Can we consider ventriculo-gallbladder shunt a first-line treatment in selected patients? Case report of a successful management.

INTRODUCTION: Ventriculo-gallbladder shunt (VGS) has been recognized as a last-resort alternative to treat hydrocephalus when the peritoneum and/or other distal sites can no longer receive shunts. In some specific conditions, it may be conceded as a first-line treatment. CASE PRESENTATION: We report the case of a 6-month-old girl with progressive post-hemorrhagic hydrocephalus who presented a concomitant chronic abdominal symptom. Specific investigations ruled out acute infection and led to the diagnosis of chronic appendicitis. Both problems were managed in a one-stage salvage procedure consisting of laparotomy sanctioning to treat the abdominal pathology and seize the opportunity to perform a VGS as a first option since the abdomen is prone to ventriculoperitoneal shunt (VPS) failure. CONCLUSION: Only few cases have reported the use of VGS as the first option to handle uncommon complex cases due to abdominal or cerebrospinal fluid (CSF) conditions. We wish to draw attention to VGS as an effective procedure not only in children with multiple shunt failures but also as first-line management in some selected cases.

Decision-making algorithm for minimally invasive approaches to anterior skull base meningiomas.

OBJECTIVE Anterior skull base meningiomas are benign lesions that cause neurological symptoms through mass effect on adjacent neurovascular structures. While traditional transcranial approaches have proven to be effective at removing these tumors, minimally invasive approaches that involve using an endoscope offer the possibility of reducing brain and nerve retraction, minimizing incision size, and speeding patient recovery; however, appropriate case selection and results in large series are lacking. METHODS The authors developed an algorithm for selecting a supraorbital keyhole minicraniotomy (SKM) for olfactory groove meningiomas or an expanded endoscopic endonasal approach (EEA) for tuberculum sella (TS) or planum sphenoidale (PS) meningiomas based on the presence or absence of olfaction and the anatomical extent of the tumor. Where neither approach is appropriate, a standard transcranial approach is utilized. The authors describe rates of gross-total resection (GTR), olfactory outcomes, and visual outcomes, as well as complications, for 7 subgroups of patients. Exceptions to the algorithm are also discussed. RESULTS The series of 57 patients harbored 57 anterior skull base meningiomas; the mean tumor volume was 14.7 ± 15.4 cm3 (range 2.2-66.1 cm3), and the mean follow-up duration was 42.2 ± 37.1 months (range 2-144 months). Of 19 patients with olfactory groove meningiomas, 10 had preserved olfaction and underwent SKM, and preservation of olfaction in was seen in 60%. Of 9 patients who presented without olfaction, 8 had cribriform plate invasion and underwent combined SKM and EEA (n = 3), bifrontal craniotomy (n = 3), or EEA (n = 2), and one patient without both olfaction and cribriform plate invasion underwent SKM. GTR was achieved in 94.7%. Of 38 TS/PS meningiomas, 36 of the lesions were treated according to the algorithm. Of these 36 meningiomas, 30 were treated by EEA and 6 by craniotomy. GTR was achieved in 97.2%, with no visual deterioration and one CSF leak that resolved by placement of a lumbar drain. Two patients with tumors that, based on the algorithm, were not amenable to an EEA underwent EEA nonetheless: one had GTR and the other had a residual tumor that was followed and removed via craniotomy 9 years later. CONCLUSIONS Utilizing a simple algorithm aimed at preserving olfaction and vision and based on maximizing use of minimally invasive approaches and selective use of transcranial approaches, the authors found that excellent outcomes can be achieved for anterior skull base meningiomas.

Plagiocephaly due to Frontosphenoidal Suture Synostosis: Report of 2 Cases and Literature Review.

