Concurrent chemotherapy and reduced - dose cranial spinal irradiation followed by conformal posterior fossa tumor bed boost for average - risk medulloblastoma: efficacy and patterns of failure

PURPOSE:To review the efficacy and patterns of failure in average-risk medulloblastoma patients treated withconcurrent chemotherapy and reduced-dose cranial spinal irradiation and a conformal tumor bed boost.METH-ODS AND MATERIALS:Thirty-three patients with average risk(defined as<==1.5 cm(2)of residual tumorafter resection,age>3 years,and no involvement of the cerebrospinal fluid or spine)medulloblastoma werediagnosed at our institution between January 1994 and December 2001.They were enrolled in an institutional

Duration of Preoperative External Ventricular Drain in Pediatric Posterior Fossa Tumors—Does It Matter?

Objective: Aim of the study was to assess the duration of preoperative external ventricular drain (EVD) as a predictor for permanent cerebrospinal fluid (CSF) diversion in pediatric posterior fossa tumors. Methods: The study was conducted in the Department of Pediatric Neurosurgery, National Neuroscience Institute, King Fahad Medical City, Riyadh, Saudi Arabia from January 2010 to December 2013. The data were collected retrospectively. The patients who had emergency insertion of external ventricular drain (EVD) due to hydrocephalus before the definitive posterior fossa tumor surgery were included in the study. Results: The preoperative emergency insertion of EVD was done in 38 patients with pediatric posterior fossa tumors. The patients were divided into two groups. Group A consists of those patients who had duration of preoperative EVD equal or less than 7 days. Group B includes those patients who had duration of preoperative EVD more than 7 days. Group A has 16 patients while Group B has 22 patients. The predominant clinical feature was symptoms and signs of raised intracranial pressure (ICP) only. Infection related to EVD was seen in seven patients. Ventriculoperitoneal (VP) shunt was required in 31.25% of Group A patients and 18.18% of Group B patients (p value = 0.35). Overall shunt rate was 23.68%. Conclusion: There are well known number of factors that can determine the need of permanent CSF diversion in patients with posterior fossa tumors. However, there is no effect of preoperative duration of EVD in determining the requirement of postoperative VP shunt.

Atypical presentation of a posterior fossa tumour:A case report

BACKGROUND We described a case of a patient with a meningioma in the posterior fossa presenting atypically with an isolated unilateral vocal cord palsy causing severe respiratory distress.This is of interest as the patient had no other symptomatology,especially given the size of the mass,which would typically cause a pressure effect leading to neurological and auditory symptoms.CASE SUMMARY This case report described a 48-year-old male who was married with two children and employed as a car guard.He had a medical history of asthma for the past 10 years controlled with an as-needed beta 2 agonist metered dose inhaler.He initially presented to our facility with severe respiratory distress.He reported a 1-wk history of shortness of breath and wheezing that was not relieved by his bronchodilator.He had no constitutional symptoms or impairment of hearing.On clinical examination,the patient’s chest was“silent.”Our initial assessment was status asthmaticus with type 2 respiratory failure,based on the history of asthma,a“silent chest,”and the arterial blood gas results.CONCLUSION A posterior fossa meningioma of such a large size and with extensive infiltration rarely presents with an isolated unilateral vocal cord palsy.The patient’s chief presenting feature was severe respiratory distress,which combined with his background medical history of asthma,was misleading.Clinicians should thus consider meningioma as a differential diagnosis for a unilateral vocal cord palsy even without audiology involvement.

