Acute Paraplegia Revealing a Hemorrhagic Cauda Equina Paragangliomas.

Cauda equina paragangliomas are rare neuroendocrine benign and slow-growing tumors. Acute paraplegia, occurring because of sudden intratumoral hemorrhage, represents an extremely rare clinical picture of this disease. We report the case of a 64-year-old male presenting with a 5-day acute lower back pain, sciatica, and leg weakness. Spinal imaging showed an intradural mass of the cauda equina region and an emergent surgical treatment was achieved. The lesion was removed "en bloc," and subarachnoid blood was noticed during surgery. The postoperative course was uneventful, with complete regression of pain and progressive motor recovery. The histological study revealed typical microscopic and immunohistochemical features of paragangliomas.

Hodgkin Lymphoma revealed by epidural spinal cord compression.

CONTEXT: Hodgkin Lymphoma is rarely diagnosed as spinal cord compression syndrome. Caused by an epidural mass, this complication is often encountered in a late stage of the disease. We report the case of a 40-year-old man presenting with symptoms of low thoracic spinal cord compression due to an epidural tumor on the MRI. FINDINGS: Emergent surgery was undertaken on this patient, consisting in laminectomy and tumor resection. After surgery, pain relief and mild neurological improvement were noticed. The histological study revealed a Hodgkin Lymphoma and the patient was referred to chemotherapy and radiotherapy. CONCLUSION: Though chemotherapy is the gold standard treatment for Hodgkin Lymphoma, surgical spinal decompression may be required in epidural involvement of the disease. Diagnosis may be suspected in the presence of lymphadenopathy and general health decay.

Symptomatic pneumocephalus: A rare complication of discal herniation's surgery.

Context: We report the case of a 40-year-old woman with no pathological history, operated from an L4-L5 disc herniation by a left unilateral approach. The dura mater enveloping the left L5 root was accidentally injured at its lateral face causing a breach with CSF leakage. This breach could not be sutured. A few hours after waking, the patient presented an agitation followed by three generalized tonico-clonic seizures. Cerebral imaging revealed pneumocephalus. The patient was hospitalized in an intensive care unit. The symptoms gradually faded and the patient was discharged 3 days after surgery.Findings: Pneumocephalus is defined by the presence of air inside the skull. The symptoms of pneumocephalus are generally non-specific and varied, and this complication should also be kept in mind to prevent potentially severe course. The prevention of postoperative pneumocephalus depends on a well-defined strategy in the case of iatrogenic dural tear.Conclusions: Symptomatic pneumocephalus is a very rare complication in the course of lumbar surgery. Conservative therapy may be appropriate even in severe symptomatic manifestations.

Bilateral Orbital Cavernous Hemangioma.

Cavernous hemangioma of the orbit (CHO) is a benign slow-growing lesion of intracanal space. Bilateral orbital cavernous hemangiomas are extremely rare, so that only a few cases have been reported in scientific literature. A 54-year-old patient presented a 1-year history of impaired visual acuity of the left eye. Physical examination showed a left axial propotosis. Orbital magnetic resonance imaging showed a 28 mm diameter intraconal space mass of the left orbit, as well as another 11 mm diameter lesion in the right eye, in the superomedial extraconal space. A superior wall left orbitotomy was performed with a total removal of the tumor. The right lesion was respected. Histopathological examination confirmed the diagnosis of CHO. Bilateral cavernous hemangiomas are extremely rare. Orbital imaging guides the diagnosis. The neurosurgeons prefer craniotomy while ophthalmologists favor various modifications of orbitotomies.

Distinguishing Falcine Chondrosarcomas from Their Mimics and Management.

