Latency of intracranial germ cell tumors and diagnosis delay

Background

Intracranial germ cell tumors (GCTs) frequently take an insidious clinical course before diagnosis. To date, clinical latency has been discussed in the context of germinoma in the suprasellar area and basal ganglia.

Objective

In this study, we classified the clinical latency of intracranial GCTs into three categories and described their characteristics in order to understand the full spectrum of the phenomenon.

Methods

In a cohort of 181 patients with intracranial GCTs, 17 patients had a delayed diagnosis of more than 3 months (90 days) from the initial brain magnetic resonance imaging to the definitive GCT diagnosis. Clinical records and radiological data of the patients were reviewed.

Results

The patients with a delayed diagnosis were categorized into three groups according to their tumor location: suprasellar (nine patients), basal ganglia (six patients), and pineal (two patients). Initial symptomatology corresponded with the tumor location: central diabetes insipidus for the suprasellar group, hemiparesis for the basal ganglia group, and precocious puberty for the pineal group. The overall survival of patients with germinoma and delayed diagnosis was significantly shorter than that of patients who were diagnosed within 3 months (P?=?0.002).

Conclusions

Clinical latency and delayed diagnosis are not restricted to germinomas in the suprasellar area and basal ganglia; they are canonical features of intracranial GCTs including pineal non-germinomatous GCTs. Early detection and proactive diagnosis of these tumors are required because diagnosis delay may negatively influence patient survival.     

Second primary brain tumors following cranial irradiation for pediatric solid brain tumors

Purpose

We describe our institution’s experience with seven patients who developed second brain tumors following cranial irradiation.

Methods

The median age at first irradiation was 8 years (range, 3–20 years). Initial diagnoses were two cases of germinoma, one non-germinomatous germ cell tumor (NGGCT), three cases of medulloblastoma, and one pineal gland tumor (pathology undetermined). All patients received craniospinal irradiation followed by local boost and the median dose to the initial tumor area was 54.0 Gy (range, 49.8–60.6 Gy). Four patients (two medulloblastomas, one germinoma, and one NGGCT) received chemotherapy.

Results

Second brain tumors were diagnosed a median of 114 months (range, 64–203) after initial radiation. Pathologic diagnoses were one glioblastoma, two cases of anaplastic astrocytoma, one medulloblastoma, one low-grade glioma, one high-grade glial tumor, and one atypical meningioma. Five patients underwent surgical resection with subsequent radiotherapy. One anaplastic astrocytoma patient received chemotherapy only following stereotactic biopsy. The meningioma patient was alive 32 months after total resection and radiosurgery for subsequent recurrences. Six patients died within 18 months and most deaths were due to disease progression.

Conclusions

Most patients diagnosed with second brain tumors had received high-dose, large-volume radiotherapy with chemotherapy at a young age. Further studies are required to determine the relationship between radiotherapy/chemotherapy and the development of secondary brain tumors.     

Intracranial germinomas in a father and his son

Background

Primary intracranial germ cell tumors (IGCTs) represent an uncommon category of neoplasms, and familial occurrence is rare. We present the first report of parent-child patients with pathologically confirmed pure germinomas.

Case report

A 36-year-old Japanese man presented with diabetes insipidus and hypopituitarism. Magnetic resonance imaging (MRI) revealed a mass lesion in the pituitary stalk, which was diagnosed as a pure germinoma by open craniotomy tumor biopsy. Seven years later, his 13-year-old son also presented with diabetes insipidus. MRI revealed mass lesions in the pituitary stalk and the pineal region. He underwent endoscopic tumor biopsy for the pineal lesion, which was diagnosed as a pure germinoma. Both the father and his son were treated with combined radiochemotherapeutic regimens and achieved complete remission after one to two cycles of chemotherapy.

Conclusion

Although there have been three previous case reports of familial germinoma, all of these involved sibling pairs. The present report represents the first parent-child cases. This type of familial occurrence suggests the possibility that germline mutations may also be involved in the development of IGCTs.     

