Intramedullary epidermoid cysts.

Intramedullary inclusion cysts are extremely rare within the rostral spinal cord. In this case report the authors outline the clinical features and surgical treatment of one dermoid cyst and one epidermoid cyst of the cervicothoracic junction. The authors also include a relevant literature discussion regarding the treatment and the embryological origin of these lesions.

Dorsal root ganglionectomy for pseudotumor of the L3 dorsal root ganglion: a rare case and a rare treatment.

Dorsal root ganglia are oval enlargements on the dorsal nerve roots and contain the cell bodies of sensory neurons. Asymmetry of dorsal root ganglia may occur naturally, yet natural occurrence of gigantic dorsal root ganglion (DRG) is rare. The patient was 61-year-old woman who presented with atypical symptoms like neuropathic pain and urinary distention. Neuroimaging has shown left L3-4 far-lateral disc herniation and a gigantic L3 DRG. At surgery, the dural sheath of the ganglion had to be opened and a firm, yellow-colored abnormal tissue was exposed. The abnormal tissue considered to be a tumor of neural origin was gross totally excised and the patient's symptoms ceased immediately after surgery. Histopathological examination of the specimen revealed nothing more than normal DRG morphology. At 4 months postoperatively, the patient is well with mild L3 hyperesthesia and hyperalgesia. Dural sheath opening in neurosurgery is not a routine practice. The sheath may need to be opened when surgeon suspects of a tumor, a free disc fragment and any inflammation within the ganglion. Operative morphology of a severely edematous but non-tumoral (pseudotumor) ganglion has not previously been documented.

Supratentorial cystic hemangioblastoma with infratentorial extension--a unique location and a rare infant case.

INTRODUCTION: Supratentorial occurrence of hemangioblastoma is an exceedingly rare event. Even rarer is the occurrence of a supratentorial hemangioblastoma in infancy. CASE REPORT: We hereby report the case of an 18-month-old girl who presented with irritability, increasing head size, and an open fontanelle. MR scans demonstrated triventricular hydrocephalus and a cystic mass within the left lateral ventricle. There was an 18 x 15 x 13 mm enhancing nodule along the medial aspect of the cyst. The cystic mass was mainly supratentorial but there was a caudal extension through the tentorial incisura that compressed the cerebellum. At surgery, the content of the cyst was xanthochromic. Enhancing medial nodule was extremely vascular and was extirpated totally. The pathological diagnosis was reticular variant of hemangioblastoma. CONCLUSION: The child is well at 4 years of age. An extensive review of the English literature revealed only three such cases. All three cases survived the operation.

Aggressive spinal germinoma with ascending metastases.

We report the case of a 28-year-old young man who presented with progressive paraparesis and urinary incontinence. Initial spinal MR scans showed a sausage-like lesion that extended between L1 and S2. At surgery, an encapsulated intradural extramedullary tumor was removed en bloc. The initial histopathological diagnosis was ependymoma. The tumor recurred locally to double its original size only 4 months later. After second surgery, 5,100 cGy of local spinal radiation was given since the pathologist believed that the new tumor exhibited anaplastic features. Subsequently tumor recurred at T6-10 levels and later in the right parasellar region. Surgery was undertaken for both recurrences and radiation to whole spine and to whole brain respectively followed surgery. At 11 months after the initial presentation, a new tumor was diagnosed at T11-T12 levels. After fifth surgery, chemotherapy with cisplatin, doxorubicin and vincristine was started. At this stage, review of all five surgical specimens by an outside neuropathologist was considered crucial. The new and correct histological diagnosis was germinoma. A new chemotherapy regimen targeted for germinoma was then started. The patient remains alive with no evidence of disease at 22 months after initial presentation. Primary spinal germinomas are exceedingly rare. A review of the literature revealed only 14 biopsy-proven spinal germinoma cases. Our case is clearly unique in aggressivity of the tumor, a feature often unexpected for germinomas. This case proves that the dissemination risk may be very serious for germinomas and that the craniospinal radiation may be a more secure treatment mode.

Intraparenchymal meningioma.

A 54 year-old woman presented with severe headache. Neuroimaging showed an enhancing intra-axial mass in the right superior temporal region associated with severe peritumoral oedema. Preliminary diagnosis was cerebral metastasis. The mass was surgically removed. Histopathology and immunohistochemistry showed the mass to be an intraparenchymal meningioma, which is rare. The relevant literature is reviewed.

Primary malignant rhabdoid tumor of the central nervous system--a comprehensive review.

