Elisabetta Marton

Malignant progression in pleomorphic xanthoastrocytoma: Personal experience and review of the literature

Pleomorphic xanthoastrocytoma (PXA) is a rare primary low-grade astrocytic tumor, recently classified as a neuroglial tumor. It generally occurs in children and young adults and shows benign behaviour (WHO II), although an anaplastic variant and malignant potential have been described. Pleomorphic xanthoastrocytomas with malignant transformation have been reported in three out of eight patients operated on for this type of tumor in our department in the last 15 years. The three patients were two adult women and a child, the primary tumors were located in the cortex of the right temporal lobe, and treatment consisted of complete surgical resection. Histological examination revealed simple PXA in two patients and a PXA with anaplastic foci in the other. Mean recurrence time was 5.7 years, with the original xanthoastrocytoma evolving to glioblastoma in two cases and anaplastic astrocytoma in the third. All three patients underwent a second operation, followed by adjuvant therapies. Two died from tumor progression and one from brain edema after intracerebral haemorrhage. A review of the available PXA literature dating back to 1979 revealed 16 cases of primary anaplastic astrocytoma and 21 cases of PXA with malignant transformation. Our experience adds three more cases of malignant transformations, outlining once again the potential malignancy of pleomorphic xanthoastrocytomas and the fact that prognosis in these cases is the same as for primary anaplastic astrocytoma and glioblastoma. Analysis of glioneuronal markers, Ki67 and p53 in all pleomorphic xanthoastrocytomas did not prove to be a discriminating factor to identify a subgroup of xanthoastrocytomas prone to malignancy. Accordingly, these tumors demand close long-term clinical and radiological follow-up.

Symptomatic Rathke's Cleft Cysts: A Radiological, Surgical and Pathological Review

Rathkes cleft cyst is a non neoplastic lesion remnant of the Rathkes poutch. Symptomatic cysts must be surgically treated. There are no specific neuroradiological features for this lesion. We present a series of 14 symptomatic Rathkes cleft cysts treated with transphenoidal endoscopic technique, and we correlate the MRI intensity images with the intraoperative findings, the pathological analysis, the growth pattern and the recurrence tendency, to understand wether there is a better way to recognize these lesions, especially the asyntomatic ones, that, despite other neoplastic cysts, colud only be observed with a MRI follow up.

Pituitary adenomas in childhood and adolescence. Clinical analysis of 10 cases

Pituitary adenomas in childhood and adolescence constitute 2–6% of all operated pituitary adenomas. We report the clinical features, treatment and follow-up of 10 pediatric patients affected by pituitary adenomas. All patients underwent clinical evaluation, endocrine tests, magnetic resonance imaging and visual field assessment. Follow-up ranged from 8 to 132 months (median 52.6). All patients were older than 10 years of age; 60% were males. In 50% the initial complaints were headache and/or visual impairment, all except one had clear evidence of endocrine dysfunction. Ninety percent were macroadenomas. According to hormone measurements and immunostaining 50% were prolactinomas, 20% were pure GH-se-creting and 30% were non-functioning adenomas. Prolactinomas in two females were successfully treated with cabergoline. The other patients un-derwent surgery: three prolactinomas are still being treated with dopamine agonists and a GH-secreting adenoma is being treated with octreotide LAR and cabergoline. Two patients were also treated with conventional radiotherapy. Treatments were completely successful in 50% of patients: these have normal hormone secretion, full pubertal development, no significant tumor mass and normal visual field. Hypersecretion of prolactin persists in two cases; partial or complete hypopituitarism is present in four, relevant tumor remnant in another four and impairment of visual field is present in two cases. In conclusion, pediatric adenomas occur mostly in pubertal age, are prevalently macroadenomas and clinically functioning. Medical therapy should be preferred for secreting adenomas, but in some cases, notably prolactinomas in males, surgery and eventual radiotherapy may be needed.

Dumbbell-shaped C-2 psammomatous melanotic malignant schwannoma. Case report and review of the literature.

The authors present the case of a dumbbell-shaped malignant psammomatous melanotic schwannoma of the upper cervical spine involving the C-2 sensory root. The family of the patient had a history of other malignant stromal tumors, without the Carney complex genetic pattern. The 30-year-old female patient complained of experiencing cervical pain and cervical muscle contractions for 6 months, and was admitted to the hospital. The cervical T1-weighted magnetic resonance (MR) images revealed the presence of a slightly hyperintense C2-3 intra-extradural lesion, moderately enhancing, which had eroded and enlarged the intervertebral foramen. The patient workup also included computed tomography scans and angiography. A posterior approach was used to perform a C2-3 hemilaminectomy, including opening of the dura mater and gross-total removal of the lesion. Histopathological examination of the lesion revealed it to be a malignant psammomatous melanotic schwannoma. The cerebrospinal MR image of the patient obtained at the 12-month follow-up examination demonstrated the presence of tumor progression into the subarachnoid space at the C-3 level. The strong malignancy potential of the lesion must be considered in the future management of the patient, especially due to the presence in the family of other stromal tumors such as gastrointestinal-stromal tumors and malignant melanomas. The authors review all the literature concerning melanotic schwannomas and report 105 cases of melanotic schwannoma that were not related to the Carney complex. The particular focus of their review is on the characteristics of the malignant progression of melanotic schwannoma, such as local recurrences, metastasis, and survival rate.

Dynamic monitors of brain function: a new target in neurointensive care unit.