The gut or the brain?—gastrointestinal misdiagnoses of infantile brain tumors

Purpose

Central nervous system tumors account for the largest number of cancer deaths in childhood. Brain tumors in infants less than 3 years of age are rare; symptoms and signs are often non-specific. Patent anterior fontanelles/unfused cranial sutures in infants can accommodate rising intracranial pressure without acutely compromising the neurological status. We hypothesize that vomiting as the initial symptom, in infants with brain tumors, can possibly lead to extensive gastrointestinal evaluation, delaying the diagnosis of intracranial pathology.

Methods

We conducted a retrospective chart review of infants less than 3 years of age diagnosed with brain tumors over the period of 4.7 years from February 2008 to October 2012 at Inova Children’s Hospital, Virginia.

Results

We identified three of 21 patients (14.3 %) who presented with vomiting and underwent initial or extensive abdominal imaging investigations. All patients were relatively young (median age, 5.4 months). Working diagnoses were pyloric stenosis, viral gastritis, or gastroesophageal reflux. All patients eventually had computed tomography of the head to rule out increased intracranial pressure and were found to have large brain tumors with obstructive hydrocephalus. Tumor locations were cerebral hemispheres (2/3) and posterior fossa (1/3). All patients had biologically aggressive high-grade tumors (glioblastoma multiforme, atypical teratoid rhabdoid tumor, and anaplastic/large cell medulloblastoma) and died within weeks of diagnosis.

Conclusions

Our study highlights a clinical challenge of persistent vomiting in infants, which in the absence of convincing gastrointestinal pathology after evaluation should raise the physician’s suspicion of an underlying intracranial pathology even if neurological features are absent.     

Intracranial germinoma: clinical and MRI findings in 56 patients

Introduction

Intracranial germinoma is a rare malignant tumor, only constituting 0.5–2.0% of all primary intracranial tumors. It usually occurs in children and young adults, it is highly sensitive to radiotherapy or/and chemotherapy, and is potentially curable without operation. To make correct pre-operative diagnosis and spare surgical intervention, we retrospectively reviewed 56 patients in our hospital, and presented clinical findings and MRI characteristics.

Materials and methods

We reviewed medical records and magnetic resonance imaging (MRI) of 56 patients. Information regarding sex, age at diagnosis, clinical presentation, duration of symptom, and associated neurologic deficits was noted. The tumor location, number of metastasis, size, margin, involved tissues, and characteristics of signal intensity on MRI were analyzed by two experienced radiologists, respectively.

Results

In the series, 57% of intracranial germinoma was located in pineal region, 32% in suprasellar region, and 9% in basal ganglia region. One patient was found germinoma with synchronous lesions in pineal and suprasellar region. Sixty-three percent of patients were male in pineal region, 39% and 100%, respectively, in suprasellar region and basal ganglia region. Age at diagnosis ranged from 3 to 21 years (mean, 12.5 years) with a peak between 10 and 18 years.

Conclusions

Intracranial germinoma has some features in terms of age, sex, clinical presentation, and MRI. These features are in favor of pre-operative diagnosis and thus make for good prognosis.     

Treatment strategies for initially disseminated intracranial germinomas

Purpose

Initially disseminated intracranial germinomas (IDIGs) can be observed in pre-adult and adolescent brain tumor patients. However, the disease prognosis is undetermined, and the method of optimal treatment remains controversial.

Methods

From January 1990 to January 2011, data on 91 intracranial germinoma patients (≤20?years old) were gathered from the Pediatric Brain Tumor database at Taipei Veterans General Hospital. A total of seven patients with a median age of 17.0?years had IDIGs (lesion sites >2), including IDIGs in the ventricular system or the spinal column. Craniospinal irradiation (CSI) plus a primary or metastatic boost was the mainstay strategy for radiotherapy. Six out of a total of seven patients (85.7%) also received systemic chemotherapy (CHT) after radiotherapy. Survivals rates between IDIGs and patients without dissemination were estimated using the Kaplan–Meier method.

