Cervical dermal sinus complicated with intramedullary abscess in a child: case report and review of literature.

BACKGROUND: Dermal sinus tract (DST) is an uncommon spinal dysraphism presenting in childhood along the midline neuroaxis. Cervical DST less frequent is in association with intramedullary abscess. METHODS: A 9-month-old baby was admitted to our unit suffering from right hemiparesis. Physical examination showed a cervical midline cutaneous fistula dripping a yellowish thick liquid. Cervical MRI showed at C5 level a sinus tract in continuity with a C3-C6 intramedullary lesion. Total removal of the fistula and the intramedullary lesion was performed. Histopathological examination confirmed the diagnosis of dermal sinus and abscess. RESULTS: Post-operative cervical MRI showed a complete removal of the spinal dysraphism, fistula, and intramedullary abscess. The baby showed a progressive clinical improvement and was discharged on day 10 post-surgery. CONCLUSION: Literature review confirms that an early diagnosis followed by prompt surgical spinal cord decompression gives a functional neurological recovery.

Central nervous system metastases from epithelial ovarian cancer: prognostic factors and outcomes.

OBJECTIVES: To analyze the clinicopathological characteristics and prognostic factors associated with survival in patients with central nervous system (CNS) metastases from epithelial ovarian cancer. METHODS: Twenty patients with CNS involvement from ovarian carcinoma were evaluated in this retrospective study; their features and survivals were analyzed using Kaplan-Meier and log-rank test methods. RESULTS: The incidence of CNS metastases was 5%, among 400 patients with ovarian cancer treated in our single institution. The median age at diagnosis of the ovarian cancer was 55 years. The median interval to the brain involvement and the median survival were 33 and 18 months, respectively. Prognostic factors associated with survival were the International Federation of Gynecology and Obstetrics stage, the surgical resection, the multimodal treatment, and the response after the therapy of the brain metastases. CONCLUSIONS: Brain involvement from ovarian cancer is uncommon but is increasing in incidence. Although the prognosis is usually poor, a multimodal approach can result in a long-term remission of the metastases and in an improvement of the overall survival.

Molecular genetic analysis in a case of ganglioglioma: identification of a new mutation.

OBJECTIVE: Ganglioglioma is a primary central nervous system low-grade tumor composed of mixed populations of glial and neuroepithelial elements. METHODS: The authors report a case of ganglioglioma in a patient affected by Peutz-Jeghers syndrome, an autosomal dominant disease with varying expressions and incomplete penetrance responsible for an increased risk of gastrointestinal and other malignant tumor forms. RESULTS: The polymerase chain reaction products of exon 6 of STK11/LKB1 showed an abnormal pattern in the single-strand conformation polymorphism analysis. Further sequencing analysis of the exon 6 identified a deletion of T and an insertion of AC at nucleotide 821 causing a shift of the reading frame. The same mutation was found in the patient's peripheral blood. The ribonucleic acid analysis on the ganglioglioma cells revealed an out-of-frame STK11 isoform, characterized by an exon 4 skipping, which resulted in nonsense mediated decay sensitive. CONCLUSION: This report details the molecular genetic analysis of a ganglioglioma that allowed the identification of a new mutation.

A polycystic variant of a primary intracranial leptomeningeal astrocytoma: case report and literature review.

BACKGROUND: Primary leptomeningeal astrocytomas are rare intracranial tumors. These tumors are believed to originate from cellular nests which migrate by means of aberration, ultimately settling in the leptomeningeal structure. They may occur in both solitary and diffuse forms. The literature reports only fifteen cases of solitary primary intracranial leptomeningeal astrocytomas. CASE PRESENTATION: The authors report the case of a seventy-eight year-old woman with a polycystic variant of a solitary primary intracranial leptomeningeal astrocytoma. The first neurological signs were seizures and aphasia. CT and MRI scans demonstrated a fronto-parietal polycystic tumor adherent to the sub arachnoid space. A left fronto-temporo-parietal craniotomy revealed a tight coalescence between the tumor and the arachnoid layer which appeared to wrap the mass entirely. Removal of the deeper solid part of the tumor resulted difficult due to the presence of both a high vascularity and a tight adherence between the tumor and the ventricular wall. CONCLUSION: A new case of a solitary primitive intracranial leptomeningeal astrocytoma of a rare polycystic variant is reported. Clinical, surgical, pathologic and therapeutic aspects of this tumor are discussed.

Emergency surgery in a severe penetrating skull base injury by a screwdriver: case report and literature review.

BACKGROUND: Very few cases of severe penetrating injuries to the skull base with a seemingly innocuous object have been described in the literature. Of the cases reported, only ten involve a penetrating screwdriver. However, the choice of therapeutic management, whether it be emergency surgical or non-surgical removal of the penetrating object as well as the selected surgical approach remain quite controversial. CASE PRESENTATION: The authors describe the case of a severe penetrating skull base injury caused by a screwdriver, following an accidental fall from a ladder. The patient was admitted in Glasgow Coma Scale (GCS) 11 with a cerebrospinal fluid (CSF) leak in the right maxillary area. The tri-dimensional computerized tomography (3-D CT) scan revealed an oblique trajectory of the screwdriver shank through the skull base. The authors opted for an emergency surgical extraction of the object. A contra-lateral pterional approach was successfully performed and a two-year follow-up showed no neurological deficits. CONCLUSION: The reported case supports the choice of emergency surgical removal of the object in penetrating skull base injuries involving the anterior skull base with neurovascular lesions. Surgical aspects of the pterional approach, and in particular the left pterional approach as well as other cranio-facial approaches in severe penetrating skull base injuries are discussed.

Central neurocytoma: two case reports and review of the literature.

Central neurocytomas are low grade tumours usually located in the lateral ventricles next to Monro foramina. This paper reviews the literature on central neurocytomas observed in the last few years and discusses their clinical, histopathological, immunohistochemical and genetic characteristics. Important correlations between therapeutic strategies and biological findings as well as new genetic discoveries are also discussed. Two illustrative cases in which the authors report preliminary results about molecular analysis of some genetic markers are described.

Radiation-induced intracranial meningiomas: review of six operated cases.

It is well known that radiation can induce meningiomas. These tumors usually arise in patients with a history of low-dose radiation to the scalp for treatment of tinea capitis or high-dose radiation for a previous brain tumor. Radiation-associated meningiomas (RAMs) morphologically resemble their spontaneously arising counterparts. However, RAMs frequently present a more malignant phenotype and, as such, are diagnosed as "atypical" or "aggressive" meningiomas and occur predominantly in younger patients. This paper describes six cases of radiation-associated intracranial meningiomas in patients previously treated with low-dose radiation to the scalp for tinea capitis.