Primary Endodermal Sinus Tumor in the Posterior Cranial Fossa:Clinical Analysis of 7 Cases

Objective To clarify the clinical features,therapeutic method and outcomes of the primary endodermal sinus tumors(ESTs)in the posterior cranial fossa.Methods The English literatures on EST in the posterior cranial fossa were retrieved from PubMed and reviewed.And a 4-year-old boy diagnosed with EST in our hospital was reported.The clinical manifestations,therapy,pathologic features,and prognosis of these cases were analyzed.Results Only seven cases of the ESTs in the posterior cranial fossa were enrolled in this review,including six cases searched from the PubMed and one case from our hospital.Six patients were boy and one patient’s gender was not available from the report.Ages ranged from 1 to 5 years(mean 3.14 years).The mean tumor size in our cohort was 4.4 cm.Six cases came from East Asia.Schiller-Duval bodies were found in all seven neoplasms.All tumors were positive for alpha-fetoprotein.The alpha-fetoprotein level in serum was increased to a very high level before therapy and depressed quickly after the effective chemotherapy.The mean follow-up time was 24.4 months(range 5-52 months).Six tumors were totally removed,and four of them recurred.Three cases died including one whose tumor was partially removed.Conclusions The serum alpha-fetoprotein level is well correlated with the severity of the tumor.A combination of operation and chemotherapy might be the effective management for EST in the posterior cranial fossa.The prognosis of extragonadal intracranial EST is poor.

Imaging of the Extra-axial Tumors and Tumor-like Lesions Involving both Middle and Posterior Cranial Fossae: Classification and Diagnosis

Objective: To study the imaging features of extra-axial tumors and tumor-likelesions involving both middle and posterior cranial fossae and to make a classification. Methods:Sixty cases of pathologically confirmed extra-axil tumors and tumor-like lesions involving bothmiddle and posterior cranial fossae were analyzed. They were divided into central and lateral types,the latter of which were subdivided into three types: middle cranial fossae type, posterior cranialfossae type and the over-riding type. The constitution and imaging features of each type wereanalyzed. Results: There were 12 cases of central type, including chordoma (n=5), pituitary adenoma(n=3), nasopharyngeal carcinoma (n=2), craniopharyn-gioma (n=1) and meningioma (n=l). 48 cases oflateral type including trigeminal nerve tumors (n=14), meningioma (n=12), epidermoid cyst (n=11),dural cavernous hemangioma (n=4), dermoid cyst (n=2), metastasis (n=2), hemangiopericytoma (n=1),paraganglioma of glonius jugular (n=1) and nasopharyngeal carcinoma (n=1). Each type of the lesionshad its own shape features, some of which were characteristic for some specific tumors. Most of thetumors and tumor-like lesions could be qualitatively diagnosed according to their imagingcharacteristics and the extent of the lesions could be defined definitely. Conclusion: It is helpfulto categorize extra-axial tumors and tumor-like lesions involving both middle and posterior cranialfossae according to their location for qualitative diagnosis and description of the extent of theselesions. It is of great clinical value in providing more precise and thorough imaging informationfor planning therapeutic methods and route of operation.

The Microscopic Surgical Treatment for Tumor of Posterior Cranial Fossa in Children

OBJECTIVE To analyze and discuss about the clinicalcharacteristics, pathological types, surgical modalities andtechniques, and postoperative complications in children withtumor of posterior cranial fossa .METHODS Retrospective study was conducted on 102 cases ofpediatric tumor of posterior cranial fossa, admitted and treated inour hospital during the period of January 1996 to January 2007.All patients underwent microscopic surgical treatment. Fifty-eight were male and 44 cases were female. The age ranged from 9months to 14 years old, with an average of 6.1 ± 0.5 of age. CranialCT or MRI examination was conducted before and after thesurgery on all patients.RESULTS The primary manifestations for this group of patientswere increased intracranial pressure and/or ataxia. Postoperativepathological diagnoses showed: 46 cases of medulloblastoma, 43cases of astrocytoma, 11 cases of ependymoma (including 1 caseof degenerative ependymoma), 1 case of dermoid cyst, and 1 caseof teratoma. In this group of the patients, radical surgery wasused in 68 cases and subtotal surgical removal used in 31 cases,while surgical removal of large section was performed on 3 cases.There were no deaths from surgery reported. Ninety-one casesshowed significant symptomatic improvement when comparedwith preoperative conditions, while 11 cases showed either noimprovement or more severely affected afterward. For 6 cases,postoperative ventriculoperitoneal shunt was performed within7 days to 2 months after the surgery. Sixty-three patients gainedfollow-up for 3 to 60 months in duration. Thirty-nine patientsregained normal life and were able to learn well, while there were7 patients who could not live normally on their own. During thefollow-up period, there were 17 cases of recurrence and 7 casesof death. In 23 cases of medulloblastoma in children with age of3 years old or above, 2 cases who underwent surgical removal ofintracranial ependymoma received small dosage of postoperativeX-ray radiotherapy on the the brain and spinal cord. Nine casesof medulloblastoma in children under age of 3 and 17 cases ofastrocytoma diagnosed after the surgery received chemotherapyof Carmustine.CONCLUSION Medulloblastomas and astrocytomas werethe most common types of pediatric tumor of posterior cranialfossa, right followed by ependymoma, and dermoid cysts andteratomas were rare. Early correct diagnosis, proper selection ofappropriate surgical modality and the surgical margin, propertreatment of postoperative complications, and the selecting rightradiotherapy or chemotherapy were the key factors in influencingthe prognostic outcome of children with tumor of posterior cranialfossa.