Results

The median follow-up time for all seven patients was 67.5?months (range, 10.3–142.3?months). None of the IDIG patients experienced a recurrence or mortality after the completion of treatment. The 5- and 10-year disease-free survival (DFS) between IDIG and non-dissemination patients were 100%, 100%, 93.0% and 78.6%, respectively (p?=?0.339). The 5- and 10-year overall survival (OS) between IDIGs and non-dissemination cases were 100%, 100%, 93.7% and 89.4%, respectively (p?=?0.473).

Conclusions

IDIG patients did not show reduced survival compared to non-dissemination patients if optimal radiotherapy and chemotherapy were used together.     

Pediatric brain tumors in Nigeria: clinical profile, management strategies, and outcome

Introduction

Although modern neuroimaging has facilitated early care of brain tumors in children worldwide, there are, however, few published reports on clinical profile, treatment, and outcome of brain tumors in children from our subregion.

Purpose

We aimed to retrospectively study the clinical profile and outcome of pediatric brain tumors in a tertiary referral center from a developing country.

Methods

Forty pediatric patients with histologically verified brain tumors managed by the authors over a 13-year period (May1994–April 2006) were studied. Patients' data from clinical, radiological, and pathology records were analyzed using the statistical package for social sciences version 16.

Results

The mean age was 9.75 years (range 1–15 years). Twenty-two males, 18 females. Common presenting symptoms were headaches (23 patients, 57.5 %) and seizures (15 patients, 37.5 %). Hyperreflexia (72.5 %) and focal motor deficits (62.5 %) were the most common neurologic signs. The mean interval from onset of symptoms to neurosurgical diagnosis was 13.4 months (95 % CI). All patients had tumor resection, while 11 (27.5 %) patients received adjuvant radiotherapy. Hydrocephalus occurred in 19 (47.5 %) patients and was associated with early presentation (X²?=?10.65, p?<?0.01). Low-grade astrocytoma (25 %) and medulloblastoma (25 %) were the most common tumors. Survival at 1 and 5 years were 56 and 47 %, respectively.

Conclusion

Focal motor signs and elevated intracranial pressure are the salient presenting features of brain tumors in children seen in Nigeria. Those of them with hydrocephalus are likely to present early. The outcome for pediatric brain tumors remains poor.     

Growing teratoma syndrome in intracranial non-germinomatous germ cell tumors (iNGGCTs): a risk for secondary malignant transformation—a report of two cases

Purpose

About 5 % of pediatric intracranial germ cell tumors and 20 % of non-germinomatous germ cell tumors (NGGCT) progress to growing teratoma syndrome (GTS) following chemoradiotherapy. The growing teratoma is thought to arise from the chemotherapy-resistant, teratomatous portion of a germ cell tumor and is commonly benign but may undergo malignant transformation.

Methods

Two pediatric patients whose intracranial NGGCTs progressed to growing teratomas during chemotherapy and later transformed to secondary malignant tumors after partial resection and radiation therapy (RT).

Results

Both tumors were diagnosed by MRI scans and elevated serum and CSF markers. Following normalization of tumor markers with chemotherapy and initial decrease in tumor volume, subsequent imaging showed regrowth during chemotherapy with pathology revealing benign teratoma. RT was administered. Several years following this treatment, further growth was seen with pathology indicating malignant carcinoma in one patient and malignant rhabdomyosarcoma in the other. The patient with carcinoma received palliative care while the patient with the sarcoma received further resection, intensive chemotherapy, and an autologous stem cell transplant and is currently in remission, 36 months since malignant transformation.