IntroductionSomatosensory evoked potential (SEP) recordings and continuous electroencephalography (EEG) are important tools with which to predict Glasgow Outcome Scale (GOS) scores. Their combined use may potentially allow for early detection of neurological impairment and more effective treatment of clinical deterioration.MethodsWe followed up 68 selected comatose patients between 2007 and 2009 who had been admitted to the Neurosurgical Intensive Care Unit of Treviso Hospital after being diagnosed with subarachnoid haemorrhage (51 cases) or intracerebral haemorrhage (17 cases). Quantitative brain function monitoring was carried out using a remote EEG-SEP recording system connected to a small amplification head box with 28 channels and a multimodal stimulator (NEMO; EBNeuro, Italy NeMus 2; EBNeuro S.p.A., Via P. Fanfani 97/A - 50127 Firenze, Italy). For statistical analysis, we fit a binary logistic regression model to estimate the effect of brain function monitoring on the probability of GOS scores equal to 1. We also designed a proportional odds model for GOS scores, depending on amplitude and changes in both SEPs and EEG as well as on the joint effect of other related variables. Both families of models, logistic regression analysis and proportional odds ratios, were fit by using a maximum likelihood test and the partial effect of each variable was assessed by using a likelihood ratio test.ResultsUsing the logistic regression model, we observed that progressive deterioration on the basis of EEG was associated with an increased risk of dying by almost 24% compared to patients whose condition did not worsen according to EEG. SEP decreases were also significant; for patients with worsening SEPs, the odds of dying increased to approximately 32%. In the proportional odds model, only modifications of Modified Glasgow Coma Scale scores and SEPs during hospitalisation statistically significantly predicted GOS scores. Patients whose SEPs worsened during the last time interval had an approximately 17 times greater probability of a poor GOS score compared to the other patients.ConclusionsThe combined use of SEPs and continuous EEG monitoring is a unique example of dynamic brain monitoring. The temporal variation of these two parameters evaluated by continuous monitoring can establish whether the treatments used for patients receiving neurocritical care are properly tailored to the neurological changes induced by the lesions responsible for secondary damage.

Parasagittal Cranial Fasciitis after Irradiation of a Cerebellar Medulloblastoma: Case Report

OBJECTIVE AND IMPORTANCE:Cranial fasciitis is a very rare, nontumoral lesion of the cranium with potential intracranial expansion typical of childhood. Radiotherapy has not been reported among the possible causes or factors associated with this condition. We present a case of cranial fasciitis in an 11-year-old patient previously admitted for cranial radiotherapy of a cerebellar medulloblastoma. Cytogenetic analysis revealed a pattern of chromosomal abnormalities suggestive of a radiation-induced lesion. CLINICAL PRESENTATION:An 11-year-old patient, who had been treated previously with craniospinal radiotherapy for a medulloblastoma, presented with a tumor resembling a parasagittal meningioma. INTERVENTION:At surgery, the tumor apparently had eroded the cranium and was deemed to originate from the external layer of the sagittal sinus. A distinct line of cleavage permitted total removal. Histological analysis was suggestive of cranial fasciitis. Cytogenetic analysis revealed the presence of a polyclonal karyotype in a background of nonclonal changes. CONCLUSION:Cranial fasciitis should be included in the differential diagnosis of intracranial tumors infiltrating the cranium. Treatment of these lesions is easier than that of other parasagittal lesions because the sinus is compressed but not infiltrated. This case is associated with previous radiotherapy; thus, cranial fasciitis could be considered one of the more common radiation-induced lesions.

Pituitary metastasis of Merkel cell carcinoma

Merkel cell carcinoma (MCC) is a malignant neuroendocrine tumor of the skin that demonstrates a remarkable tendency to metastasize. However, only a few cases of MCC brain metastases have been reported in the literature. We here present a unique case of a pituitary metastasis of MCC in a 65-year-old patient with a history of pituitary adenoma. This case is particularly novel due to the fact that the primary site of the MCC is unknown.

Neuroendoscopic aspiration of tumors in the posterior third ventricle and aqueduct lumen: a technical update.

BackgroundSmall soft ventricular tumors are good candidates for complete removal by a purely endoscopic technique. This approach is particularly interesting for lesions located in the posterior third ventricle and aqueductal lumen.MethodsWe present our method of endoscospic aspiration through direct contact between the tumor and the working channel of a flexible scope.ResultsAspiration without the intermediate use of cannulas is safe and has proved to be effective in the apparently total or partial removal of three soft tumors of different pathologies located in the third ventricle or aqueductal lumen. In one case, a second neuroendoscopy a few months later to repeat endoscopic third ventriculostomy (ETV) offered a unique opportunity to observe the absence of the tumor and the restored anatomy.ConclusionsNeuroendoscopy provides a safe, effective way to radically resect small soft tumors in these troublesome locations and can be a valuable alternative to microsurgery.

Atypical cystic meningioma overexpressing AQP1 in early infancy: case report with literature review

Meningiomas in early infancy are rare lesions, worth to be reported for their exceptional occurrence. The authors report a case of an 11‐month‐old female child with asymmetric macrocephaly due to the presence of a cystic atypical meningioma associated to bilateral subdural collections.

A preliminary study of aquaporin 1 immunolocalization in chronic subdural hematoma membranes.

Aquaporin 1 (AQP1) is a molecular water channel expressed in many anatomical locations, particularly in epithelial barriers specialized in water transport. The aim of this study was to investigate AQP1 expression in chronic subdural hematoma (CSDH) membranes. In this preliminary study, 11 patients with CSDH underwent burr hole craniectomy and drainage. Membrane specimens were stained with a monoclonal antibody targeting AQP1 for immunohistochemical analysis. The endothelial cells of the sinusoid capillaries of the outer membranes exhibited an elevated immunoreactivity to AQP1 antibody compared to the staining intensity of specimens from the inner membrane and normal dura. These findings suggest that the outer membrane might be the source of the increased fluid accumulation responsible for chronic hematoma enlargement.