1/44. Primary meningeal rhabdomyosarcoma in a child with hypomelanosis of Ito.Intracranial rhabdomyosarcomas are rare neoplasms, and those thought to be primary meningeal tumors are even more rare. Hypomelanosis of Ito is a neurocutaneous disorder believed to involve a defect in cells of neural crest origin. We report the case of a 15-month-old boy with hypomelanosis of Ito who developed a primary meningeal rhabdomyosarcoma. The patient initially presented with hydrocephalus and 2 months later developed neurologic signs localizing to the spinal cord. Radiologic studies revealed widespread leptomeningeal enhancement with compression of the spinal cord at C5-C7. A brain biopsy revealed a tumor diffusely involving the meninges. Microscopically, the tumor was composed of rhabdomyoblasts, many of which showed prominent cross-striations on routine hematoxylin-eosin staining. To the best of our knowledge, this is the first reported case of meningeal rhabdomyosarcoma in a patient with hypomelanosis of Ito and the fourth reported case of a primary meningeal rhabdomyosarcoma reported in the world literature.- - - - - - - - - - ranking = 1keywords = neurocutaneous (Clic here for more details about this article) |
2/44. Neurocutaneous vascular hamartomas mimicking Cobb syndrome. Case report.The authors report the rare case of a patient with neurocutaneous vascular hamartomas mimicking Cobb syndrome. An 8-year-old boy was admitted to the authors' hospital with progressive urinary disturbance and upper back pain. Multiple skin nevi had been noted at the child's birth. Radiological examination revealed multiple cavernous angiomas in the spinal cord in the same metamere in which the skin nevi had been observed and also in the left cerebral hemisphere. His symptoms gradually improved without surgical intervention. Four years later he was readmitted because of a cerebral hemorrhage involving the left cerebral peduncle. Nonsurgical treatment was chosen because his symptoms promptly improved. To the best of the authors' knowledge, this is the first case of multiple cavernous angiomas in the brain and spinal cord associated with skin nevi. The authors discuss this clinical entity and the significance of the disease.- - - - - - - - - - ranking = 1keywords = neurocutaneous (Clic here for more details about this article) |
3/44. radiosurgery of meningeal melanocytoma.The authors present a case of meningeal melanocytoma arising from Meckel's cave. A coal-black, vascular tumor was partially removed by surgery. Histopathologically, the tumor lacked anaplastic features. Immunohistochemical studies confirmed that the tumor was of neuroectodermal origin and had low proliferating activity. The patient underwent gamma knife radiosurgery for the residual tumor, in which 25 Gy of radiation was delivered to the tumor margin. Three years after irradiation, the tumor showed marked shrinkage without complication.- - - - - - - - - - ranking = 0.04566194556349keywords = neuroectodermal (Clic here for more details about this article) |
4/44. Early diffuse leptomeningeal primitive neuroectodermal tumors can escape detection by magnetic resonance imaging.Primitive neuroectodermal tumors are easily detected by neuroradiologic imaging, as a rule. We report on two patients with early diffuse leptomeningeal primitive neuroectodermal tumors which escaped detection by contrast-enhanced magnetic resonance imaging.- - - - - - - - - - ranking = 0.27397167338094keywords = neuroectodermal (Clic here for more details about this article) |
5/44. Surgical management of meningioma en plaque of the sphenoid ridge.BACKGROUND: meningioma en plaque represents a morphological subgroup within the meningiomas defined by a carpet or sheet-like lesion that infiltrates the dura and sometimes invades the bone. Differential diagnosis includes fibrous dysplasia, osteoma, and osteoblastic metastasis. This study was conducted to obtain pathological information on patients with meningioma en plaque and to correlate with the surgical management. methods: A retrospective review of all the adult operative cases at the University Hospital in a seven and a half-year period from July 1, 1990 to December 31, 1997 identified 150 patients who were operated on for intracranial meningiomas. The medical records were reviewed to identify cases of meningioma en plaque. Forty-seven patients had involvement of the sphenoid ridge and 6 of them fulfill the