Conclusion

Malignant transformation of presumed residual teratoma has been seldom reported. Treatment of NGGCT involves platinum-based chemotherapy with craniospinal RT and boost to the primary site, with cure rates of around 80 %. Teratomas are characteristically chemotherapy and RT resistant and are treated surgically. In the event that residual or growing teratoma is suspected, a complete resection should be considered early in the management as there is a risk of malignant transformation of residual teratoma.     

Pediatric diffuse intrinsic pontine glioma patients from a single center

Background

The prognosis of children with diffuse intrinsic pontine gliomas (DIPG) is dismal. This study aims to evaluate the characteristics and treatment outcome of children with DIPG in a single center.

Methods

We reviewed the outcome of children with DIPG treated at the Oncology Institute of Istanbul University from February 1999 to May 2012.

Results

Fifty children (26 female, 24 male) with the median age of 7 years were analyzed. The median duration of symptoms was 30 days. All patients received radiotherapy (RT). Before the year 2000, 12 patients received only RT. Thirty-eight had concomitant and/or adjuvant chemotherapy with RT. Between 2000 and 2004, 17 patients received cis-platinum or vincristine as sensitizers during RT and CCNU + vincristine combination after RT. Since 2004, 21 patients received temozolomide (TMZ) concomitantly during RT and as adjuvant chemotherapy after RT. The median survival time of all patients was 13 months (1–160 months). Patients receiving RT + TMZ had a significantly higher overall survival than patients with only RT (p?=?0.018). Patients receiving RT + chemotherapy other than TMZ also had a significantly higher overall survival than patients receiving only RT (p?=?0.013). Patients receiving RT + TMZ + and chemotherapy other than TMZ had a significantly higher survival than patients receiving only RT (p?=?0.005).

Conclusion

In our series, patients receiving RT + TMZ and also patients receiving RT + chemotherapy other than TMZ had a significantly higher overall survival than patients treated with only RT. Hence, administering chemotherapy during and after RT seems to prolong survival in some DIPG patients.     

An unusual case of primary central nervous system germinoma with meningeal dissemination

Purpose

Central nervous system germinomas often extend or disseminate into the ventricular and subarachnoid space. We present a case of primary central nervous system germinoma consisting mainly of meningeal dissemination, which is extremely unusual.

Case report

A 16-year-old boy presented with pure germinoma, manifesting as anorexia and headache for 10 days. Radiological examinations revealed a large mass lesion in the posterior fossa and a small mass lesion on the pineal lobule with thickening of the tentorium cerebelli and falx. The patient underwent partial removal of the infratentorial tumor via open surgery. Intraoperative findings indicated extension of the dural and supracerebellar mass lesions into the epiarachnoid space. Three chemotherapy courses comprising ifosfamide, etoposide, and cisplatin were administered after the surgery. Craniospinal irradiation was administered subsequently. The adjuvant therapy had a complete response.

Conclusion

Central nervous system germinoma cells may have similar compatibility to meningeal components as they do to cerebrospinal fluid or the ventricular system. If there is a possibility of the tumor extending into the subdural space during surgical intervention or other events, a range of postdiagnostic irradiation should be considered to cover a wider range.     

Intracranial Pressure Dose and Outcome in Traumatic Brain Injury

Objective

Detecting and treating elevated intracranial pressure (ICP) is a cornerstone of management in patients with severe traumatic brain injury. The aim of this study was to determine the association between area under the curve measurement of elevated ICP and clinical outcome.

Methods

Single center observational study using prospectively collected data at a University hospital, level one-trauma center. Sixty prospective patients with severe traumatic brain injury were prospectively enrolled over a 2-year period. Intracranial pressure measurements were captured using a real-time automated, high resolution vital signs data recording system. Mortality and functional outcome were assessed at 30 days, 3 and 6 months using Extended Glasgow Outcome Scale.