BACKGROUND: Primary intracranial chondrosarcoma is an extremely rare malignant tumor of the central nervous system, which accounts for <0.16% of all primary intracranial tumors. This rare tumor has a high associated morbidity from the tumor itself as well as from treatment modalities. CASE DESCRIPTION: A 33-year-old man presented with a diffuse headache of 3 months' duration. He was admitted to our department with weakness in the right extremities that had persisted for more than a month. Findings of the neurologic examination revealed right hemiparesis. Cranial magnetic resonance imaging demonstrated a well-demarcated, parasagittal left frontal mass, which compressed to the lateral ventricle. It was hypointense on T1-weighted and hyperintense on T2-weighted images without creating edema in the surrounding tissue. A left frontoparietal craniotomy with complete excision of the mass was performed. The postoperative period was uneventful, and patient was discharged on the fourth postoperative day without any neurologic deficit. Histopathology showed a morphology that was in favor of chondrosarcoma grade 1. CONCLUSIONS: Dural chondrosarcoma is a possible entity in the differential diagnosis of a presumed meningioma, particularly when atypical features are present. We report a grade 1 intracranial chondrosarcoma of the classical subtype without any neurologic problems after complete surgical excision. The patient did not receive any adjuvant therapy and at 26 months' follow-up showed no recurrence.

Embryonal tumor with multilayered rosettes: illustrative case and review of the literature.

BACKGROUND: Embryonal tumor with multilayered rosettes (ETMR) is a very rare entity and has seldom been reported. It has been newly defined tumor entity included in the latest update (revised fourth edition) of WHO 2016 Classification of Tumors of the Central Nervous System which portends a uniform dismal prognosis and survival even with the best of multimodality approaches. ILLUSTRATIVE CASE: This report documents the presentation of a 2-year-old girl with voluminous intracranial ETMR in the right parieto-occipital region. We describe clinical diagnosis, histological aspects, radiological features, and current management of this very aggressive tumor. CONCLUSION: Pediatric intracranial ETMR is a highly aggressive neoplasm, and it should be considered in the differential diagnosis of pediatric brain tumors.

Pediatric Intracranial Anaplastic Gangliogliomas: Illustrative Case and Systematic Review.

OBJECTIVE: We present an illustrative case of pediatric intracranial anaplastic ganglioglioma and systematically reviewed the current reported data of anaplastic ganglioglioma in the pediatric population. METHODS: A comprehensive literature search for our review was conducted using PubMed, Scopus, Web of Science, PsycINFO, Cochrane, and Embase databases. The search terms included "ganglioglioma," "anaplastic," "pediatrics," "children," and "intracranial." We used no limitations for date, type, or language. Reports of pediatric patients (age <19 years) with intracranial anaplastic gangliogliomas were included. Baseline patient demographic characteristics, clinical presentations, imaging characteristics, management strategies, and outcomes data were extracted. RESULTS: We included 24 studies involving 34 patients in the quantitative synthesis. The mean patient age was 9.18 ± 5.46 years (range, 0.16-18). A male predominance was observed (approximate male/female ratio, 1.61:1). Increased intracranial pressure was the most common symptom (n = 19; 55%), followed by seizures (n = 11; 32%). These tumors were most often found in the supratentorial compartment (n = 26; 76%). All 34 patients had undergone surgical removal. The mean follow-up was 22.2 months (range, 2-72). The mean overall survival duration was 43 months (95% confidence interval, 32.45%-55.31%; 1- and 3-year overall survival, 76.6% and 45.5%, respectively). The median event-free survival was 34 months (95% confidence interval, 10.6%-57.3%; 1- and 3-year event-free survival rate, 76.6% and 42.4%, respectively). CONCLUSIONS: Our results contribute to our understanding of the characteristics of this rare malignant tumor and show that anaplastic ganglioglioma should be considered in the differential diagnosis of intracranial tumors in pediatric patients.

A case of adult anaplastic cerebellar ganglioglioma.

BACKGROUND: Anaplastic posterior fossa ganglioglioma in adults is exceedingly rare. To date, only one case of adult anaplastic posterior fossa ganglioglioma has been reported in the English literature and none has been described at the cerebellum. To our knowledge, this report is the third case of malignant posterior fossa ganglioglioma in adults and the first at the cerebellum. In general, this entity can be misdiagnosed preoperatively as a primary posterior fossa neoplasm, and by reporting our clinical and radiographic observations we want to add to the existing literature on this rare entity. CASE DESCRIPTION: A 40-year-old man presented with a history of headaches and dizziness and progressive gait disturbance and was diagnosed with anaplastic ganglioglioma in the posterior fossa. CONCLUSIONS: Although rare, our case demonstrates that anaplastic ganglioglioma should be considered in the differential diagnosis of infratentorial tumors in adult patients.