criteria for meningioma en plaque. RESULTS: All 6 patients with meningiomas en plaque were female and had hyperostosis of the sphenoid bone. In 5 of them, the bone was sent for histopathological examination. Four of those had infiltration of the bone by meningioma cells. Proptosis was the most common presentation. Half of the patients presented with visual disturbances that improved after surgery. All patients were operated using a fronto-temporal approach with orbital decompression. CONCLUSION: All the involved bone should be removed to prevent recurrence. In those cases with involvement of the cavernous sinus and/or the orbital apex, a subtotal but extensive removal combined with bony decompression of the cranial nerves at the superior orbital fissure and optic canal frequently produces good functional and cosmetic results.- - - - - - - - - - ranking = 0.00012922707294402keywords = dysplasia (Clic here for more details about this article) |
6/44. Intracranial meningeal melanocytoma associated with ipsilateral nevus of ota. Case report.In this report, the authors review the case of a man with a neurocutaneous syndrome. He presented with an intracerebral melanocytoma associated with a blue nevus of the scalp; its location and its appearance during childhood supported the diagnosis of a nevus of ota. Meningeal melanocytomas are increasingly being diagnosed, but remain rare. Primary meningeal malignant melanoma is the first differential diagnosis to eliminate. Despite their common embryonic origin. the association of a melanocytoma with a nevus of ota is rare. A nevus of ota exhibits the same melanocytic proliferation and affects the trigeminal nerve territory. An ocular effect is not always observed. In contrast to an ocular lesion, a nevus of ota rarely transforms into a malignant melanoma. It is found only among caucasians. During 4 years of follow-up review after surgery, the patient remained asymptomatic. Other than antiepileptic therapy, he received no complementary treatment and cerebral imaging revealed no evidence of recurrence.- - - - - - - - - - ranking = 1keywords = neurocutaneous (Clic here for more details about this article) |
7/44. Primary peripheral PNET/Ewing's sarcoma of the dura: a clinicopathologic entity distinct from central PNET.We describe two cases of peripheral primitive neuroectodermal tumor-Ewing's sarcoma (PNET-ES) arising intracranially in the leptomeninges. Both tumors exhibited a primitive undifferentiated round-cell morphology. Immunohistochemical stains revealed strong membrane expression of CD99 in both cases. A t(11;22)(q24;q12) could be demonstrated with reverse transcriptase-polymerase chain reaction in one case, whereas fluorescence in situ hybridization analysis performed in the second case showed a rearrangement of the EWS gene. The occurrence of PNET-ES at this site is very unusual. Immunophenotypical as well as genetic analysis play a key role in the diagnosis and the distinction from central PNET.- - - - - - - - - - ranking = 0.04566194556349keywords = neuroectodermal (Clic here for more details about this article) |
8/44. Spinal low-grade neoplasms with extensive leptomeningeal dissemination in children.OBJECTIVE: The aim of this study is to re-appraise paediatric leptomeningeal disseminated spinal low-grade neoplasms putting forward the hypothesis that these tumours may represent a possible distinctive clinico-pathological entity. CASE REPORT: Three children affected by these rare neoplasms are reported, 2 boys and 1 girl aged 40 months, 7 and 12 years respectively. RESULTS: Despite treatment, 2 died at 9 months and 9 years, while 1 has survived with stable disease at 24 months after diagnosis. Histologically, these tumours shared the generic histological appearance of low-grade neuroectodermal neoplasms, without any features that allowed them to be included in a specific classification niche. The magnetic resonance imaging of the leptomeningeal dissemination took the form of a thick, diffuse subarachnoidal enhancement involving the brain as well the spine in 2 patients; in 1 patient there was an extensive enhancement along the surface of the brain and of the spinal cord and multiple small cysts, creating the impression of a diffuse microcystic meningoencephalopathy. DISCUSSION: The patients described here and the similar ones found in the literature cannot be easily placed into the present WHO system of classification for central nervous system tumours.- - - - - - - - - - ranking = 0.04566194556349keywords = neuroectodermal (Clic here for more details about this article) |