Results

Increasing elevated intracranial pressure time dose was associated with mortality (OR 1.08; 95 % confidence interval [CI], 1.01–1.15, p = 0.03) and poor functional outcome at 3 (OR 1.04; CI 1.00–1.07, p = 0.03) and 6 months (1.04; CI 1.01–1.08, p = 0.02). However, there was no association between episodic ICP data and outcome.

Conclusions

These results suggest that pressure time dose measurement of intracranial pressure may be used to predict outcome in severe traumatic brain injury and may be a candidate biomarker in this disease.     

Transventricular endoscopic biopsy of suprasellar tumors: a pediatric case series

Background and purpose

The purpose of this study was to investigate the efficacy and safety of transventricular neuroendoscopic biopsies in pediatric patients with suprasellar tumors.

Methods

Twenty-three pediatric patients (12 males and 11 females) with suprasellar tumors underwent transventricular neuroendoscopic biopsy at our institute by a single surgeon from 2000 to 2011. Neuronavigation has been combined with endoscopic procedures since 2008. Neuroendoscopic biopsies were performed to verify the histopathological diagnosis of neoplasms and to plan appropriate treatment strategies.

Results

Neuroendoscopic biopsy specimens were appropriate for diagnosis in 22 of the 23 patients (95.7 %) and revealed 14 germ cell tumors (12 germinomas, one choriocarcinoma, and one immature teratoma), seven astrocytomas, and one craniopharyngioma. Subsequent treatment modalities including chemotherapy, radiation therapy, or microscopic surgery were determined according to the pathological findings. Seventeen of the 23 patients (73.9 %) showed ventriculomegaly. Among them, ventriculomegaly in 14 patients was resolved after an endoscopic procedure and/or adjuvant chemotherapy, but the remaining three patients (17.6 %) required a ventriculoperitoneal shunt to relieve the ventriculomegaly. The pathologic diagnosis of these three patients was uniformly a large astrocytoma. Navigational tracking was helpful to enter small ventricles and the narrow foramen of Monro in patients without hydrocephalus. No mortalities were related to the procedures, but three transient diabetes insipidus (13.0 %) cases occurred but fully recovered before the patients received adjuvant therapy.

Conclusion

Endoscopic biopsy is feasible and shows acceptable operation-related complications to obtain tissue from suprasellar tumors in pediatric patients. Navigation-assisted neuroendoscopic procedure improves the accuracy of the endoscopic approach. An associated endoscopic procedure can resolve hydrocephalus, but it has limitations with large ventricle-occupying tumors.     

Brain stem tumors in children and adolescents: single institutional experience

Purpose

Pediatric brain stem tumors (BsT) are a heterogeneous group of diseases. Our aim was to analyze our experience to find out prognostic factors.

Method

A retrospective study with BsT patients was performed. Imaging characteristics, extension of surgery, pathology, and adjuvant therapy were analyzed and correlated with overall survival (OS) and progression-free survival (PFS) as outcome measures.

Result

Since 1980 to 2010, we analyzed 65 BsT patients, 41 of them girls (63 %), median age of 8 years (range 13.9?months to 17.6 years). Twenty-two patients (33.8 %) had diffuse intrinsic pontine gliomas (DIPG) and 43 (66.2 %) presented with focal BsT. Histology was available in 42 patients; the most frequent is low-grade glioma in 24/42 patients (57 %). DIPG’s histology (obtained usually at necropsy) confirmed five high-grade gliomas. After median follow-up of 49.3 months (0.5–175 months), 20/22 DIPG patients have died (90.9 %), while 27/43 with focal tumors were alive (62.8 %). Variables related to outcome were histology (better for low-grade glioma (LGG) OS p?<?0.001), surgery (better if operated OS p?<?0.001), and adjuvant therapy (worse if given, PFS p?=?0.001, OS p?=?0.024). The outcome for DIPG was dismal, median OS/EFS of 14.2/9.4 months, significantly worse than focal BsT (p?=?0.000), while OS/EFS was 122.8/87.2 months for focal intrinsic, 88.2/47.1 months for exophytic, and 124.4/54 months for cervico–medullary tumors: no differences were found among them, except the histology (OS p?<?0.001 for low-grade vs high-grade tumors).