BACKGROUND/AIMS: The most common cause of anterior plagiocephaly is the fusion of the unilateral coronal suture. In some rare cases, however, the fusion of the frontosphenoidal suture may lead to a resembling pattern with specific clinical and radiological features. The aim of the present study is to further enlighten this entity as it is misdiagnosed most of the time. METHODS: A report of 2 cases of plagiocephaly due to frontosphenoidal synostosis with a review of the literature. RESULTS: One female and one male baby were identified. The mean age at presentation was 11.5 months (range: 1-22). Head circumference was normal in each patient. The two fusions were on the left side. Unilateral frontal flattening and recession of the supraorbital rim were the most important physical findings. Computed tomography confirmed the patency of the frontoparietal suture and the closure of the frontosphenoidal suture. Fronto-orbital advancement was performed in both cases with good cosmetic outcome. CONCLUSION: Frontosphenoidal synostosis should be suspected and carefully searched when dealing with plagiocephaly with patent coronal suture. Good outcome requires a prompt diagnosis and early correction.

Primary pure and nonsecreting embryonal carcinoma of the anterior third ventricle: a case report.

Central nervous system germ cell tumors (GCTs) account for less than 5% of primary brain tumors in children and adolescents but continue to attract much attention. To the best of our knowledge, a primary pure and nonsecreting embryonal carcinoma of the anterior third ventricle has never been previously reported. A 15-year-old boy presented with signs of increased intracranial pressure for the past 2 weeks complicated by 2 episodes of generalized tonic-clonic seizures 1 day before admission. Neurological examination was normal, and funduscopic examination disclosed a grade II papilledema bilaterally. CT and MRI revealed a well-demarcated and enhancing mass within the anterior third ventricle associated with a left lateral ventricle hydrocephalus. There was no evidence of tumor within the pineal or suprasellar region, and systemic and cerebrospinal fluid evaluation demonstrated normal levels of α-fetoprotein and human chorionic gonadotropin. Radical surgery was advised, and total tumor resection was achieved via a transcallosal transforaminal approach. The postoperative course was uneventful, and the final histological diagnosis was a pure embryonal carcinoma. Further screening showed no other location, and adjunctive high-dose chemotherapy was administered. The patient has been symptom free with no clinical or radiological sign of progression at the most recent follow-up examination 2 years after surgery. Primary pure and nonsecreting embryonal carcinoma can develop within the anterior third ventricle and should be considered in the differential diagnosis of anterior third ventricular masses especially in young patients. Accurate identification, radical surgery and high-dose chemotherapy can result in better tumor control and improve the postoperative outcome.

Primary mucinous adenocarcinoma of the orbit: A rare clinical entity.

Primary mucinous adenocarcinoma is an exceptionally rare neoplasm with a propensity for local recurrence and metastasis. We report the second case in the world literature of a primary mucinous adenocarcinoma of the orbit in a 66-year-old man suffering from pain, progressive protrusion of left eye, and a deep drop in vision on the left for several weeks. His first external examination revealed significant proptosis with downward displacement of the left globe with no signs of lagophthalmos. A limitation of abduction was also noted. A CT of the orbit with and without contrast showed intra- and extra-conical solid expansive process. MRI of the orbit with contrast and without contrast has shown a process of the supero-internal angle of the left orbit. The patient was operated via a combined approach, and complete enucleation was done. The final pathologic diagnosis was mucinous adenocarcinoma of the orbit. The postoperative neuroimaging showed a complete resection of the tumor. The patient is referred for adjuvant radiotherapy. A CT of the orbit was made 3 months postoperatively and did not show any local recurrence.

Treatment strategies for craniopharyngiomas.

Craniopharyniomas arise from the sellar region and are particularly challenging because of their close proximity to critical neurovascular structures, including cranial nerves, brainstem, internal carotid arteries, posterior cerebral arteries, hypothalamus, and the pituitary gland. The tumors are benign on histology but can cause serious symptoms by compression of surrounding vital structures. While radical surgery and gross total resection (GTR) remains the first line treatment, choosing the optimal surgical approach is critical for the minimization of complications and postoperative morbidity. The extended endoscopic endonasal approach (EEEA) offers a direct route towards the sellar and suprasellar regions and has proven to be a safe and effective for GTR, with lower rates of complications and postoperative morbidity than open transcranial approaches. However, traditional transcranial approaches remain relevant in tumors with significant lateral extension. Radiotherapy can be considered in cases of subtotal resection (STR) or if surgery is not possible, although progressive growth of cysts with neurological deterioration has been demonstrated no infrequently. Newly developed chemotherapy for papillary craniopharygniomas might expand treatment options in the future.

