Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Olfactory neuroblastoma is a rare, malignant neoplasm arising from the olfactory epithelium. It has an aggressive biological behavior that is characterized by local recurrence, atypical distant metastasis, and poor long-term prognosis. The incidence of cervical lymph node metastasis in olfactory neuroblastoma is variable, and treatment modalities are controversial. Moreover, few reports have been published concerning retropharyngeal lymph node metastasis from olfactory neuroblastoma. We present two cases of olfactory neuroblastoma with retropharyngeal lymph node metastasis. In addition, we provided a review of the current literature regarding olfactory neuroblastoma and retropharyngeal lymph node metastasis from olfactory neuroblastoma.
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PMID:Retropharyngeal lymph node metastasis from olfactory neuroblastoma: A report of two cases. 1669 16

Olfactory neuroblastoma is such a rare malignancy that no consensus has been reached on its management. We analyzed 17 patients with olfactory neuroblastoma treated between April 1980 and March 2004--9 men and 8 women, aged 16 to 76 years old(mean: 50.4 years). Follow-up of current survivors was 1 year 8 months to 16 years 6 months (average: 7 years 9 months). Initially, 2 were treated with surgery alone, 5 with surgery and radiotherapy, and 2 with a combination of these and chemotherapy. Without surgery, radiotherapy alone was conducted in 3 and combined of radiation and chemotherapy in 5. Three of the 5 patients treated with surgery and radiotherapy survive without locoregional recurrence as do 2 with chemotherapy added. All 5 initially treated with craniofacial resection survived more than 5 years. Combined radiotherapy and chemotherapy without surgery was effective in 2. 5- and 10-year overall survival for all patients were 75.5% and 64.7%. Overall 5-year survival of 8 patients with low-grade tumors was 87.5% and of 6 with high-grade tumors 33.3%. In conclusion, combined craniofacial resection plus radiotherapy and chemotherapy seemed to improve survival. Histopathological grading is a prognostic factors.
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PMID:[Olfactory neuroblastoma: the Hokkaido University experience]. 1676 58

Olfactory neuroblastoma is an uncommon neoplasm. Typically, these tumors are indolent with long-standing symptomatology, but the fact that the lesions are indeed malignant has been proven by the repeated demonstration that they can metastasize to distant organs. Suitable prognostic factors are lacking and therapeutic strategy still remains controversial. Expression of human telomerase reverse transcriptase (hTERT) is associated with most human malignancies and high levels have been correlated with poor prognosis in many cancers. In comparison, overexpression of cyclin-D1 occurs in several malignancies and has been associated with aggressive tumor behavior and poorer prognosis. In this study, we collected 16 olfactory neuroblastomas from the Kaohsiung Medical University Hospital. The aim was to investigate the value of immunoexpression of hTERT and cyclin-D1 in correlation with clinicopathologic features of olfactory neuroblastoma. Low and high cyclin-D1 expression was found in 6 and 10 cases, respectively. For hTERT, low and high protein expression was detected in 5 and 11 tumors, respectively. Cyclin-D1 expression was not correlated with selected parameters. However, high hTERT expression was significantly correlated with high Kadish stage. In conclusion, high hTERT expression can be considered a potential indicator of aggressive olfactory neuroblastoma.
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PMID:Expression of human telomerase reverse transcriptase and cyclin-D1 in olfactory neuroblastoma. 1722 47

Esthesioneuroblastoma (olfactory neuroblastoma) is an uncommon neuroectodermal tumor. Its biological activity ranges from indolent growth to local recurrence and rapid widespread metastasis. Treatment options consist of surgical resection followed by radiation therapy for primary lesions and the addition of chemotherapy for advanced, recurrent, or metastatic lesions. Patients often present with nasal obstruction, rhinorrhea, recurrent epistaxis, hyposmia, or anosmia. However, we report the highly unusual case of a patient with an esthesioneuroblastoma who presented with atypical symptoms of headaches, sinus congestion, and fatigue before acutely losing consciousness. Imaging showed a large frontal skull-based tumor associated with intratumoral hemorrhage. The findings prompted an emergent combined anterior craniofacial resection with gross total resection of the tumor. Except for anosmia, the patient recovered almost completely. Postoperatively, she received adjuvant intensity-modulated radiation therapy and chemotherapy. This is the first reported case of an esthesioneuroblastoma presenting with hemorrhage and rapidly declining mental status, an acute neurological manifestation of which clinicians should be aware.
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PMID:Esthesioneuroblastoma (olfactory neuroblastoma) with hemorrhage: an unusual presentation. 1726 90