Conclusion

BsT in children comprised two different groups: diffuse (DIPG) and focal gliomas. The DIPGs continue having a dismal prognosis, needing new approaches, while focal tumors including LGG have better prognosis.     

The management of bifocal intracranial germinoma in children

Objectives

Bifocal intracranial germinoma (BFG) is a tumour of the pineal and suprasellar regions, which is known to be highly radiosensitive. The definitive treatment and outcomes are not well defined, particularly in the paediatric population. We review our series of purely paediatric cases from a single institution and combine them with the limited reports in the literature to determine the results of different management strategies.

Methods

Four patients were treated at our institution with a median age of 15.3 years. A literature search identified a further 38 paediatric cases with a median age of 12.9 years.

Results

All four patients had normal serum and CSF tumour markers. One patient had a diagnosis made based on imaging findings of bifocal pineal and suprasellar lesions presenting with diabetes insipidus. Three others underwent biopsy. All had craniospinal radiotherapy, which has led to complete cure with no cases of progression at a mean follow-up of 3 years. The most common treatment modality in published cases is craniospinal irradiation. In the cases reviewed, limited radiation treatments (whole ventricle or focal) combined with chemotherapy regimens yield comparable outcomes where there is no spinal dissemination. Outcomes do not appear to be altered by biopsy in cases with negative tumour markers and characteristic imaging appearances.

Conclusion

Patients who present with a classic appearance of germinoma, negative tumour markers and diabetes insipidus probably do not require a biopsy to confirm the diagnosis. No evidence of dissemination may obviate the need for craniospinal irradiation, but good quality long-term follow-up data are required to demonstrate the benefits of combined focal radiotherapy and chemotherapy regimes.     

Survival of children with malignant brain tumors receiving valproate: a retrospective study

Purpose

The treatment of pediatric patients with malignant brain tumors has evolved considerably in the past decades. However, results are still unsatisfactory for some patients. Valproate has been shown to positively affect the survival of adult glioblastoma patients. We have been giving prophylactic antiepileptic drugs to newly diagnosed children with brain tumors. Since then, we noted a trend towards a better survival from our patients. In order to study this, we performed a retrospective evaluation in our institution.

Methods

Standard survival analysis was used, calculating survival until death by all causes or censoring. Comparisons were made by Cox’s proportional hazards model regression.

Results

Between 2000 and 2010, 94 patients were treated (12 with high-grade gliomas, 56 medulloblastomas, and 26 ependymomas); median and mean ages were 7.7 and 7.8 years. Median follow-up was 60 months (35 for treated and 109 for untreated patients). Of these, 47 received valproate 10–15 mg/kg/day every 8–12 h and 47 did not. Patients who received valproate had a median survival of 34 months, whereas the other group had a median survival of 24 months (hazard ratios?=?0.99, 0.57–1.75, p?=?0.99).

Conclusions

These results do not prove that valproate prophylactic treatment in pediatric patients with malignant brain tumors had an influence on their survival. However, our cohort showed an effect of higher size than the recent European Organization for Research and Treatment of Cancer trial analysis, even though not significant. Clinical trials with valproate in pediatric malignant brain tumors should be carefully planned, in order to detect a possible effect of this drug in survival.     

The pathogenesis of intracranial growing teratoma syndrome: proliferation of tumor cells or formation of multiple expanding cysts? Two case reports and review of the literature

Purpose

Growing teratoma syndrome (GTS) is a rare clinical phenomenon defined as the paradoxical growth of mature teratoma components during or after chemotherapy. The mechanism of this phenomenon is not well understood. We present two cases of pineal mixed germ cell tumors that exhibited the similar course to GTS and speculate its pathogenesis.

