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.

Trigeminal Ganglioneuroma in the Middle-posterior Cranial Fossa： a Case Report

GANGLIONEUROMA is considered as the most mature and noninvasive form of neuroblastic tumors. It derives from neural crest cells, and can arise from wherever sympathetic tissue exists, including neck, posterior mediastinum, adrenal gland, retroperitoneum and pelvis. The two most common locations for this tumor are retroperitoneum and posterior mediastinum; infrequently it occurs in the intracranial re-gion,2-8 with only three cases has been reported arising from trigeminal nerve.2-4 The current paper presents a 49-year-old male patient with a ganglioneuroma arising from right trigeminal ganglion and extending to the mid-dle-posterior cranial fossa. We summarized the clinical and diagnostic characteristics of this extremely rare tumor, in comparison with the three reported cases in literatures.

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.

Adult Posterior Cranial Fossa Arachnoid Cyst: A Case Report

Adult posterior fossa arachnoid cysts are rare lesions that are considered to be mostly congenital in origin. We present 49-year-old man admitted with a chief complaint of tremor and balance problems for the past 2 months. He had ataxia on examination. Magnetic resonance imaging revealed a well circumscribed midline cystic lesion of the posterior fossa sharing the same signal characteristics as cerebrospinal fluid and non-communicating to the fourth ventricle. He underwent surgical treatment for his symptomatic posterior fossa arachnoid cyst with a good outcome at 2 years follow up.

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.

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

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.

THE RELATIONSHIP BETWEEN THE INVASION OF PARAPHARYNGEAL SPACES AND THE INVOLVEMENT OF CERVICAL NODES AND POSTERIOR GROUP CRANIAL NERVES IN NASOPHARYNGEAL CARCINOMA

Two hundred cates of nasopharyngeal carcinoma (NPC) admitted to this department from Feb. 1985 to May. 1988 were analysed according to the CT scanning and clinical findings of the primary lesions prior to radiotherapy. The results showed that involvement of parapharyngeal space was very common in NPC, about 80% (160/200 cases) ; particularly unilateral or bilateral retro-styloid spaces, about 69.5% (139/200 cases). It was proposed that patients with NPC had a high Incidence of ipsilateral cervical node metastasis. Contralateral cervical node metastasis was rare. The development of cervical node metastasto in NPC has two modes: one Is direct Infiltration of the retro-stylold space by the lesion; the other Is along the nasopharyngeal lymphatic rete. The data also showed that patients with NPC who presented symptoms of Ⅸ- Ⅲ cranial nerve paralyses always had ipsilateral or bilateral retro- styloid space Infiltrations.

Comparative Study on Two Surgical Procedures for Middle Cranial Fossa Arachnoid Cysts

In this study, we explored the operation options for middle cranial fossa arachnoid cysts （MCFAC）. One hundred and forty-nine patients who were operated for a symptomatic MCFAC between 1993 and 2006 in our hosptial were analyzed. Follow-up time ranged from 1 y to 14 y （mean=5.4 y）. All these patients were divided into three subgroups according to Galassi classification. Long-term outcome and complications were studied respectively. Fenestration （F） resulted in a more favorable long-term outcome and less complication for cysts of types I and Ⅱ, whereas a favorable outcome was noted in type Ⅲ patients who underwent cysto-peritoneal shunting （S）. We are led to conclude that Fenestration is suitable for cysts of types Ⅰ and Ⅱ （Galassi classification）, cysto-peritoneal shunting is better for cysts of type Ⅲ.

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.

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.

Calcium pyrophosphate deposition disease of the temporomandibular joint invading the middle cranial fossa:Two case reports

BACKGROUND Pseudogout is a benign joint lesion caused by the deposition of calcium pyrophosphate dihydrate crystals,but it is invasive.Pseudogout of the temporomandibular joint(TMJ)is uncommon,and it rarely invades the skull base or penetrates into the middle cranial fossa.The disease has no characteristic clinical manifestations and is easily misdiagnosed.CASE SUMMARY We present two cases of tophaceous pseudogout of the TMJ invading the middle cranial fossa.A 46-year-old woman with a history of diabetes for more than 10 years was admitted to the hospital due to swelling and pain in the right temporal region.Another patient,a 52-year-old man with a mass in the left TMJ for 6 years,was admitted to the hospital.Maxillofacial imaging showed a calcified mass and severe bone destruction of the skull base in the TMJ area.Both patients underwent excision of the lesion.The lesion was pathologically diagnosed as tophaceous pseudogout.The symptoms in these patients were relieved after surgery.CONCLUSION Tophaceous pseudogout should be considered when there is a calcified mass in the TMJ with or without bone destruction.A pathological examination is the gold standard for diagnosing this disease.Surgical treatment is currently the recommended treatment,and the prognosis is good after surgery.

