool that is easy to use, reproducible, and quick to obtain measurements.
Methods
This study retrospectively evaluated choroid plexus size in 90 children between the ages of 0 and 16. To determine the choroid plexus thickness, a total of 97 studies (71 MRIs and 26 Ultrasounds) were reviewed, from children without any signs of choroid plexus pathology; 6 measurements were taken from MR studies, and 3 measurements were taken from US studies. Averages and ranges of choroid plexus thickness were computed across age groups and gender.
Results
Across all ages, the mean choroid plexus thickness in the lateral ventricles was found to be 3.4, 3.3, and 3.1 mm in the axial, coronal, and sagittal views; 3.2 mm in the temporal horns; 2.5 mm in the fourth ventricle with the lateral limb of the choroid plexus in the fourth ventricle measuring 11.3 mm in length. No trends were observed with respect to age subgroups or gender.
Conclusion
Choroid plexus thickness on average was 3.2 mm in the lateral ventricles and 2.5 mm in the fourth ventricle.
Optimal patient-physician communication in the outpatient clinical setting is critical for safe and effective patient care. Keeping track of multiple patient telephone messages can be difficult and ...hazardous if a structured system is not in place. A multidisciplinary group at Hershey Medical Center developed a standardized approach for addressing patient telephone calls at their outpatient surgical clinics. This program was designed to improve the patient experience by providing a realistic time frame for phone calls to be returned and requests fulfilled. Additionally, this system permitted phone calls to be tracked and documented appropriately and allowed for prioritization of urgent and emergent messages. Our intent for this program was to close potential gaps within the communication chain at our outpatient surgical clinics, improve overall communication between clinicians and their patients, and improve both patient and employee satisfaction.
The thresholds for shunting CSF in children with asymptomatic hydrocephalus are unclear; there are neither guidelines nor sufficient research to determine what degree of hydrocephalus should be ...treated. The authors hypothesize that 1) pediatric neurosurgeons currently have high thresholds for recommending treatment for these children, but 2) there is significant variability among these treatment thresholds.
The authors surveyed attendees of the Joint Pediatric Neurosurgery Section meeting in Spokane, Washington, in December 2008, regarding their treatment thresholds for 22 clinical scenarios. Each participant was provided an illustration of 5 imaging studies (3 slices each) showing progressively larger ventricles. For each scenario, respondents were asked to indicate the minimum ventricular size they would treat, if any. Responses were quantified from 1 to 6 from smaller to larger, with 6 being no treatment, and a mean theoretical treated ventricular size (MTTVS) was calculated for each scenario.
Respondents were relatively conservative in recommending treatment, with MTTVSs of 3.7-6.0; in 13 scenarios, the MTTVS was greater than 5.0 (larger than the largest presented ventricular size). For scenarios in which a mean frontooccipital ratio could be calculated, the value ranged from 0.55 to 0.67 (moderate to severe hydrocephalus). Although there were clear majority responses for each scenario, there was also significant variability. There were no patterns of association with the respondent's age, training, board certification, or type or location of practice.
This study demonstrates that pediatric neurosurgeons' thresholds for treating asymptomatic children with hydrocephalus are generally high, but there is also significant variability.
Dysembryoplastic neuroepithelial tumors (DNETs) are rare, generally benign, mixed neuronal-glial neoplasms occurring most often between 10 and 14 years of age. These lesions are classically ...cortically based and solitary, found preferentially in the temporal lobe, and most commonly present with seizures. On magnetic resonance imaging (MRI), these lesions are generally cystic and have variable contrast enhancement, which, when present, often involves the periphery. Rarely, lesions followed radiographically may demonstrate delayed contrast enhancement. Here, we present a case of multifocal DNETs involving the cerebellum that demonstrated delayed contrast enhancement. In addition, these occurred in a patient with Noonan syndrome (NS), a “RASopathy” disorder associated with low-grade glial and glioneuronal tumors. We present a summary of all previously reported cases of cerebellar DNETs. Our patient was successfully treated surgically and is doing well clinically, now one year status post his last procedure, and is being closely monitored with serial MRIs for progression. Gross total resection is often curative without adjuvant therapy for most DNETs. Our case emphasizes the importance of radiographic surveillance, as multifocality and recurrence may necessitate more than one procedure. Lastly, clinicians should be suspicious for DNETs and other low-grade glial tumors when treating patients with NS, acknowledging their predisposition for multifocal involvement and atypical presentations.
ETV is emerging as the treatment of choice for aqueductal stenosis caused by anatomic, inflammatory, and selected neoplastic etiologies. The technique has also proven useful in the pathologic ...diagnosis and treatment of these conditions. Long-term results of this procedure and comparison to standard shunting procedures are necessary to define indications for patients with pathologic findings in the intermediate response groups. Development of new studies for pre-operative assessment of CSF absorptive capacity and quantitative postoperative measures of ventriculostomy function would be invaluable additions to our ability to assess candidates for this procedure and their eventual outcome. Further study and technical refinements will, no doubt, lead to many more potential uses for these procedures in the treatment of hydrocephalus and its associated etiologies. The challenge for neuro-surgeons will be to define the operative indications and outcomes, while refining techniques for safely performing these useful procedures.
Accuracy in documenting clinical care is becoming increasingly important; it can greatly affect the success of a neurosurgery department. As patient outcomes are being more rigorously monitored, ...inaccurate documentation of patient variables may present a distorted picture of the severity of illness (SOI) of the patients and adversely affect observed versus expected mortality ratios and hospital reimbursement. Just as accuracy of coding is important for generating professional revenue, accuracy of documentation is important for generating technical revenue. The aim of this study was to evaluate the impact of an educational intervention on the documentation of patient comorbidities as well as its impact on quality metrics and hospital margin per case.
All patients who were discharged from the Department of Neurosurgery of the Penn State Milton S. Hershey Medical Center between November 2009 and June 2012 were evaluated. An educational intervention to improve documentation was implemented and evaluated, and the next 16 months, starting in March 2011, were used for comparison with the previous 16 months in regard to All Patient Refined Diagnosis-Related Group (APR-DRG) weight, SOI, risk of mortality (ROM), case mix index (CMI), and margin per discharge.
The APR-DRG weight was corrected from 2.123 ± 0.140 to 2.514 ± 0.224; the SOI was corrected from 1.8638 ± 0.0855 to 2.154 ± 0.130; the ROM was corrected from 1.5106 ± 0.0884 to 1.801 ± 0.117; and the CMI was corrected from 2.429 ± 0.153 to 2.825 ± 0.232, and as a result the average margin per discharge improved by 42.2%. The mean values are expressed ± SD throughout.
A simple educational intervention can have a significant impact on documentation accuracy, quality metrics, and revenue generation in an academic neurosurgery department.
Primary intramedullary spinal cord germinoma (PISCG) is an exceedingly rare diagnosis, with fewer than 30 cases reported in the literature. It is even less common in the pediatric population. ...Usually, initial imaging at patient presentation reveals a mass. The authors describe the unique case of a child whose initial imaging showed only focal spinal cord atrophy, which was the earliest sign of a slowly growing intramedullary lesion that was eventually proven via biopsy to represent a PISCG. The authors outline this child's diagnostically challenging presentation, review the events leading up to a diagnosis, briefly discuss PISCG, and summarize their recommendations for other physicians who may encounter a similar case. They assert that PISCG should be considered as a rare entity in the differential diagnosis of progressive spinal cord dysfunction even in the absence of an MRI abnormality of an intrinsic spinal cord mass, especially if there is unexplained focal atrophy of the cord.
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