Case report

The first case was accompanied by slightly elevated alpha-fetoprotein (8.8 ng/ml; normal <6.6 ng/ml). The tumor rapidly grew from 1.5 to 2.7 cm in diameter within 4 weeks. Despite this rapid preoperative growth, thorough pathological investigation found only mature teratoma components along with multiple micro- and macro-cysts. The other case was diagnosed as a pure germinoma based on biopsy and serological examinations. During three courses of chemotherapy, this tumor presented a honeycomb-like appearance on magnetic resonance (MR) images and an exceptionally rapid enlargement. Second-look surgery confirmed the histological diagnosis of a mature teratoma. In both cases, meticulous pathological examination of all whole tumor sections revealed no malignant histological features, and the MIB-1 labeling indices were too low to account for the extremely rapid tumor growth. Instead, both MR images and histological findings demonstrated a predominant formation of multiple cysts.

Conclusion

We speculate that this paradoxical growth might not be tumorous proliferation but instead the formation and expansion of multiple cysts inside mature teratoma components and that the presence or absence of growth might be a subsidiary phenomenon. Our hypothesis appears consistent with the characteristic radiological findings of GTS reported in the literature.     

“Groans less, seems more comfortable:” Harvey Cushing's redefinition of success in the operative treatment of pediatric intracranial lesions

Background

Challenges to diagnosing and localizing intracranial lesions in pediatric patients were immense during the advent of neurosurgery. For patients with suspected intracranial tumors, but with negative findings on operation, there were few options. The role of palliative surgical interventions, from decompressive craniectomies to lumbar and ventricular punctures, is not well-represented in the literature during this early stage.

Objective

To review Harvey Cushing's original surgical files and analyze his use of decompressive procedures in pediatric patients with suspected intracranial tumors, with negative findings during operative interventions.

Methods

Following IRB approval, and through the courtesy of the Alan Mason Chesney Archives, we reviewed the Johns Hopkins Hospital surgical files from 1896 to 1912. Patients, ≤18 years old, presenting with suspected intracranial tumors, undergoing surgical intervention by Cushing, without discovery of intracranial pathology, were selected for further analysis.

Results

Of the 23 pediatric patients selected, 17 were male. The mean age was 10.6 years. Cushing used three main operative approaches: infratentorial/suboccipital, subtemporal, and hemisphere flaps. Post-operative condition was improved in 13 and unchanged in three patients. Seven patients died during their inpatient stay. The mean time to follow-up was 34.79 months; the mean time to death was 11.9 months.

Conclusions

These examples illustrate Cushing's commitment to improving quality of life in patients, offering decompressive procedures, including craniectomies as well as cerebrospinal fluid drainage in the operating room and at the bedside, when attempts at localizing and resecting of suspected tumors were unsuccessful.     

Intermittent Versus Continuous Cerebrospinal Fluid Drainage Management in Adult Severe Traumatic Brain Injury: Assessment of Intracranial Pressure Burden

Introduction

There is clinical equipoise regarding whether neurointensive care unit management of external ventricular drains (EVD) in severe traumatic brain injury (TBI) should involve an open EVD, with continuous drainage of cerebrospinal fluid (CSF), versus a closed EVD, with intermittent opening as necessary to drain CSF. In a matched cohort design, we assessed the relative impact of continuous versus intermittent CSF drainage on intracranial pressure in the management of adult severe TBI.

Methods

Sixty-two severe TBI patients were assessed. Thirty-one patients managed by open EVD drainage were matched by age, sex, and injury severity (initial Glasgow Coma Scale (GCS) score) to 31 patients treated with a closed EVD drainage. Patients in the open EVD group also had a parenchymal intracranial pressure (ICP) monitor placed through an adjacent burr hole, allowing real-time recording of ICP. Hourly ICP and other pertinent data, such as length of stay in intensive care unit (LOS-ICU), Injury Severity Score, and survival status, were extracted from our prospective database.