Endoscopic endonasal transclival removal of tumors of the clivus and anterior region of the posterior cranial fossa (results of surgical treatment of 140 patients)

Background: Until recently, tumors of the clivus and the anterior region of the posterior cranial fossa were considered extremely difficult to access and often inoperable using standard transcranial approaches. With the introduction into the neurosurgical practice of minimally invasive methods utilizing endoscopic techniques, it became possible to effectively remove hard-to-reach tumors, including central tumors of the anterior region of the posterior cranial fossa. Methods: From 2008 to the present time, the inpatient institution has operated on 140 patients with various tumors of the base of the skull, localized to the clivus and anterior region of the posterior cranial fossa (65 men and 75 women). The age of patients ranged from 3 to 74 years. Tumor distribution according to the histopathological features was as follows: chordomas, 103 (73.57%);meningiomas, 12 (8.57%);pituitary adenomas, 9 (6.43%);fibrous dysplasia, 4 (2.86%);cholesteatoma, 3 (2.14%);craniopharyngiomas, 2 (1.43%);plasmacytomas, 2 (1.43%);and other tumors (giant cell tumor, neurohypophyseal glioma, osteoma, carcinoid, chondroma), 5 (3.57%). The tumors had the following size distribution: giant (more than 60 mm), 35 (25%);large (35–59 mm), 83 (59.3%);medium (21–35 mm), 21 (15%);and small (less than 20 mm), 1 (0.7%). In 11 cases, intraoperative monitoring of the cranial nerves was performed (21 cranial nerves were identified). Results: Upper, middle, and lower transclival approaches provide access to the anterior surface of the upper, middle, and lower neurovascular complexes of the posterior cranial fossa. The chordoma cases were distributed as follows according to extent of removal: total removal, 68 (66.02%);subtotal removal, 25 (24.27%);and partial removal, 10 (9.71%). The adenomas of the pituitary gland were removed totally in 6 cases, subtotally in 1 case and partially in 2 cases. The meningiomas were removed totally in 1 case, subtotally in 5 cases, and partially in 5 cases, with less than 50% of the tumor removed in 1 case. Other tumors (cholesteatoma, craniopharyngioma, fibrous dysplasia, giant cell tumor, glioma of the neurohypophysis, osteoma, plasmacytoma, carcinoid, and chondroma) were removed totally in 9 cases and subtotally in 7 cases. Postoperative CSF leaks occurred in 9 cases (6.43%) and meningitis in 13 cases (9.29%). Oculomotor disorders developed in 19 patients (13.57%), 12 of which regressed during the period from 4 to 38 days after surgery, and 7 of which were permanent. In 2 cases, surgical treatment had a lethal outcome (1.43%). (Continued on next page) (Continued from previous page) Conclusion: The endoscopic endonasal transclival approach can be used to obtain access to the centrally located tumors of the posterior cranial fossa. It is an alternative to transcranial approaches in the surgical treatment of tumors of the clivus. The results of using this approach are comparable with the results of transcranial and transfacial approaches and, in some cases, surpass them in effectiveness. The extended endoscopic endonasal posterior (transclival) approach, considering its minimally invasive nature, allows fora radical and low-risk (in terms of postoperative complications and lethality) removal of various skull base tumors of central localization with the involvement and without the involvement of the clivus, which, until recently, were considered to be almost inoperable.

Undiagnosed Anterior Cranial Fossa Dural Arteriovenous Fistula with Intracranial Hematoma: Case Report and Review of the Literature about Its Natural History

Dural arteriovenous fistulas (dAVFs) of an anterior cranial fossa are rare. Because of the high risk of intracranial hemorrhage and relatively easy access for direct surgery, aggressive treatment has been recommended. The natural history of anterior cranial fossa dAVFs (ACF dAVFs) is unclear in spite of many reports for the natural history of general dAVFs. To treat ACF dAVFs, direct surgery has traditionally been performed and endovascular surgery has recently been introduced. A 74-year-old man was transferred with severe consciousness disturbance and presented with devastating intracerebral hemorrhage on the CT scan. Digital subtraction angiography revealed the ACF dAVFs with a large venous pouch. The patient received direct surgery, nevertheless he became vegetative state. Later on, a smaller venous pouch was recognized on the CT scan when he had suffered from the thalamic hemorrhage sixteen months before. There are twelve cases including our case which was treated for a certain period and documented in detail. Eleven of twelve cases were asymptomatic. Three of the six cases with a venous pouch had some events possibly related to the disease, though none of the six cases without a venous pouch had any events during observation. In conclusion, an ACF dAVF with a venous pouch should be treated by direct surgery or endovascular surgery even if it is incidentally found. By contrast, careful observation might be a possible therapeutic option for an ACF dAVF without a venous pouch if there is mild reflux flow.

Discussion of overlaped CT image in cerebral function of fossa cranii posterior

AIM:To discuss the value of technique of overlapped CT image in cerebral function of fossa cranii posterior.METHODS:27 cases of diseases of fossa cranii posterior were examined by three techenics(10 mm scan, 6 mm scan, 3mm scan and overlapped image),and then compare the quality of image, accuracy rate of diagnoses, misdiagnosis rate between the three techniques.RESULTS:The quality of image of ovelaped image was better than the other.The accuracy rate of diagnosis were 77.8%, 85.2%and 96.3%.The false positive rate were 14.8%,11.1%and 3.7%.The false negtive rates were 7.4%,3.7%and 0.CONCLUSION:There was important value for diagnosis of diseases of fossa cranii posterior with technique of overlapped CT image,and can provide help for estimation of the cerebral function.