Simultaneous Occurrence of a Pituitary Adenoma and a Foramen Magnum Meningioma: Case Report.

The coexistence of a pituitary adenoma and a meningioma is a rare event. In all previously reported cases, only 1 of these 2 tumors was symptomatic. We present the case of 61-year-old woman with no significant medical history who was treated for a simultaneously symptomatic pituitary nonfunctioning adenoma and foramen magnum meningioma. Such an association has not been reported previously.

Posttraumatic Uncal Herniation in a Conscious Patient.

The incidence of primary traumatic oculomotor nerve palsies in craniocerebral trauma is approximately 1.2% and is usually persistent and associated with loss of consciousness, other neurologic deficits, and skull base or orbital fractures. This case is a rare demonstration of complete left third nerve palsy from uncal herniation after trauma without any loss of consciousness.

Hemangiopericytoma of the Cerebellopontine Angle: A Wolf in Sheep's Clothing.

Primary meningeal hemangiopericytoma (HPC) is a rare, aggressive dura based tumor that remarkably mimics a meningioma clinically and radiologically. Its occurrence within the cerebellopontine angle (CPA) is exceptional, and establishing the exact diagnosis is of the utmost importance since total resection remains the cornerstone of treatment. A 42-year-old man presented with a three-month history of progressively worsening vertigo and difficulty in walking. On admission, his neurological examination revealed a right peripheral facial palsy, right abducens palsy and left hemiparesis, suggesting the diagnosis of Millard-Gubler syndrome. Computed tomography and magnetic resonance imaging demonstrated a homogeneously enhancing dura based lesion of the right CPA causing major brain stem compression. There was no widening of the ipsilateral internal auditory canal. A standard retrosigmoid craniotomy was performed to access the right CPA. Exposure of the lesion revealed a well-encapsulated, gray, fibrous lesion, which appeared to originate from the tentorium. Gross total resection was achieved and confirmed radiologically. The microscopic features and the immunohistochemical profile confirmed the diagnosis of a HPC, and adjuvant radiation therapy was administered. Ten years later, the patient presented with a severe neurological deficit due to a local recurrence, but at that time refused any second intervention. He died three months later. HPC can locate within the CPA and present as a Millard-Gubler syndrome. The diagnosis should be kept in mind in case of a CPA dura based tumor. Radical surgery plus radiation therapy can maximize the recurrence-free survival and close follow-up remains mandatory to spot recurrences early.

Epidural Venous Angioma Presenting with Spinal Cord Compression in a 42-Year-Old Woman with Previous History of Ovarian Malignancy.

BACKGROUND: Venous angioma is an extremely rare vascular malformation of the epidural space. To the best of our knowledge, only 5 cases have been documented to date and none has been reported in the setting of a previous malignancy. CASE DESCRIPTION: We report the case of a 42-year-old woman with a previous history of ovarian cancer, treated by surgery plus chemotherapy; who presented with signs of spinal cord compression for 3 weeks. Magnetic resonance imaging showed an intensely enhancing epidural mass at the T2-T6 level causing major spinal cord compression, for which urgent surgery was indicated. During surgery, the tumor was extremely hemorrhagic and the hemostasis was hazardous. Blood loss was estimated at 1.5 L, causing hemodynamic instability and requiring intensive resuscitation with fluids and blood transfusions. Gross total resection was achieved and the pathologic examination confirmed the diagnosis of venous angioma. The patient recovered quickly postoperatively and was able to walk independently within 2 weeks of starting intensive rehabilitation. She was symptom free with no clinical or radiologic evidence of recurrence at 1 year follow-up. CONCLUSIONS: Venous angioma should be included in the differential diagnosis of spinal epidural masses even in case of previous malignancy. Subtle imaging features should alert clinicians to this rare yet potentially life-threatening condition. Surgery remains the cornerstone of the treatment and can result in remarkable recovery.