Peripheral primitive neuroectodermal tumor of the posterior mediastinum: A case report

Peripheral primitive neuroectodermal tumor(pPNET) is an extremely rare disease entity of malignant tumors belonging to the Ewing sarcoma family that usually occurs in children and adolescents. We describe a 41-year-old female who presented with right upper abdominal pain. Surgical resection and biopsy revealed small round-cell tumor. Combined with immunohistochemical analysis, pPNET was diagnosed. No evidence of recurrence was noted at 18 months postoperatively. Even thought pPNET is a highly malignant tumor, Wide tumor-free resection and multi-agent chemotherapy can also obtain good clinical outcomes.

Treatment implications of posterior fossa ependymoma subgroups

Posterior fossa ependymoma comprises two distinct molecular entities,ependymoma_posterior fossa A(EPN_PFA)and ependymoma_posterior fossa B(EPN_PFB),with differentiable gene expression profiles.As yet,the response of the two entities to treatment is unclear.To determine the relationship between the two molecular subgroups of posterior fossa ependymoma and treatment,we studied a cohort of 820 patients with molecularly profiled,clinically annotated posterior fossa ependymomas.We found that the strongest predictor of poor outcome in patients with posterior fossa ependymoma across the entire age spectrum was molecular subgroup EPN_PFA,which was recently reported in the paper entitled "Therapeutic impact of cytoreductive surgery and irradiation of posterior fossa ependymoma in the molecular era:a retrospective multicohort analysis" in the Journal ofClinical Oncology.Patients with incompletely resected EPN_PFA tumors had a very poor outcome despite receiving adjuvant radiation therapy,whereas a substantial proportion of patients with EPN_PFB tumors can be cured with surgery alone.

Inflammatory Myofibroblastic Tumor Mimicking Malignant Meningioma in the Middle Cranial Fossa:a Case Report

INFLAMMATORY myofibroblastic tumor（IMT）,alsoknown as inflammatory pseudotumor or plasma cellgranulomas,is an uncommon fibro-inflammatorylesion which is composed of inflammatory cells andmyofibroblastic spindle cells.1Its pathogenesis is still un-known.The tumor commonly occurs in the lung,upperrespiratory tract,live,orbit,abdominal membrane,retro-peritoneum,and genitourinary tract.2-4It rarely involvesthe central nerve system,5,

Ondine's curse after posterior fossa decompression: report of one case

Objective and Methods: A case of Ondine’s curse after posterior fossa decompression was reported. A 33-year-old woman was admitted complaining 2 years of dizziness and progressive worsening of gait disturbances, and 1 year of repetitively ictal nausea. The diagnosis of Arnold-Chiari malformations was established. Results: The patient underwent suboccipital craniectomy and C1-3 laminectomy. In addition to this decompression measure, a fascial graft was sutured between the edges of the dural incision. Postoperatively, the patient lost automatic control of her respiration doting sleep and became hypercapnic and hypoxemic. Assisted ventilation was initiated. Conclusion: Ondine’s curse is possibly due to insensitivity of central chemoreceptors to carbon dioxide resulting in defective control of minute ventilation. Propofol is not recommended in eases of Ondine’s curse, and assisted ventilation until the restoration of automatic control of respiration can be of value. Oxygen inspiration alone is rather harmful than beneficial.

