Organic bipolar disorder secondary to central neurocytoma resection: a case report.

entral neurocytoma is a neuroepithelial tumor described by Hassoun in 1982, predominantly located in the midline at the level of the septum pellucidum, or in the lateral ventricles wall1. They represent approximately 50% of intraventricular lesions in adults, and they are in total 0.25-0.5% of intracranial tumors.

Amenorrhoea and reversible infertility due to obstructive hydrocephalus: literature review and case report.

BACKGROUND: Endocrine abnormalities are well-recognized consequences of intracranial pathology such as pituitary tumours. Less commonly, hydrocephalus may lead to dysfunction of the endocrine system, presenting as amenorrhoea or precocious puberty. We present a case report and literature review of hydrocephalus causing endocrine abnormalities including reversible infertility. CASE DESCRIPTION: A 34 year-old female presented with amenorrhoea and infertility. MRI showed a third ventricular mass and hydrocephalus. The amenorrhoea resolved within weeks of endoscopic third ventriculostomy and tumour biopsy; pregnancy ensued within 6 months. Thirty-two cases of hydrocephalus-related amenorrhoea were reported between 1915 and 2007. All patients who underwent modern hydrocephalus treatment experienced partial or complete resolution of endocrine dysfunction. Successful pregnancy was reported in three patients, as in our case presentation. While mechanisms of dysfunction have not been completely elucidated, studies point toward loss of GnRH pulsatility due to compression of the medio-basal hypothalamic structures. CONCLUSION: Hydrocephalus can cause endocrine dysfunction, including amenorrhoea, which may reverse with CSF diversion. Therefore, cranial imaging is an important component in the evaluation of such endocrine abnormalities.

Ventricular Central Neurocytoma: Rate of Shunting and Outcome 2 Years After Total and Subtotal Excision.

BACKGROUND: Central neurocytoma is an intraventricular tumor that affects young adults. It has a favorable prognosis after adequate surgical intervention; however, an aggressive course may take place in some cases. OBJECTIVE: The objective of the study was to evaluate the rate of shunting and the outcome of control measures in patients with central neurocytoma submitted to total and subtotal excision. METHODS: Twelve patients were included in this study, with a follow-up of 24 months. Data collected included: age, sex, clinical presentation, early morbidity and mortality, radiological findings (tumor location, features, residual, recurrence, and hydrocephalus). All patients underwent surgery for total or subtotal excision through a transcortical approach. External Ventricular Drain (EVD) was inserted then removed or replaced by a shunt. Histopathology and the MIB index were used to confirm diagnosis and guide the follow-up; adjuvant radiotherapy or Gamma Knife radiosurgery were used for residual tumor or recurrence. RESULTS: The ages of the patients ranged from 14 to 48 years. Two patients died early, after total and subtotal excision, from sepsis and thalamic infarction, respectively. Six patients (60 %) had a total excision; two of them had a high MIB index and showed small recurrence at 12 months and 18 months, respectively, and received Gamma Knife radiosurgery. One of the six patients with total excision needed a shunt, and no shunt was needed in the four otherpatients; a subtotal excision was done for four patients (40 %). An early shunt was inserted for two of these patients, radiosurgery-controlled for one patient, while radiotherapy was used for control in the other three patients; radiotherapy control failed in one patient, who underwent a second surgery at 18 months. CONCLUSION: Central neurocytoma may have a favorable prognosis, with a lower incidence of shunt insertion throughout its course than that for other intraventricular tumors, if total removal is achieved.

Behavioral disorders as unusual presentation of pediatric extraventricular neurocytoma: report on two cases and review of the literature.