The presence of small cell carcinoma within the submandibular gland is an uncommon clinical entity. However, other small round blue cell tumors are encountered in the head and neck with greater frequency. These include lymphoma, Ewing's sarcoma, melanoma, esthesioneuroblastoma, and neuroblastoma. A basic knowledge of the immunohistochemical studies available to distinguish each these tumors from one another significantly improves the frequency of accurate and timely initial diagnosis. We report a case of small cell carcinoma of the submandibular gland and review the other common small round blue cell tumors that occur within the head and neck. We utilize an acronym, LEMONS, to organize our review and facilitate improved retention of the differential diagnosis for small round blue cell tumors of the head and neck.
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PMID:Small cell carcinoma of the submandibular gland: a rare small round blue cell tumor. 1736 18

Olfactory neuroblastoma is a rare tumor of the nasal cavity. It is a locally aggressive tumor with local recurrence, and distant metastasis occurs in 22-40% of patients. We report a case of olfactory neuroblastoma with cauda equina metastases. A 49-year old male had undergone surgery twice previously; the first for olfactory neuroblastoma in October, 1990, and the second for its intracranial and orbital metastasis in September, 1999. He complained of lumbago in autumn, 2005 and MRI showed two enhanced lesions in the cauda equina. The mass was partially removed and histologically diagnosed as olfactory neuroblastoma metastasis. Whole-spine irradiation of 32 Gy and lumber-spine irradiation of 10 Gy were performed. The mass at L2/3 was disappeared after the irradiation although the mass at L4/5 was not changed in size. The patient was discharged without neurological deficit and is now kept under observation as an outpatient. Olfactory neuroblastoma with spinal metastasis is rare and only 11 cases have been reported in the literature. A very poor prognosis was observed in the patients of olfactory neuroblastoma with spinal metastasis. Olfactory neuroblastoma is a radiosensitive tumor, and radiotherapy for spinal metastasis was reported to be effective. However, effectiveness of chemotherapy was still uncertain. The patient with olfactory neuroblastoma should be observed carefully even though no local recurrence had been detected over 10 years. Radiotherapy and further treatment including chemotherapy should be considered in case of spinal metastasis.
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PMID:[Olfactory neuroblastoma with spinal metastasis: case report]. 1749 47

Olfactory neuroblastoma is a rare malignant tumor of the nasal cavity arising from the olfactory neuroepithelium. It usually presents as a polypoid mass high in the nasal vault including the cribriform plate, superior turbinate, and superior portion of nasal septum. An 89-year-old man visited our office with symptoms of occasional left nasal bleeding and obstruction. On the endoscopic examinations, polypoid mass with bleeding tendency was observed in the left inferior meatus. Biopsy was performed, and the diagnosis of olfactory neuroblastoma was confirmed by histopathologic examination and immunohistochemical staining. The tumor was resected via medial maxillectomy, and the final pathologic report established that it was primary olfactory neuroblastoma that occurred from the inferior meatus. In this report, a literature review was performed on the pathologic characteristics and prognosis of the tumor, and possible hypotheses that olfactory neuroblastoma has originated from the inferior meatus were discussed.
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PMID:Primary olfactory neuroblastoma originating from the inferior meatus of the nasal cavity. 1749 40