Surgical resection of intradural extramedullary tumors in the atlantoaxial spine via a posterior approach

BACKGROUND The anatomical features of the atlantoaxial spine increase the difficulty of complete and safe removal of atlantoaxial intradural extramedullary(IDEM)tumors.Studies concerning surgical interventions via a posterior approach are limited.AIM To investigate the safety and efficacy of atlantoaxial IDEM tumor resection using a one-stage posterior approach.METHODS We retrospectively analyzed clinical databases for one-stage atlantoaxial IDEM tumor resection via a posterior approach between January 2008 and January 2018.The analyzed data included tumor position,histopathological type,pre-and postoperative Japanese Orthopedic Association(JOA)scores and Nurick grades,postoperative complication and recurrence status.RESULTS A total of 13 patients who underwent C1-C2 Laminectomy and/or unilateral facetectomy via the posterior approach were enrolled in the study.In all cases reviewed,total tumor resection and concomitant C1-C2 fusion were achieved.The average follow-up was 35.3±6.9 mo(range,26-49 mo).A statistically significant difference was noted between the preoperative JOA score(11.2±1.1)and the score at the last final follow-up(15.6±1.0)(P<0.05).A statistically significant difference was noted between the preoperative Nurick grade(2.3±0.9)and that at the last follow-up(1.2±0.4)(P<0.05).However,no statistically significant difference was noted between the preoperative and last follow-up C1-2 Cobb angle and C2-7 Cobb angle(P>0.05).No mortalities,severe complications or tumor recurrence were observed during the follow-up period.CONCLUSION Total resection of atlantoaxial IDEM tumors is feasible and effective via a posterior approach.Surgical reconstruction should be considered to avoid iatrogenic kyphosis and improve spinal stability and overall clinical outcomes.

Surgical Excision of Ischiorectal Fossa Tumors

Background: Ischiorectal Fossa Tumors are rare and their management has not been protocolized. Patients: We retrospectively review four consecutive cases treated in our department, from January 2015 to July 2015. All of them were discussed in a multidisciplinary team meeting. None of them were secondary to an inflammatory process. Results: A Magnetic Resonance Imaging was performed in all the four patients, and as it was not a malignant diagnosis made in any of them, we proceeded to resecate the lesions. None biopsies were performed and the benign diagnoses were confirmed by the pathologist. All the patients underwent a local posterior or perineal approach, because all the lesions (epidermoid cyst, hamartoma, lipoma and an aggressive angiomyxoma) were localized purely in the ischiorectal fossa, under the levator ani muscle and not invading any adjacent structures. In all of them, an R0 resection was performed. Conclusions: Ischiorectal fossa tumors are rare and there are few cases already published. Non-inflammatory lesions located in the ischiorectal fossa, with none invasion of rectum or levator ani muscle, are mainly benign lesions prone to their complete excision by a posterior approach. Biopsy is not always necessary unless there’s a suspicion of a malignancy or invasion of adjacent structures, and only in that case a biopsy should be made, because in some of them, a neo-adjuvant treatment can be useful to reduce the tumor and to perform an R0 resection.

Modified surgical techniques in total en bloc spondylectomy for thoracic and lumbar tumors with a single posterior approach

Objective To investigate the operation key points,instrument improvement and shortterm effects in total en bloc spondylectomy (TES) via a single posterior approach for thoracic and lumbar tumors.Methods A series of modified

Posterior approach (Kraske procedure) for surgical treatment of presacral tumors

Presacral tumors are rare,but can comprise a great variety of histological types.Congenital tumors are the most common.Once the diagnosis is established,surgical resection is essential because of the potential for malignancy or infection.Previous biopsy is not necessary or may be even harmful.To decide the best surgical approach(abdominal,sacral or combined) an individual and multidisciplinary analysis must be carried out.We report three cases of cystic presacral masses in which a posterior approach(Kraske procedure) enabled complete resection,the only way to decrease local recurrence.All patients had a satisfactory recovery.A brief overview of retrorectal tumors is presented,focusing on classification,clinical presentation,diagnosis and surgical management.