BACKGROUND: Extraventricular neurocytomas (EVNs) are rare parenchymal brain tumors, distinct from central neurocytomas that are typically located within the supratentorial ventricular system. Seizures and headache represent the most common symptoms of extraventricular neurocytomas in the cerebral hemisphere both in adult and pediatric population. CASE PRESENTATION: We describe two cases of pediatric EVN with clinical onset characterized by behavioral and attention deficit/ hyperactivity disorders. The association between behavioral/attention disorders in childhood and the presence of a frontal neurocytoma has never been described before. Furthermore, inappropriate levels of inattention, hyperactivity and impulsivity are common among the neurobehavioral and developmental disorders in childhood. We reviewed 43 pediatric cases of extraventricular neurocytoma included in the PubMed database and their clinical presentation, and we never found this unusual relationship. CONCLUSION: In childhood, the attention/hyperactivity disorders seem to be often over-diagnosed. When these deficits are more subtle and do not well-fit in a specific neurocognitive disorder, the clinicians should have a suspicion that they might mask the clinical features of a frontal lesion. This paper is focused on the clinical presentation of the extraventricular neurocytoma and the possible organic etiology of an attention and hyperactivity deficit.

Mesial temporal extraventricular neurocytoma: a rare cause of refractory complex partial seizure.

AIM: Mesial temporal extraventricular neurocytoma (mtEVN) is a rare cause of refractory complex focal seizures. The characteristics of this clinical entity are discussed in this article. METHODS: We report two cases of mtEVN and review the related literature, with particular emphasis on radiological characteristics, clinical features, and operative techniques. RESULTS: After successful surgery, our two cases of mtEVN achieved excellent outcome. Including the cases presented here, a total of three cases of mtEVNs and 11 of neocortical temporal extraventricular neurocytoma (ntEVNs) are reported in the literature. mtEVNs are distinct from ntEVNs with regards to demographics, aetiology, radiological features, and operative techniques. CONCLUSION: mtEVNs and ntEVNs exhibit distinguishing features. Under electrocorticographic monitoring, tailored resection of the neocortical epileptogenic focus, as well as the entire tumour and mesial temporal structures, can yield excellent outcome and satisfactory seizure control.

Two novel tumours with NTRK2 fusion in the methylation class of extraventricular neurocytomas, including one intraventricular.

We report here about two novel tumours classified as extraventricular neurocytomas (EVN) using DNA-methylation profiling, associated with NTRK2 fusions instead of the usual FGFR1 alterations so far attributed to this tumoural entity. We present the second detailed case of an intraventricular presentation in the MC EVN. Our findings broaden the spectrum of MC EVN and have implications in terms of diagnosis, therapy and terminology.

Radiosurgery for central neurocytoma: long-term outcome and failure pattern.

Despite the favorable outcomes of radiosurgery for central neurocytoma (CN), these results are based on case series that included a limited number of patients and short follow-up periods because of the scarcity of CN. Because CN is a benign tumor with an indolent clinical course, long-term follow-up and analysis of failure pattern are required for the establishment of the role of radiosurgery in the management of CN. Twenty consecutive patients (10 patients who received Gamma Knife radiosurgery (GKRS) as a primary treatment and 10 patients who received GKRS as a secondary treatment) with a radiological follow-up period ≥36 months were included in this study. The mean radiological follow-up duration was 100 months (range 43-149 months). The mean tumor volume was 10.4 cm(3) (range 0.4-36.4 cm(3)) and the mean marginal dose was 15.4 Gy (range 9-20 Gy). Local control failure was found in six patients at the last radiological follow-up. Overall actuarial local control rates were 89.5 % at 5 years and 83.1 % at 10 years. The primary GKRS group included two cases with local failure, with cyst formation or local recurrence. In contrast, in the secondary GKRS group, local control failure was found in four cases (including three cases with an "out-of-field recurrence" pattern) and occurred earlier compared with the primary GKRS group. Our study suggests that GKRS could be a primary or secondary treatment option for CN. However, long-term radiological follow-up is mandatory. In particular, more careful consideration during margin delineation and planning procedure is required in the secondary GKRS group.

Computed tomography and magnetic resonance features of extraventricular neurocytoma: a study of eight cases.