Intraventricular Glioblastomas.

BACKGROUND AND IMPORTANCE: Although glioblastoma is the most common primary brain tumor, primary intraventricular locations are extremely rare; only 21 cases have been reported to date. METHODS: A retrospectively acquired database of all intracranial glioblastomas treated in 2 different neurosurgical departments during the last 10 years was queried. Patients with histologically proven intraventricular glioblastomas were included in the study. RESULTS: Eight patients were identified as having a histologically confirmed intraventricular glioblastoma. Patient age at diagnosis ranged from 6 to 74 years (mean 29.6 years) and the male/female ratio was 5:3. Increased intracranial pressure due to hydrocephalus was the main cause of the clinical manifestations. The tumor was located within the lateral ventricle in 6 cases and the anterior third ventricle in 2 others. Gross total tumor excision was achieved in 3 patients, whereas the surgical resection was subtotal in 4 cases and a surgical biopsy was performed in 1 patient. Postoperative adjuvant therapies were administered in 5 patients. Median survival time was 32.1 months, and 3 patients were alive at the end of study. All of them had isocitrate dehydrogenase-mutated tumors. CONCLUSIONS: Intraventricular glioblastoma is extremely rare and can affect younger individuals including children. This malignant tumor should be included in the differential diagnosis of intraventricular lesions, especially in the lateral ventricles. Radical surgical resection can be associated with remarkable disease-free survival, especially in isocitrate dehydrogenase-mutated tumors. Because recurrence virtually is unavoidable, long-term follow-up is mandatory.

Atypical teratoid rhabdoid tumor of the lateral ventricle.

Atypical teratoid/rhabdoid tumors (AT/RTs) are rare and highly malignant embryonal central nervous system neoplasms, usually seen in very young children with rapid fatal outcome despite aggressive treatment. They are most commonly located in the posterior fossa. Intraventricular location is extremely rare. To the best of our knowledge, only 4 cases of lateral ventricle location were reported in the literature. We report the fifth case of lateral ventricle AT/RT in a 2-month-old male who presented with rapid increase of his head circumference. Brain computed tomography scan and magnetic resonance imaging showed heterogeneous huge mass within the left lateral ventricle extending to the parieto-occipital parenchyma and markedly enhancing by contrast. The baby underwent left transparietal approach with complete removal of the tumor. Histological examination confirmed the diagnosis of AT/RT. An aggressive chemotherapy was administrated postoperatively. The outcome is good without neurological deficit or recurrence after 3 years and half of follow-up.

Calcified Suprasellar Xanthogranuloma Presenting with Primary Amenorrhea in a 17-Year-Old Girl: Case Report and Literature Review.

BACKGROUND: Xanthogranuloma, also known as cholesterol granuloma, is an extremely rare intracranial neoplasm most commonly located in the middle ear, petrous apex, or choroid plexus. Exclusively suprasellar xanthogranulomas are exceptional and this report presents a very rare case in the pediatric population, particularly unique due to the presence of calcification. CASE DESCRIPTION: A 17-year-old girl presented with primary amenorrhea with computed tomography and magnetic resonance imaging showing a large calcified enhancing suprasellar mass, which was presumptively diagnosed as a craniopharyngioma on the basis of its clinical and radiologic appearance. Gross total resection of a well-encapsulated, exclusively suprasellar tumor was achieved, without postoperative neurologic deficits. Histologic examination found fibrous tissue with abundant cholesterol clefts, multinucleated giant cells, and hemosiderin deposits but no epithelial cells. The final histologic diagnosis was a xanthogranuloma. CONCLUSIONS: Xanthogranuloma, although extremely rare in the pediatric population, may present as a calcified suprasellar mass and manifest with primary amenorrhea. The prognosis after gross total resection is likely favorable; however, long-term follow-up is indicated for these rare neoplasms.

Metastatic sacrococcygeal yolk sac tumor: A misleading diagnosis.

