1/115. Primary non-Hodgkin's lymphoma of brachial plexus.We report the case of a 65-year-old man with non-Hodgkin's lymphoma (NHL) not only in the brachial plexus but also in the central nervous system and parotid gland. He was referred to our hospital for evaluation of a right parotid mass. He also presented with bilateral facial palsy and paralysis of the left superior limb. Computed tomography scan and magnetic resonance imaging revealed mass lesions in the right parapharyngeal space, the deep lobe of the right parotid gland. and the left brachial plexus. A gallium-67 citrate scan demonstrated abnormal uptake in the left brachial plexus. These symptoms and lesions improved during steroid therapy. However, the symptoms worsened again after steroid therapy was discontinued. We performed a right parotidectomy to confirm the diagnosis. Histopathological study revealed NHL. He was treated with combination chemotherapy, and most of the lesions and symptoms, except bilateral facial palsy, improved. Despite follow-up treatment, a brain metastasis occured, and he died 16 months after the onset of symptoms.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/115. Molecular analysis of malignant triton tumors.Triton tumors are rare variants of malignant peripheral nerve sheath tumor (MPNST) with muscle differentiation, often seen in patients with neurofibromatosis 1 (NF1). Individuals affected with NF1 harbor mutations in the NF1 tumor suppressor gene and develop neurofibromas and MPNSTs. The NF1 gene is expressed in schwann cells and its expression is lost in schwannian neoplasms, suggesting a role in malignant development. Separately, there is evidence that p53 suppressor gene mutations are involved in MPNSTs. To determine the role of the NF1 and p53 genes in the development of the malignant Triton tumor we examined 2 such tumors, 1 from a 3-year-old boy without clinical manifestations of NF1 and another from a 24-year-old man with NF1. Histological analysis of these tumors showed both neural and muscle differentiation with S-100 and desmin immunoreactivity, respectively. Reverse transcribed rna polymerase chain reaction (RT-PCR) of NF1 mRNA showed NF1 expression in the sporadic tumor. Strong nuclear immunoreactivity for p53 was observed throughout the malignant population in both tumors. This was confirmed by loss of heterozygosity for p53 in the non-NF1 patient, suggesting that p53 is involved in both hereditary and sporadic Triton tumors. The finding of preserved NF1 gene expression in the non-NF1-related Triton tumor suggests that different genetic events predispose to the development of this rare neoplasm in sporadic cases.- - - - - - - - - - ranking = 1765.3147364742keywords = neoplasm (Clic here for more details about this article) |
3/115. Leptomeningeal metastases from ethmoid sinus adenocarcinoma: clinico-radiological correlation.A patient with sinonasal adenocarcinoma is presented with leptomeningeal metastases affecting multiple cranial nerves and spinal nerve roots. head and neck cancer is known to be an extremely rare source for leptomeningeal metastatic spread. The cranial nerves, the spinal cord and roots and the cerebral hemispheres can be affected in case of leptomeningeal metastatic spread. Examination of the CSF is the hallmark of the diagnosis if leptomeningeal metastatic spread is suspected, but this case illustrates that the combination of specific clinical features on one hand and specific lesions on the Gd-enhanced T1-weighted MRI study on the other hand is reliable enough to make a presumed diagnosis if the CSF analysis remains negative. We suggest that in our patient direct leptomeningeal spread occurred through the cribriform plate to the CSF, followed by further spread in a gravity dependent way.- - - - - - - - - - ranking = 0.16457846531061keywords = cerebral (Clic here for more details about this article) |
4/115. paraganglioma of the cauda equina. Case report with 33-month recurrence free follow-up and review of the literature.paraganglioma of the cauda equina is an unusual tumor and do not have the secretory properties of the same tumors arising outside the nervous system. In none of the few cases reported in literature a preoperative diagnosis was possible, and the surgical findings raised questions in the differential diagnosis with ependymomas. A rare case of paraganglioma of the cauda equina studied both pre- and postoperatively by MRI, and treated with subtotal excision combined with radiotherapy is described. Results and recurrence rates of the cases reported in literature are reviewed. Though MRI imaging has proven to be more sensitive than other radiological procedures, we stress the difficulties of preoperative diagnosis of paragangliomas in this site. The correct diagnosis of the paraganglioma of the cauda equina still relies on immunochemistry and electron microscopy. Total excision is often very difficult owing the tendency of these neoplasms to infiltrate cauda's roots. A 33-month recurrence free follow-up of our patient confirms that successful treatment is achieved by subtotal resection combined with radiotherapy.- - - - - - - - - - ranking = 882.65736823712keywords = neoplasm (Clic here for more details about this article) |
5/115. Neoplasm as a cause of brachial plexus palsy in neonates.Two patients with neonatal onset of arm weakness resulting from neoplastic involvement of the brachial plexus who were initially considered to have obstetric brachial plexus palsies are reported. The first patient was a 7-day-old female who presented with a left supraclavicular mass that was first detected at 2 days of age and left proximal arm weakness. The weakness involved the whole arm within 3 days. The mass was a malignant rhabdoid tumor. The second patient was a 28-month-old male who presented with slowly progressive right arm weakness, which began at 3 weeks of age, and episodes of scratch marks on the arm that began at 4 months of age. magnetic resonance imaging revealed a plexiform neurofibroma of the brachial plexus. The features that are suggestive of a brachial plexus palsy caused by a neoplasm rather than of obstetric brachial plexus palsy include the following: the onset of weakness after the first day of age, with a progressive course; a history of a normal delivery and birth weight; the absence of signs of a traumatic injury or injuries; the appearance before 7 days of age of a growing supraclavicular mass without radiographic evidence of a clavicular fracture; and recurrent scratch marks on the weak arm.- - - - - - - - - - ranking = 882.65736823712keywords = neoplasm (Clic here for more details about this article) |