AIM: To present the neuroradiological and clinical findings of extraventricular (central) neurocytomas (EVNs) to increase awareness of this entity. MATERIALS AND METHODS: The computed tomography (CT; n = 6), magnetic resonance imaging (MRI; n = 8), and clinical presentations of eight patients with pathologically documented EVN were retrospectively analysed. RESULTS: Most tumours were well circumscribed and occurred in young adults. Six tumours were solid or solid-cystic, five of these showed contrast enhancement and three contained calcifications. Multiple small cysts were present in one solid mass and had a "soap bubble" or spongy appearance on MRI. Two other tumours were predominantly cystic; these demonstrated slight contrast enhancement, which contained calcifications. Of the six cases assessed using CT, three showed predominantly hyperdensity and three showed hypodensity, with a mean attenuation value of 75 HU. At MRI, eight masses were isointense (n = 4) or hypointense (n = 4) to grey matter on T1-weighted images and hyperintense (n = 6), isointense (n = 1), or hypointense (n = 1) on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images. Signal voids were visible in two cases. Four tumours had mild or moderate peritumoural oedema. CONCLUSION: EVN is a rare neoplasm that can have significant overlap in imaging appearance with other primary brain neoplasms; therefore, it is difficult to make an accurate preoperative diagnosis. However, EVN should be considered in the differential diagnosis when a large cerebral parenchymal mass with cystic change and calcification is encountered in younger patients.

Third ventricle, cerebral aqueduct, and fourth ventricle mixed ependymal neurocytoma: a case report and review of the literature.

PURPOSE: A case report of an 8-year-old female who underwent interhemispheric transcallosal approach to the third ventricle, cerebral aqueduct, and fourth ventricle for resection of a mixed ependymal neurocytoma and review of the literature was evaluated. METHODS: An Ovid MEDLINE review of the literature was conducted starting in 1946 to current using search terms as described in our keywords. RESULTS: A total of 16 neurocytoma have been described in the literature as either posterior third ventricle or posterior fossa in origin. Of these lesions, five have been described as mixed glial-neuronal and all of these were located in the fourth ventricle. To our knowledge, this is the first described mixed glial-neuronal tumor located in the posterior third ventricle and aqueduct.

Central Neurocytoma with Extensive Intratumoral Hemorrhage: A Case Report.

BACKGROUND Central neurocytoma (CN) is a rare neuronal tumor of neuroepithelial origin. It has been assigned to World Health Organization classification grade 2. These tumors are usually benign and located in the anterior half of the lateral ventricle, though they can also be found in the third and fourth ventricles. Left untreated, a CN can cause blockage of cerebrospinal fluid, thus leading to hydrocephalus. CNs are exceedingly uncommon, making up just 0.1-0.5% of primary intracranial tumors. The tumors typically develop in people aged 20 to 40. There are no official guidelines on how to treat CN, so treatment options are often individualized on the basis of specific case findings. CASE REPORT A 39-year-old man with an uncomplicated medical history presented with dizziness, increasingly worse headaches, presyncope, and a loss of appetite. Radiological data and postoperative histopathological and histochemical analysis led to the diagnosis of CN with extensive intratumoral hemorrhage. Surgical resection of the tumor was proposed to the patient, to which he agreed. CONCLUSIONS CN is a benign tumor, but it can cause serious or life-threatening complications. Gross total resection of the tumor is recommended if possible, and if deemed beneficial to the patient's clinical condition. This case reports the symptoms of a patient with CN, who underwent gross total resection and showed no sign of any residual tumor tissue on postoperative MRI. By reporting these types of cases, we can take necessary steps ahead of widespread agreement on optimal treatment of patients with neurocytomas.

Secondary normal pressure hydrocephalus in a patient with isolated frontal dilatation--an insight into pathophysiology?

Current theories of the pathophysiology of normal pressure hydrocephalus suggest the classical symptoms are a consequence of disruption of normal frontal function. We present the case of a 70-year-old patient with an isolated, frontal dilatation of his lateral ventricles in the presence of a complete triad as supportive of these theories.

Unusual exophytic neurocytoma of thoracic spine mimicking meningioma: a case report and review of the literature.