Sacrococcygeal yolk sac tumor (YST) is an extremely rare malignant extra-gonadal germ-cell tumor, which usually succeeds to the degeneration of more common benign teratoma. We describe here an unprecedented case of conus medullaris compression by a spinal metastasis from a pure sacrococcygeal YST in a 1½ years old girl, which was misdiagnosed initially as an anal fissure and stress the need of a meticulous clinical examination and further screening in young patients presenting with sphincter disturbances.

Giant cell tumor of the sixth thoracic vertebra: case report.

Giant cell tumor is an uncommon but most aggressive benign tumour of the spine with unpredictable outcome and challenging treatment. Spinal giant cell tumors located above the sacrum are rare and treatment recommendations are still unclear. We report a rare case of this lesion in an adult and discuss the management and outcome of such uncommon tumors. A 31-year-old woman presented with progressive motor weakness of both lower limbs with back pain during the past month, associated with sphincter disturbances for the past two days. She was diagnosed with a lytic heterogeneously enhancing mass depending mainly on the T6 posterior arch with small vertebral body involvement. The tumor extent reached surrounding soft tissue and the spinal canal with marked spinal cord compression. A posterior approach was realized as an emergency. Histological examination showed evidence of a giant cell tumor and a complementary irradiation was used. The patient improved well post operatively. There was no recurrence or metastasis over 5 years of follow-up.

Primary Cerebellar Gliosarcoma with Extracranial Metastases: An Orphan Differential Diagnosis.

BACKGROUND: Gliosarcomas are rare, malignant primary brain tumors, most commonly located in the temporal lobe, that contain both glial and mesenchymal elements. Gliosarcomas located within the cerebellum are exceedingly rare. The previously unreported finding of a cerebellar gliosarcoma concurrently with an extracranial metastasis to the lungs is discussed here. CASE DESCRIPTION: A 57-year-old man presented with a 3-month history of chest pain, weight loss, headaches, and vomiting. Physical examination revealed a left cerebellar dysfunction, and the radiological work-up revealed a 6 × 6-cm right apical pulmonary tumor and a 4 × 3.5 × 3.8-cm peripherally enhancing left cerebellar mass. On the basis of a smoking history in the setting of a lung lesion and cerebellar mass, the presumptive diagnosis was primary lung cancer with metastasis to the cerebellum. Gross total resection of a firm pseudo-encapsulated cerebellar mass was performed. The microscopic features and the immunohistochemical profile confirmed the diagnosis of Gliosarcoma. The thoracic lesion was removed subsequently, and pathology confirmed it as an extracranial metastasis from the cerebellar gliosarcoma. Adjuvant radiation and chemotherapy were then administered. No clinical or radiographic evidence of recurrence was observed during one year of follow-up monitoring. CONCLUSIONS: To the best of our knowledge, a primary infratentorial gliosarcoma with extracranial metastases has not been previously described.

Cervico-occipital meningioma in a 5-year-old child: a case report.

AIM: Childhood meningiomas are scarce in clinical practice with an incidence ranging from 0.4 to 4.6% of all pediatric central nervous system (CNS) tumors. Cervico-occipital meningiomas account for 3.7% of childhood meningiomas and are slightly more frequent in male. RESULTS: A 5-year-old female presented with febrile posterior cervico-occipital pain for 3 weeks. She was diagnosed with meningitis and treated for a similar period with adapted antibiotics. The pain persisted even after treatment. Magnetic resonance imaging revealed an enhancing subdural extra medullary mass of the cervico occipital junction, developing around the left vertebral artery. The characteristics of the lesion were strongly suggestive of a neuroma. Surgical removal of the tumor aiming the decompression of the spinal cord and nerve roots was performed with a surprising discovery: The tumor was tightly attached to the dura at the entry of the left vertebral artery. The resection was total and only a thin part close to the artery was left. The pathological findings confirmed the diagnosis of meningothelial meningioma. CONCLUSION: Meningioma should be considered in the differential diagnosis of contrast enhancing subdural extra medullary lesions of the cervico-occipital junction in children.