This paper presents the case of an eight-year-old girl who presented with headache and vomiting and was found to harbor a right fronto-temporo-parietal, partially cystic and centrally solid tumor that measured 11 x 8 x 7 cm. This vascular tumor was gross totally removed. The initial histopathologic diagnosis was hemangiopericytoma and the patient received a total dose of 5330 cGy of external cranial radiation. Twelve months later, the patient presented with left lower quadrant pain and limping and the spinal MR scans showed metastases at T4-5, T7, T12-L1 and L3 levels. The voluminous lesion at T12-L1 was surgically removed. Histopathological examination of both specimens revealed that both tumors in fact were malignant rhabdoid tumor (MRT). The patient did not benefit from spinal surgery and died 4 months later. A review of the literature has shown that since Briner et al'. first report in 1985 [Pediatr Pathol 3: 117-118, 1985], 100 MRT cases have been published. More than two-thirds of reviewed cases presented with local recurrence or subarachnoid spread after a mean period of 6.9 months after diagnosis and died two months later. Infratentorial and pineal location and surgery limited to biopsy were poor prognostic indicators. Twenty-two cases remained alive at a mean period of 24.5 months. The longest survival with an intracranial MRT was 65 months. Of those remaining alive, 15 had no evidence of disease (NED). Our case is the first MRT case immunopositive for HMB-45 and has also shown that the MRT cells grow aggressive over time as demonstrated by a four-fold increase in MIB-1 labeling index.

Malignant cerebral infarction secondary to internal carotid injury in closed head trauma: good outcome with aggressive treatment--a case report.

A 25-year-old male patient in whom occlusion of the internal carotid artery developed secondary to a skull base fracture is presented. The diagnosis of internal carotid artery occlusion was reached 12 hours after the admission and 17 hours after the injury. The patient was initially treated for ischemic edema and when the patient showed signs of cerebral herniation, decompressive craniectomy was necessary. The outcome was good. The clinical and radiologic characteristics of internal carotid artery occlusion in closed head injury are highlighted and treatment options are reviewed in light of pertinent literature.

Triple neural tube defect--cranium bifidum with rostral and caudal spina bifida--live evidence of multi-site closure of the neural tube in humans.

OBJECTIVE: The coexistence of three neural tube defects (NTDs) in a single child is an exceptional event. A review of the literature revealed nine published "double" NTD cases, but no cases of "triple" NTDs have been reported to date. CASE REPORT: The rare case of a two-year-old boy with three distinct NTDs is presented. The boy had a 17x15x15-cm(3) parieto-occipital encephalocele, a small cervical myelomeningocele, and a 11x11x8-cm(3) thoracolumbar myelomeningocele. Hydrocephalus and Chiari II malformation accompanied the NTDs. All three lesions were surgically treated with good cosmetic results and satisfactory neurologic outcome. CONCLUSIONS: Current neural tube closure theories and models are reviewed in an attempt to better understand this extremely unusual coexistence. The multi-site closure model is clearly more useful in our understanding of NTDs.

Anaplastic pleomorphic xanthoastrocytomas. Review of the literature with reference to malignancy potential.

Malignancy potential of pleomorphic xanthoastrocytomas (PXAs) has rather been an underestimated reality. We report the case of a 13-year-old boy who presented with signs of increased intracranial pressure. The child had been epileptic since the age of 2. Computed tomography and magnetic resonance scans revealed a huge left frontal mass. At surgery, a subtotal excision was accomplished. Histopathological diagnosis was anaplastic PXA (grade III; WHO, 2000). The tumor showed an increased mitotic index and minimal endothelial proliferation. The patient died 3.5 months later due to a fatal intracranial hemorrhage. A review of the entire PXA literature revealed 15 well-documented cases of PXA with subsequent malignant transformation and 11 cases of primary anaplastic PXA. The prognosis was grim for both subsets of patients. Anaplastic PXAs clearly represent the transition between the original PXA concept and lipidized giant-cell glioblastoma.

Extradural lumbosacral cavernous hemangioma.

Purely extradural cavernous hemangiomas of the spinal canal are extremely rare. Their occurrence at the lumbosacral level is a true exception. We describe a case of lumbosacral extradural hemangioma and review the behaviour and MR imaging characteristics of these lesions. Cavernous hemangioma should be considered in the differential diagnosis of an enhancing extradural periradicular mass causing chronic lumbar radiculopathy especially in fertile women. Lumbar extradural hemangiomas appear and behave differently, not only from intramedullary cavernous hemangiomas but also from extradural hemangiomas of other spinal locations.

Post-traumatic intradiploic leptomeningeal fistula and cyst.

A 59 year old female patient presented with ataxia and difficulty in walking. The neurological examination revealed right homonymous hemianopia and ataxia. Radiographic evaluation revealed a large occipital intradiploic cyst mainly in the left suboccipital area. There was also moderate hydrocephalus and encephalomalacia of the left occipital pole. Bone window studies also demonstrated a growing fracture extending from the upper pole of the cyst to the vertex. Both pathologies were attributed to child abuse the patient suffered when she was a child. At first surgery, decompression of the cerebellum was followed by duroplasty and acrylic cranioplasty to the posterior cranial fossa. A month later, a shunt had to be inserted for hydrocephalus. At 7 months postoperatively, the patient is well and free of any symptoms or recurrence.