1/19. Heterotopic nasopharyngeal brain tissue associated with cleft palate.OBJECTIVE: The occurrence of extracranial brain tissue is rare. Most of the literature describes cases in which it is located around the nose and throat and has been classified as nasal glioma. Even more unusual is heterotopic brain tissue in the nasopharynx. We were able to find only 17 previously reported cases. Of these 17 cases, 6 had heterotopic brain tissue located in a cleft palate. This report comments on the identification and treatment of heterotopic brain tissue associated with cleft palate without connection to the central nervous system. Our case subject is a 10-month-old girl diagnosed with heterotopic nasophranygeal brain tissue associated with cleft palate. RESULTS: Excision and palatoplasty were performed conjunctively with excellent results. CONCLUSIONS: Simultaneous excision of heterotopic nasopharyngeal brain tissue and palatoplasty of the cleft palate is an excellent option for treatment of these cases.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/19. Nasal NK/T cell lymphoma presenting as transverse myelopathy.A case of nasal NK/T cell lymphoma with central nervous system (CNS) involvement is reported. A 56-year-old man presented with eyelid edema and transverse myelopathy. cerebrospinal fluid examination revealed atypical lymphoid cells with azurophilic granules, which were positive for CD2, CD8, and CD56, and negative for CD3 and CD5 by flow cytometry. Because a tumor mass was found involving the ethmoid and maxillary sinuses, CNS involvement was considered to have resulted from local invasion by the nasal lymphoma. In spite of intensive chemotherapy including intrathecal infusion, the patient died 6 months after the initial diagnosis. autopsy revealed that lymphoma cells were positive for cytotoxic molecules, granzyme B and TIA-1, and EB virus-encoded rna-1 (EBER-1), and they showed no rearrangement of TCR-beta, -gamma, or -delta genes, suggesting an NK-cell origin of the lymphoma cells. They showed an angiocentric and angiodestructive pattern in the subarachnoid space, focally extending to the cerebral cortex and cranial and spinal nerve roots. Marked demyelination was found in the lateral and posterior funiculi of the spinal cord. Thus, the pathogenesis of this spinal demyelination might be attributed to ischemia secondary to angiocentric and angiodestructive infiltration by lymphoma cells.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/19. Sinonasal undifferentiated carcinoma: case series and review of the literature.OBJECTIVE AND IMPORTANCE: We report on four cases of sinonasal undifferentiated carcinoma (SNUC), a relatively newly described clinicopathological entity of the nasal cavity and paranasal sinuses. SNUC tends to present with advanced-stage disease, often with intracranial invasion, and requires an aggressive treatment approach that includes surgical resection. A review of the literature identified several reports of SNUC in pathology and otolaryngology journals since its initial description in 1986, but no report has yet appeared in the neurosurgery literature. CLINICAL PRESENTATION: Four patients presented with various symptoms related to the nose and/or orbit, including one or more of the following: obstruction, epistaxis, decreased visual acuity, diplopia, and pain. All patients were noted to have masses in the nasal cavity or paranasal sinuses, with or without intracranial extension. INTERVENTION: All four patients underwent multimodal treatment with chemotherapy, radiotherapy (60-65 Gy), and aggressive surgical resection via a combined bifrontal craniotomy and a subcranial approach to the anterior cranial fossa. Three of four patients died as a result of their disease, an average of 15 months after diagnosis. Only one patient remains alive, although with metastatic intracranial disease, at 24 months after diagnosis. CONCLUSION: SNUC is a rare neoplasm with a poor prognosis despite an aggressive multimodal approach to treatment. On the basis of our experience, we advocate radical resection as part of the initial combined therapy for patients who present with locally advanced, nonmetastatic disease but we suggest reserving surgery for patients with early brain invasion until there has been a radiographically proven central nervous system response to adjuvant therapy.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/19. Evaluation of central nervous system involvement in nasal lymphomas.OBJECTIVES: To clarify the usefulness of evaluating central nervous system (CNS) involvement in patients with nasal lymphomas at the initial staging procedure, and of CNS prophylaxis for patients with clinical stage I/II. patients AND methods: We retrospectively reviewed 43 patients with nasal lymphomas who had been treated from 1973 through 1999. The staging procedure included mainly computed tomography (CT), ultrasonography, gallium scintigraphy, upper gastrointestinal study, magnetic resonance (MR) imaging, and bone marrow biopsy. Forty-two patients received radiotherapy, and 25 patients received chemotherapy. All 38 patients with stage I/II were not subjected to CNS prophylaxis. RESULTS: Four patients demonstrated CNS involvement at the staging procedure. MR imaging demonstrated the tumor had directly infiltrated the skull base in 3 patients, but CT demonstrated CNS infiltration in only one patient. In another patient, cerebrospinal fluid (CSF) cytologic analyses demonstrated CNS involvement, but MR imaging and CT did not. These 4 patients complained of frontonasal pain and/or cerebral nerve dysfunction. No patient with stage I/II developed CNS relapse. CONCLUSIONS: MR imaging and CSF cytologic analyses should be performed at the initial staging of nasal lymphomas, especially in patients with frontonasal pain and/or cerebral nerve dysfunction. patients with stage I/II might not need CNS prophylaxis.