Extraventricular neurocytoma (EVN) in the spinal cord is extremely rare and only 15 cases have been reported in the English literature. Fourteen cases presented as an intramedullary lesion with spinal cord enlargement in cervico-thoracic segment and one case presented as an extramedullary lesion originating from cauda equina. Herein, we report an unusual spinal neurocytoma with exophytic growth from the thoracic spinal cord with cord compression mimicking meningioma. To our knowledge, this is the first case of exophytic spinal neurocytoma recorded in the English literature.

Olfactory neurocytoma as a unique cause of chronic SIADH.

A 32-year-old man was found to have a nasal mass on DOTATATE positron emission tomography (PET) scan to investigate the cause of his syndrome of inappropriate antidiuretic hormone secretion (SIADH). The patient presented 6 years earlier with malignant hypertension followed by a second emergency admission for hyponatraemia. Multiple scans and blood tests over 6 years yielded no cause for his SIADH. Nasendoscopy was unremarkable. A PET scan prompted endoscopic sinus surgery which resulted in the resection of a mass in the anterior hiatus semilunaris. The histological findings were fitting with a diagnosis of a neurocytic-type tumour favouring an olfactory neurocytoma. Following resection, the patient remains well and is cured of his SIADH. An olfactory neurocytoma although rare should be considered as a benign differential for a mass in the nasal space. This case demonstrates how an olfactory neurocytoma can present as a cause of SIADH.

Treatment of atypical central neurocytoma in a child with high dose chemotherapy and autologous stem cell rescue.

The authors describe a 9 month old female with recurrent atypical central neurocytoma and leptomeningeal spread treated with high dose chemotherapy, autologous stem cell rescue, and adjuvant therapy. She had a complete response to therapy and was disease free at 4 years of age until a recurrence 6 months later. The use of intensive chemotherapy followed by autologous stem cell rescue for atypical neurocytoma may be considered as an adjunct to surgical therapy in young patients with atypical neurocytoma not amenable to radiation therapy.

Leptomeningeal dissemination of a pediatric neoplasm with 1p19q deletion showing mixed immunohistochemical features of an oligodendroglioma and neurocytoma.

Leptomeningeal dissemination of an oligodendroglioma is rarely reported in the neurosurgical literature, especially in cases with a classical 1p19q deletion. The authors describe a case wherein a 1p19q deletion in a disseminated tumor with mixed immunohistochemical features of oligodendroglioma and neurocytoma was encountered and treated. Stereotactic right frontal craniotomy was undertaken for obtaining definitive histological diagnosis. The results revealed a neuroectodermal neoplasm with histologic and immunohistochemical features of oligodendroglioma and neurocytoma. FISH analysis confirmed classical 1p19q deletion. The patient was treated postoperatively with chemotherapy and radiation therapy. He showed good clinical response and remains alive 16 months after diagnosis.

Death in custody and undiagnosed central neurocytoma.

Fatal brain tumors are often diagnosed well before death. Rarely, they present as sudden and unexpected death. Most of these undiagnosed brain tumors are gliomas. Death in custody is uncommon and can be caused by natural diseases. These are mainly associated with the cardiovascular system and rarely by cancers such as primary intracranial tumors.We present a case of a 55-year-old man who died while in police custody. At the autopsy there was a previously undiagnosed large soft intraventricular tumor. Cerebrospinal fluid flow was obstructed causing a raised intracranial pressure which resulted in displacement of the pons, and death. Microscopy and immunohistochemistry confirmed a central neurocytoma.

Cerebellar liponeurocytoma: an updated follow-up of a case presenting histopathological and clinically aggressive features.

Cerebellar liponeurocytoma is a rare tumor recently included by World Health Organization in the classification of brain tumors as a separate clinicopathological entity separate from medulloblastoma. However, because of the rarity of the tumor, the natural history of the tumor is still not yet been defined. We report a patient with cerebellar liponeurocytoma with unusual clinical and pathological aggressive features. This patient suggests the possible existence of different histological grades of liponeurocytoma and its possible progression to a malignant grade.