A case of nodular posterior scleritis simulating intraocular tumor

Dear Editor,I am Dr. Yukiko Shibata, from the Department of Ophthalmology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University. I write to present the case of posterior scleritis simulating intraocular tumor.

Extrapineal mature teratoma of the posterior fossa in a child

Introduction: teratomas are non-malignantgerm cell tumors commonly composed of cell types derived from all of the three germ layers. Intracerebral teratomas typically are midline or paraxial lesions located in the pituitary stalk or the pineal region. We report our experience with mature teratomas of the posterior fossa. Case report: we present the case of an 11-year-old caucasian female with progressive headache that caused interrupted sleep. Cerebral magnetic resonance imaging showed a midline lesion in the posterior fossa with mass effect and without contrast enhancement. Anatomic pathology revealed a mature teratoma. Conclusion: differential diagnosis of midline lesions in pediatric patients must include teratomas in spite of being posterior fossa lesions.

Decompressive Craniectomy in Posterior Fossa Ischemic Stroke

Ischemic damage produced in the posterior cerebral territory causes significant morbidity and urgently must be considered if the patient need a surgical attitude. Surgical decompression by suboccipital craniectomy seams to be effective to treat secondary edema due to cerebellar damage or in posterior fossa, when medical treatment is not able to control side effects. We report a clinical case of a patient with a subacute ischemic infarction in the vertebro-basilar territory, with perilesional edema, and a posterior fossa decompressive craniectomy (DC) was carried out.

Condom-Assisted Transurethral Resection: A New Surgical Technique for Urethral Tumor

Backgrounds: It is difficult to develop clear endoscopic field during transurethral resection (TUR) of urethral tumors, because irrigation water easily spills out from the meatus. We have devised a surgical technique to facilitate TUR of urothelial tumors. Methods: A 79-year old man developed urethral tumors in the anterior urethra and fossa navicularis 6 months after TUR of bladder tumor. We covered his penis and the resectoscope with a tip-cut condom. The tip-end of condom and the resectoscope sheath was tightly sealed and irrigation water was filled inside the condom. Results: The urethral tumors were successfully resected under a clear vision by filling irrigation water inside a tip-cut condom that covered over the resectoscope and the penis. Conclusion: This technique may be useful for TUR of anterior urethral tumors, especially tumors at fossa navicularis, which is often associated with difficulty in obtaining clear surgical vision.

Treatment of Pediatric Brain Tumors in Brazzaville (Congo) about a Case Series

Introduction: Tumors of the central nervous system are the most common group of solid neoplasm in children and account for 20% to 25%. They are common in Sub-Saharan countries, despite the insufficiency of histological diagnosis. No study has been performed concerning the pediatric brain tumors in the Republic of Congo. The aim of this study was to describe the conditions of neurosurgical management of pediatric tumors in Brazzaville. Materials and Methods: We performed a retrospective and descriptive study, from January 2014 to December 2017 (48 months), into the neurological unit of the surgical department of Brazzaville. We included all patients aged from 17 years old and below, hospitalized for a brain tumor. Results: We have identified 11 cases of brain tumors. The average age was 8.1 ± 4.3 years old, a sex ratio of 0.57. Ten out of the eleven patients of our series have intracranial hypertension. We found six cases of infratentorial tumors and five of supratentorial location. Only three cases had histology. Ten patients were operated, limited by ventriculoperitoneal shunt in 6 cases, surgical resection in three cases, biopsy in one case. There were no possibilities of radiotherapy and chemotherapy during this period of study. Conclusion: A multidisciplinary team must be organized to improve the management of pediatric brain tumors in our context. Histological diagnosis and possibilities of radiotherapy are imperatively needed.