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.

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.

[Central nervous system dysgerminoma: a clinicopathological study of 3 cases]. / Dysgerminomes du système nerveux central: étude anatomo-clinique de 3 cas.

BACKGROUND: Intracranial germ cell tumors are rarely seen and typically localize in the pineal or suprasellar region. The largest category of germ cell tumors is dysgerminoma. AIM: to describe clinicopathological features and immunohistochemical profile of dysgerminomas. CASE REPORT: We report three cases of central nervous system dysgerminomas. There were two young women and a man who were 6, 11 and 23-year-old. They presented with symptoms of insipidus diabetes (n=3) with association to visual field defects in the third case. Radiological findings showed a supra seller lesion in two cases. Double localization in the pineal and suprasellar regions was seen in the third case. Histologic examination and immunohistochemical study of surgical specimen were consistent with primary central nervous system dysgerminoma.

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.

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.

Transcortical approach to a huge pineal mature teratoma.

Intracranial teratomas are rare germ cell neoplasms occurring more often during childhood. We report the case of a huge mature teratoma of the pineal region in a 10-year-old patient that was not correctly diagnosed preoperatively by stereotactic biopsy. The tumor was revealed by intracranial hypertension and a Parinaud syndrome. The tumor markers were within normal levels in the serum. A left transcortical parietal approach was used to completely resect the tumor. No adjuvant treatment was given. A complete neurological recovery was observed after the surgical procedure. Follow-up at 2 years did not show any recurrence. Pineal mature teratomas have a good prognosis, in contrast to their immature or mixed counterparts. A rigorous histological examination of the tumor samples is mandatory, in order to not omit a mixed contingent within the tumor. The treatment is exclusively surgical.

Descriptive epidemiology of childhood central nervous system tumours in Tunisia. experience of a single institution over a 15-year period (1990-2004).

INTRODUCTION: Central nervous system tumours represent 20% of all childhood cancers, and are the second most common group of neoplasms after leukaemias. OBJECTIVE: To describe epidemiological characteristics of central nervous system tumours in a paediatric Tunisian population. PATIENTS AND METHODS: A retrospective study of 492 childhood central nervous system tumours operated between 1990 and 2004 was undertaken. We investigated the age-related location, gender distribution and the histology of all tumours, and adopted the latest WHO classification (2007) in grouping all the tumours. RESULTS: There were 488 primary and 4 secondary tumours; 426 (86.6%) were intracranial and 66 (13.4%) were intraspinal. Of the 426 intracranial tumours, 214 (50.24%) were supratentorial and 212 (49.76%) were infratentorial. The median age at diagnosis was 8 years, with a male:female ratio of 1.14:1. Low-grade tumours (WHO I/II) constituted 67.3% of all lesions and the rest (32.7%) were high-grade tumours (WHO III/IV). The most common tumour found in our series was astrocytoma (38%), followed by medulloblastoma (16.2%), then ependymoma (6.9%), cystic tumours (6.3%) and craniopharyngioma (5.3%). The overall 5-year survival rate was 45% with a mean follow-up period of 36 months. CONCLUSION: In our patient population, the incidence and distribution of central nervous system tumours were similar to those reported in literature. Overall survival rates varied according to tumour location and histopathology.

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.

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.

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.

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.