Olfactory neuroblastoma is an unusual neuroectodermal malignancy, which is thought to arise at the olfactory membrane of the sinonasal tract. Due to its rarity, little is understood regarding its molecular and cytogenetic abnormalities. The aim of the current study is to identify specific DNA copy number changes in olfactory neuroblastoma. Thirteen dissected tissue samples were analyzed using array comparative genomic hybridization. Our results show that gene copy number profiles of olfactory neuroblastoma samples are complex. The most frequent changes included gains at 7q11.22-q21.11, 9p13.3, 13q, 20p/q, and Xp/q, and losses at 2q31.1, 2q33.3, 2q37.1, 6q16.3, 6q21.33, 6q22.1, 22q11.23, 22q12.1, and Xp/q. Gains were more frequent than losses, and high-stage tumors showed more alterations than low-stage olfactory neuroblastoma. Frequent changes in high-stage tumors were gains at 13q14.2-q14.3, 13q31.1, and 20q11.21-q11.23, and loss of Xp21.1 (in 66% of cases). Gains at 5q35, 13q, and 20q, and losses at 2q31.1, 2q33.3, and 6q16-q22, were present in 50% of cases. The identified regions of gene copy number change have been implicated in a variety of tumors, especially carcinomas. In addition, our results indicate that gains in 20q and 13q may be important in the progression of this cancer, and that these regions possibly harbor genes with functional relevance in olfactory neuroblastoma.
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PMID:Array comparative genomic hybridization analysis of olfactory neuroblastoma. 1840 57

Olfactory neuroblastoma is extremely rare as a second neoplasm. We report an unusual case of olfactory neuroblastoma in a 59-year-old woman who had undergone two operations and received 54 Gy of irradiation for pituitary adenoma 20 years ago. At the time of admission, the patient presented with nasal obstruction and frequent epistaxis. Imaging studies showed a large mass in the nasal cavities and ethmoid sinus, extending to the intracranial area, with no evidence of any recurrence of the previous pituitary adenoma. The tumor was completely excised via a trans-cranial and trans-nasal approach. A diagnosis of olfactory neuroblastoma was established, and the patient was given postoperative chemotherapy. Although relatively uncommon, second neoplasms are an important consideration in the differential diagnosis of patients with new or recurring symptoms after treatment for pituitary adenoma. Furthermore, it is likely that radiation played a critical role in the development of olfactory neuroblastoma in our patient.
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PMID:Olfactory neuroblastoma following treatment for pituitary adenoma. 1867 81

Olfactory neuroblastoma is an uncommon neuroectodermal tumor of the sinonasal tract. It represents 2% to 3% of sinonasal neoplasms. Most olfactory neuroblastoma behave locally aggressive with 30% recurrence rates. A subset metastasizes to lymph nodes and/or distant sites. Grading of olfactory neuroblastoma involves a combination of factors with low-grade tumors having better survival than high-grade tumors. The grade does not always predict prognosis, however, as metastases can be seen in all grades of olfactory neuroblastoma. Trk-A, Trk-B, and p75NRT are neurotrophin receptors associated with numerous solid malignancies, particularly pediatric neuroblastoma. GRP78 is an endoplasmic reticulum protein, associated with differentiation of neuroblastic cells. Trk-A, p75NRT, and GRP78 overexpression are favorable prognostic factors in pediatric neuroblastoma, whereas Trk-B is associated with a poorer prognosis in these tumors. Olfactory neuroblastoma is clinically distinct from pediatric neuroblastoma but shares some histological features. Trk-A and p75NRT have been demonstrated in olfactory neuroblastoma previously. Trk-B and GRP78 have not been investigated in olfactory neuroblastoma. None of these markers have been correlated with grade or outcome in olfactory neuroblastoma. To investigate the role of Trk-A, Trk-B, p75NRT, and GRP78, a series of 20 olfactory neuroblastomas was stained with these antibodies. Trk-A and Trk-B stained most cases of olfactory neuroblastoma (90% and 85%). GRP78 stained most cases (90%), although weakly. P75NRT demonstrated focal membranous staining in a sustentacular pattern (60%). None of these markers correlated with Hyams grade. None of these markers definitively correlated with patient outcome. Neurotrophin receptors do not appear to have a prognostic role; however, Trk's may play an oncogenic role in olfactory neuroblastoma.
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PMID:Expression patterns of Trk-A, Trk-B, GRP78, and p75NRT in olfactory neuroblastoma. 1938 45


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