- - - - - - - - - - ranking = 5keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/19. Midline nasal mass in infancy: a nasal glioma case report.Congenital midline nasal masses are rare anomalies that occur in about one in 20,000-40,000 live births. The most common are dermoid/epidermoid tumors, nasal cerebral heterotopias (nasal gliomas), and nasal encephaloceles; some have an actual or potential central nervous system connection. Nasal gliomas are CNS masses of neurogenic origin which have lost their intracranial connections and present as an obvious external or intranasal mass at birth without associated surgical symptoms. Careful evaluation is required to confirm the diagnosis and appropriate management. The interpretation of CT and MR images can be difficult but is useful in differentiating nasal gliomas from other congenital nasal masses. The presence of a fibrous stalk may be associated with cranial defects and CSF leak. Excisional biopsy allows histopathologic diagnosis and is the definitive treatment. They are benign lesions, and recurrences are rare, so conservative cosmetic surgical techniques should be chosen for gliomas where there is no proven intracranial extension. The authors report an illustrative nasal glioma case in a one-year-old male infant with extranasal and intranasal components, and discuss the therapeutic options.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/19. Nasal glioma or nasal glial heterotopia? Morphological, immunohistochemical and ultrastructural study of two cases.The term nasal glioma has been used to describe a congenital benign tumor of the nasal region containing neural tissue. The nature of these lesions remains open to controversy, because of the different locations of the heterotopic neural tissue involved, the deficient development of the bony structures and the persistence or not of the structural relations with the central nervous system. More recent terms define these lesions as ectopic nervous tissue. A clinical, morphological, ultrastructural and immunohistochemical study is made of two cases of nasal glioma, one associated with agenesis of the corpus callosum. In this case, the mother had been treated with clomiphene. In such cases, morphological and immunohistochemical findings support that "nasal glioma" remain valid as a descriptive term defining a congenital benign tumor composed of heterotopic neural tissue within the nasal region and covered by skin, that may recur following incomplete surgical resection.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/19. Pseudotumor of the nasal fossa secondary to mucoid impaction in choanal atresia.An intranasal mass was discovered during evaluation of unilateral choanal atresia. Radiologic investigation of the lobulated cystic mass showed no communication with the central nervous system or any other bony abnormalities. During surgery for the choanal atresia, the mass proved to be a mucous impaction. The case illustrates the need to utilize decongestants and aspiration prior to instillation of contrast agent.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/19. Plasmablastic lymphoma presenting in a human immunodeficiency virus-negative patient: a case report.Plasmablastic lymphoma (PBL), an aggressive non-Hodgkin's lymphoma that carries a poor prognosis, previously has been identified almost exclusively in patients infected with the human immunodeficiency virus (hiv). We present a case of a 42-year-old hiv-negative patient presenting with an isolated nasal cavity mass, the typical presentation for PBL. The patient was given systemic chemotherapy, central nervous system prophylaxis, and consolidative locoregional radiotherapy and achieved a complete clinical response. This case suggests PBL should be considered in hiv-negative patients with characteristic findings.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/19. Treatment of postrenal transplantation lymphoproliferative disease manifesting as plasmacytoma with nonmyeloablative hematopoietic stem cell transplantation from the same kidney donor.Posttransplantation lymphoproliferative disease (PTLD) presenting as an Epstein-Barr-virus (EBV)-related nasal plasmacytoma developed in a renal-allograft recipient 13 years after transplantation. Systemic dissemination occurred despite immunosuppression withdrawal, surgery, irradiation, and chemotherapy. A nonmyeloablative hematopoietic-stem-cell-transplantation (HSCT) with peripheral blood HSC from the kidney donor was performed. With the onset of graft-versus-host disease, resolution of the systemic disease was demonstrated clinically and molecularly by serial quantification of plasma EBV-dna. Isolated relapse occurred in the central nervous system (CNS), a known tumour sanctuary site, ultimately leading to death. Nonmyeloablative HSCT might be considered a cellular therapy for PTLD, but possible CNS relapse must be effectively tackled.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/19. Intracranial nasal natural killer/T-cell lymphoma: immunopathologically-confirmed case and review of literature.Advances in immunophenotypic profiling now permit characterization of natural killer/T-cell (NK/T-cell) lymphoma as distinct from other extranodal T- and B-cell Non-Hodgkin's lymphomas. NK/T-cell lymphoma presents most commonly in the nasal cavity. disease progression to the central nervous system (CNS) is a rare phenomenon. We present here, to our knowledge, the first immunophenotypically-confirmed case of direct extension of nasal NK/T-cell lymphoma to the brain. In addition, we review the literature with respect to NK/T-cell lymphoma metastasis to the CNS. The overall prevalence of NK/T-cell lymphoma CNS metastasis is less than 3%. Although rare, CNS invasion portends a poor prognosis, emphasizing the importance of early and accurate immunophenotype profiling and the need for novel, aggressive therapy.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
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