Spinal epidural angiolipomas: Clinical characteristics, management and outcomes.

PURPOSE: The spinal epidural angiolipomas are rare expansive processes made of mature lipomatous and angiomatous elements. They often have a benign character. Their etiology, pathogenesis remains uncertain, and it is a cause of spinal cord compression. The magnetic resonance imaging is the most important neuroradiological examination. Histological examination is the only examination to confirm the diagnosis. Surgery is the treatment of choice. METHODS: A retrospective study of all patients operated on for a spinal epidural angiolipoma at the Department of Neurosurgery at the National Institute of Neurology of Tunis between January 2000 and December 2014 (15 years) was performed. The aim of this study is to describe the clinical, radiological, histological characteristics and the treatment of this tumor. RESULTS: A total of nine patients were operated from January 01, 2000 to November 30, 2014. The average age of our patients was 51 years with ages that ranged from 29 to 65 with a male predominance. The period between onset of symptoms and diagnosis ranged from 24 months with an average 12 months. Posterior localization of the tumor was seen in all patients. Surgical resection was performed for all cases. The postoperative course has been satisfactory, with a complete recovery of neurological functions in all patients. CONCLUSIONS: The spinal epidural angiolipomas is rare expansive process causing spinal cord compression. Treatment is exclusively surgical resection. The functional outcome of spinal epidural angiolipomas is particularly favorable with a complete neurological recovery is if the patient was quickly operated.

Secondary Chondrosarcoma of the Upper Thoracic Costovertebral Junction with Neural Foraminal Extension and Compressing the Spinal Cord.

BACKGROUND: Chondrosarcoma is a rare malignant tumor of bone. This family of tumors can be primary malignant tumors or a secondary malignant transformation of an underlying benign cartilage tumor. Secondary chondrosarcoma arising from a benign solitary costal osteochondroma is extremely rare. Data show that the reported incidence of costal osteochondroma is very low and they are usually found in the anterior region at the costochondral junction. To our knowledge, however, there have been no previous reports, in English literature, describing osteochondroma malignant transformation located in the thoracic costovertebral junction. CASE DESCRIPTION: We report the case of a man with chondrosarcoma arising from the malignant degeneration of an osteochondroma at the right first thoracic costovertebral junction with neural foraminal extension and compressing the spinal cord. CONCLUSIONS: Although it is rare in solitary osteochondromas of rib, malignant transformation must always be considered.

Intradural Extramedullary Capillary Hemangioma in the Upper Cervical Spine: First Report.

BACKGROUND: The occurrence of intradural extramedullary capillary hemangiomas is exceedingly rare. To date, only 39 cases of intradural extramedullary capillary hemangiomas have been reported in the English literature, and all of these cases have been described at the lumbar and thoracic spinal levels. To our knowledge, this report is the first case of capillary hemangiomas of the cervical spine in the literature. In general, this entity is misdiagnosed preoperatively as a neoplasm. CASE DESCRIPTION: A 29-year-old man presented with neck pain and progressive gait disturbance, and was diagnosed with an intradural extramedullary capillary hemangioma in the cervical region. CONCLUSIONS: Although rare, our case demonstrates that capillary hemangioma should be considered in the differential diagnosis of intradural extramedullary tumor of the cervical spine.

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.

Primary sporadic Burkitt lymphoma of the orbit, clinical characteristics, management, and outcomes: a case study.

BACKGROUND: Involvement of the orbit with Burkitt's lymphoma is a very rare presentation of extra-nodal lymphoma. ILLUSTRATIVE CASE: We report a case of a 2-year-old female presented an unusual location of sporadic Burkitt's lymphoma arising in the orbital region. Diagnostic magnetic resonance imagining identified an oval-shaped mass on the lateral rectus of the right orbit that caused dislocation of eyeball, for which she underwent a biopsy from the periorbital swellings. The mass was histologically confirmed as Burkitt's lymphoma, and postoperative aggressive chemotherapy was initiated. We describe clinical diagnosis, histological aspects, radiological features, and current management of this rapidly growing malignant tumor. CONCLUSION: Because of the rapid progression of Burkitt lymphoma, and considering that it responds well to treatment, early recognition and appropriate management are important factors for survival and to preserve visual function.