6/115. Gastrointestinal autonomic nerve (GAN) tumor of the rectum.Gastrointestinal autonomic nerve (GAN) tumor is a relatively newly defined member of the gastrointestinal stromal tumor family, which is a rare group of mesenchymal neoplasms originating in the bowel wall throughout the entire gastrointestinal tract. A thorough search of the literature revealed only one brief mention of this tumor arising in the rectum. We present a full description of such a tumor and summarize the clinical characteristics, therapeutic measures used, biological behavior, and outcome of this unique case.- - - - - - - - - - ranking = 882.65736823712keywords = neoplasm (Clic here for more details about this article) |
7/115. ganglioneuroblastoma of the cauda equina.A 39-year-old lady presented with low back pain and neurogenic claudication. Magnetic resonance imagining revealed an intradural neoplasm in the cauda equina region. The patient underwent lumbar laminectomy and total excision of the neoplasm. biopsy showed it to be a ganglioneuroblastoma, which is rare in the spinal canal and so far does not appear to have been reported in the region of the cauda equina. Its management is discussed.- - - - - - - - - - ranking = 1765.3147364742keywords = neoplasm (Clic here for more details about this article) |
8/115. Primary lymphoma of peripheral nerve: report of four cases.lymphoma presenting as a solitary tumor of peripheral nerve is exceedingly rare, with only six previously reported cases. The authors describe an additional four cases of primary lymphoma of peripheral nerve involving the sciatic nerve (two cases), the radial nerve, and the sympathetic chain and spinal nerve. The patients were two men and two women with an average age of 55.5 years. All tumors were high-grade B-cell lymphomas. Two patients experienced relapse of disease with involvement of other nervous system sites and died of lymphoma. One patient is alive with stable local disease at 57 months. The fourth patient is alive with no evidence of disease at 54 months. Expression of neural cell adhesion molecule (CD56) has been reported to correlate with an increased incidence of central nervous system involvement in peripheral T-cell lymphoma; all their cases were CD56 negative. Recent reports indicate a high proportion of primary brain lymphomas show loss of CDKN2A/p16 gene expression. Therefore, CDKN2A/p16 was evaluated in their patients both by polymerase chain reaction and by immunohistochemistry for the p16 protein. The authors found homozygous deletion of the CDKN2A/p16 gene in one of three patients studied, confirmed immunohistochemically by absent staining for p16. The fourth patient showed absent staining for p16, suggesting inactivation of the gene in this case as well. The two patients with p16 loss both died of lymphoma, whereas the two patients with normal p16 expression are alive. Primary lymphoma of peripheral nerve is a rare neoplasm, usually of large B-cell type, has a variable prognosis, and appears to have less consistent loss of p16 expression than primary central nervous system lymphoma.- - - - - - - - - - ranking = 883.65736823712keywords = neoplasm, brain (Clic here for more details about this article) |
9/115. Primary primitive neuroectodermal tumor of the cauda equina.Primitive neuroectodermal tumors (PNETs) are aggressive neoplasms composed predominantly of undifferentiated cells that show evidence of neural differentiation. Although their classification has been controversial, PNETs are well recognized primary tumors of both central and peripheral nervous systems. PNETs must be distinguished from other round-cell tumors, including Ewing's sarcoma, lymphoma, rhabdomyosarcoma, and small cell carcinoma. Intraspinal PNETs are rare neoplasms that are usually metastatic in origin. We describe the eighth reported primary PNET of the cauda equina that developed in a 52-year-old man with no significant medical history. The tumor was characterized by Homer-Wright rosettes and immunoreactivity for CD99, glial fibrillary acidic protein, neuron-specific enolase S100, and synaptophysin. The anatomic location of primary intrathecal PNETs is important as those arising in the spinal cord develop in the central nervous system, whereas those arising in the cauda equina develop in the peripheral nervous system. The histogenesis of intrathecal PNETs may be multifactorial.- - - - - - - - - - ranking = 1765.3147364742keywords = neoplasm (Clic here for more details about this article) |
10/115. Haemangioblastoma of a cervical sensory nerve root in Von Hippel-Lindau syndrome.Spinal haemangioblastomas are rare, accounting for only about 7% of all central nervous system cases. The case of a 40-year-old woman with a haemangioblastoma arising solely from a cervical sensory nerve root is presented. At operation via a cervical laminectomy, it was possible to resect the tumour en masse with the sensory ramus, by extending the laminectomy through the exit foramen for C6. Haemangioblastomas are commonly intramedullary, and have only been reported in this location on one previous occasion. The patient has Von Hippel-Lindau syndrome and a history of multiple solid tumours. The possible role of the Von Hippel-Lindau tumour suppressor gene in the pathogenesis of these neoplasms is discussed.- - - - - - - - - - ranking = 882.65736823712keywords = neoplasm (Clic here for more details about this article) |
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