9/44. Multiple subpial lipomas with dumb-bell extradural extension through the intervertebral foramen without spinal dysraphism.BACKGROUND: Intradural subpial lipomas not associated with spinal dysraphism, account for less than 1% of spinal cord tumors. The simultaneous existence of multiple intradural subpial lipomas with dumb-bell extradural extension through the intervertebral foramen in the same patient without any evidence of spinal dysraphism has not been previously reported. CASE DESCRIPTION: A 38-year-old man presented with progressive spastic paraparesis, and weakness of right elbow extension and opposition of the medial three fingers. He also had ascending paraesthesia from the C6 dermatome to the saddle region and loss of joint and position sense of both lower limbs with hesitancy and precipitancy of micturition. There was no spinal tenderness, deformity, neurocutaneous markers, or spinal dysraphism. The total duration of illness was 11 years.The oblique views of the plain radiographs of the cervical spine revealed an enlarged right C7-D1 intervertebral foramen. The T1- and T2-weighted magnetic resonance (MR) images showed two intradural, hyperintense lesions (with extensive loss of signal on fat suppression sequences), one extending from C5 to D2 and the other opposite the C3-4 disc space. The parasagittal and axial images showed the extradural component of the lesion emerging from the right C7-D1 intervertebral foramen.At surgery, a C2 to D2 laminectomy was performed. The lipoma, enclosed in a fine pial membrane, was situated on the right posterolateral aspect of the cord. The right-sided nerve roots from the C6 to D1 levels were completely enmeshed by the lipoma. There was a separate superficial subpial lipoma adherent to the posterior aspect of the cord at the C3-4 level. A distinct area of normal cord was present between the two lesions. A subtotal decompression of the lesions including the component emerging through the right C7-D1 intervertebral foramen and a duraplasty were performed.At follow-up after 18 months, the posterior column impairment, lower limb hypoaesthesia, and right upper limb paraesthesia had improved. However, residual elbow extension and lower limb weakness, mild lower limb spasticity and sphincteric dysfunction persisted. CONCLUSIONS: The multiplicity of intradural subpial lipomas without spinal dysraphism points to a dysembryogenetic basis similar to that seen in patients with spinal dysraphism that results in lipomas, but in which the defect is not severe enough to give rise to coexisting vertebral and soft tissue anomalies. The dumb-bell extradural extension through the intervertebral foramen is extremely rare. The magnetic resonance imaging and surgical principles are discussed.- - - - - - - - - - ranking = 1keywords = neurocutaneous (Clic here for more details about this article) |
10/44. Primitive neuroectodermal tumor in the cerebellopontine angle with isochromosome 17q presenting as meningioma in a woman 26 years of age.An unusual posterior fossa neoplasm in a 26-year-old woman with short history of the cerebellar symptoms is presented. CT and MR images showed the tumor within the cerebellopontine angle, suspected as meningioma. At surgery, the tumor was dura-attached and did not infiltrate the arachnoid. Histologically, the neoplasm was a small blue cell tumor with solid and microcystic pattern, consistent with primitive neuroectodermal tumor (PNET). Immunohistochemically the cells were strongly positive for NCAM and GFAP. fluorescence in situ hybridization (FISH) was performed with the cosmids G9 and F7 (flanking EWSR1/22q12 region) dna probes and dual-color spectrum-orange LSI HER-2/neu (17q11.2)/spectrum green CEP17 (17p11.1-q11.1) DNA probe. The presence of isochromosome 17q within neoplastic cells was found. The tumor was classified as a medulloblastoma. We demonstrate the utility of a multidisciplinary approach to nervous system tumor diagnosis. The clinical features together with histological, immunohistochemical, and characteristic molecular alteration allowed classification of the presented case.- - - - - - - - - - ranking = 0.22830972781745keywords = neuroectodermal (Clic here for more details about this article) |
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