Results

With age, injury severity (initial GCS score), and neurosurgical intervention adjusted for, there was a statistically significant difference of 5.66 mmHg in mean ICP (p < 0.0001) between the open and the closed EVD groups, with the closed EVD group exhibiting greater mean ICP. ICP burden (ICP ≥ 20 mmHg) was shown to be significantly higher in the intermittent EVD group (p = 0.0002) in comparison with the continuous EVD group.

Conclusion

Continuous CSF drainage via an open EVD seemed to be associated with more effective ICP control in the management of adult severe TBI.     

Chiari I malformation after cranial radiation therapy in childhood: a dynamic process associated with changes in clival growth

Object

The small posterior fossa is believed to be relevant to the development of Chiari I malformation (CMI). In this study, we evaluated children with supratentorial tumors developing CMI after radiation therapy (RT) that involved the skull base. Changes in clivus and supraocciput growth rate were correlated with tonsillar herniation.

Methods

Ten children who underwent RT for supratentorial tumors at St. Jude Children's Research Hospital (1994–2008) developed CMI on follow-up magnetic resonance imaging (MRI). Four other patients with supratentorial tumor and CMI who did not receive RT were identified. Length of the supraocciput, basisphenoid, and basichondrium and extent of tonsillar herniation were measured on serial midsagittal MRI.

Results

Over the median follow-up of 85.5 months, basisphenoid annual growth rate was significantly lower in children receiving RT for suprasellar tumor (n?=?8) than those who did not (p?=?0.04). Growth of clivus and basisphenoid was significantly lower in the first 12 months after RT in children who received RT for suprasellar tumor (p?=?0.03 and p?=?0.04, respectively). In these patients, tonsillar herniation increased over 24 months after RT, reaching maximal descent at a mean of 20.2 months; this resolved as clival growth returned to normal. No patient was symptomatic.

Conclusions

In these children, restriction of clival growth occurred after RT for suprasellar tumors. Changes in clival growth were associated with changes in the extent of tonsillar herniation. This supports the importance of the small posterior fossa and reduced clival length in the etiology of CMI.

     

Intracranial germ cell tumors: pathobiological and immunohistochemical aspects of 70 cases

Seventy cases of histologically verified intracranial germ cell tumor were reviewed: 43 germinomas, 16 immature teratomas, seven mature teratomas, two embryonal carcinomas, one choriocarcinoma, and one yolk sac tumor. The male-to-female ratio was 2.6:1. The average age was 19 years in patients with germinoma, 11 years in patients with immature teratoma, and 17 years in patients with mature teratoma. Duration of symptoms averaged 19 months for germinoma, three months for immature teratoma, and 11 months for mature teratoma. Sixty-six lesions were located in the midline. Fifty-eight percent of the germinomas arose anterior to the pineal gland, whereas 29% of the immature and 14% of the mature teratomas were located anteriorly. The histologic appearance of the germinomas was indistinguishable from that of the usual testicular seminoma. The immature teratomas contained tissue from all three germ layers and exhibited morphologic features of fetal tissue. Of 14 immature teratomas, seven contained, in addition, foci of other malignant germ cell elements; thus, there were two teratocarcinomas, two lesions with germinoma and immature teratoma, two lesions with extensive rhabdomyoblastic differentiation in an immature teratoma, and one lesion with both germinoma and embryonal carcinoma in addition to immature teratoma. The seven mature teratomas consisted of fully differentiated epithelial and mesenchymal tissues. In 23 cases, immunoperoxidase stains for human chorionic gonadotropin (HCG), alpha-fetoprotein (AFP), and carcino-embryonic antigen (CEA) revealed patterns which, with minor exceptions, were essentially identical to those found in genital germ cell lesions. Survival was longest for patients with germinomas. In classifying germ cell tumors of the central nervous system, the World Health Organization's (WHO) classification of testicular germ cell tumors is preferable to its present classification of intracranial germ cell tumors.