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)

Esthesioneuroblastoma is a rare tumor, which in many cases is diagnosed at an advanced stage with an high recurrence rate and incidence of metastases. Regionary metastases predict a poor prognosis. There is no standard therapy approach for these tumors. The most widly accepted primary therapy is radical craniofacial enbloc resection followed by radiation therapy. Today chemotherapy is getting more important and is administered with curative intention. Multidisciplinary management results in significantly longer survival in advanced tumor stages and recurrence. A clinical staging system as well as histopathological grading according of Hyams could be from importance for selection and timing of the different therapeutic modalities. We present a case of a 34-year-old female patient who was diagnosed with an advanced olfactory neuroblastoma of the upper nasal cavity with bilateral cervical lymph node metastasis (modified Kadish-stage D). Craniofacial resection and bilateral neck dissection was performed, followed by postoperative radiotherapy. Reviewing the recent literature the different therapeutic approaches are compared and discussed.
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PMID:[Metastatic esthesioneuroblastoma. Challenge in interdisciplinary combined modality therapy]. 1242 41

Olfactory neuroblastoma is a rare malignant tumour, usually diagnosed at advanced stages. We studied 3 patients who were treated at our Institute between 1991 and 1999. One patient presented with a stage A and 2 with a stage B tumour. One patient presented with coma due to inappropriate secretion of antidiuretic hormone associated with a stage B tumour. All 3 patients were treated with complete surgical resection via a lateral rhinotomy approach and postoperative radiotherapy. There was no involvement of the cribriform plate. One patient developed a metachronous regional metastasis and was treated with neck dissection and radiotherapy. All 3 patients are free from recurrence with a follow-up period of 9 years, 18 months and 1 year, respectively. Combination therapy is the cornerstone of treatment for olfactory neuroblastoma. Complete surgical resection is the most important prognostic factor and can be accomplished via lateral rhinotomy for early stage tumours.
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PMID:Olfactory neuroblastoma. A report of 3 cases. 1249 74

Esthesioneuroblastoma, also known as olfactory neuroblastoma, is a rare neuroendocrine tumor originating from the olfactory epithelium of the upper nasal cavity and representing < 3% of all tumors of the nasal cavities. This malignant tumor is characterized by a slow growth and local recurrencies and has a more favorable prognosis as compared with other more frequent forms of neuroblastomas originating from the suprarenal medulla and the sympathetic nervous system. Affected patients usually present with a history of progressive nasal obstruction, rhinorrhea, and severe epistaxis--sign of the conspicuous vascularity of this type of tumor. The combined use of CT and MR techniques allows the diagnostic suspicion of esthesioneuroblastoma and is of vital importance in the accurate staging of the disease and in the treatment planning.
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PMID:[Esthesioneuroblastoma. Description of a case investigated with CT and MR]. 1251 Apr 21

Neuroblastoma is the most common extracranial solid tumor of childhood. A wide variety of tumor locations and clinical presentations have been described. However, neuroblastoma is rarely located in the central nervous system, except in the case of esthesioneuroblastoma. We report a child with primary central nervous system (CNS) neuroblastoma who admitted to the hospital in coma. The tumor could be partially removed in our patient. After surgery, we treated the patient successfully with combined radiotherapy and eight courses of cisplatin-based chemotherapy. Our patient was followed-up for 34 months without any evidence of relapse.
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PMID:Primary cerebral neuroblastoma: a case treated with adjuvant chemotherapy and radiotherapy. 1521 53

Maffucci's syndrome consists of multiple cutaneous hemangiomas, dyschondroplasia, and enchondromas with potential for malignant change. We report a case of a 33-year-old man with Maffucci's syndrome who presented with a several month history of nasal congestion, facial pain, and diminished vision in his left eye. Radiological studies showed a large soft tissue mass centered in the sinonasal area, extending bilaterally into maxillary sinuses and orbits with compression of left optic nerve. Biopsy of the mass showed esthesioneuroblastoma (olfactory neuroblastoma). Chemotherapy resulted in initial improvement, but the tumor recurred and did not respond to further treatment, resulting in his death. Sarcomatous tumors are reported in Maffucci's syndrome, but this is a rare case of a neuroendocrine tumor in a patient with Maffucci's syndrome.
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PMID:Esthesioneuroblastoma in Maffucci's syndrome. 1522 Dec 18

Esthesioneuroblastoma (olfactory neuroblastoma) is a rare, malignant neoplasm that typically arises in the nasal vault, invades adjacent tissues, and causes locoregional (cervical lymph nodes) and distant metastases. Only two cases of tumors arising in the sellar region that had the histological characteristics of esthesioneuroblastoma have been reported in the literature to date. The authors present the case of a 35-year-old woman with secondary amenorrhea and a rapidly growing tumor located in the adenohypophysis. After total removal of the lesion through a transseptal-transsphenoidal approach, the histological examination revealed an esthesioneuroblastoma Grade II/III according to Hyams. Considering the particular location of the lesion and the absence of residual tumor on postoperative magnetic resonance imaging, no adjuvant therapy was performed. The patient remained free from tumor recurrence 2 years postoperatively. Because all published cases of this esthestoneuroblastoma have been large neuroblastic tumors of the pituitary gland arising in middle-aged women, pituitary neuroblastoma might represent a rare, specific clinicopathological entity.
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PMID:Esthesioneuroblastoma of the pituitary gland: a clinicopathological entity? Case report and review of the literature. 1559 68

Sinonasal undifferentiated carcinoma (SNUC) is a rare tumor of the nasal cavity and paranasal sinuses first described in 1986. Olfactory neuroblastoma and SNUC may appear quite similar on histological examination. Due to the fact that olfactory neuroblastoma has a much better prognosis, a distinction with SNUC has to be drawn. We report a case of SNUC and describe the role of immunohistochemistry in making an accurate histological diagnosis. In addition, potential factors influencing the development of SNUC described in the literature and current treatment modalities are discussed. Despite aggressive and multimodal treatment regimens, the outcomes of patients suffering from SNUC have remained dismal. A randomized controlled clinical study could be the basis for determining the optimal treatment for SNUC.
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PMID:[Sinonasal undifferentiated carcinoma. A rare and aggressive neoplasm of the nasal cavity and paranasal sinuses]. 1603 34

Olfactory neuroblastoma or esthesioneuroblastoma is a rare embrionary tumor, much more in old patients, which uses to present as a pollipoid mass with nasal obstruction, epistaxis and anosmia of long evolution. We report the case of a 82-year-old male with such symptoms diagnosed by biopsy as neuroblastoma class III according the histologic Hyams grading. Due to the age of the patient and grade of the tumor we did not perform surgery and, although a chemotherapy-radiotherapy treatment was suggested, the patient was not agree with it and he has not been attended or followed in our consulting rooms.
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PMID:[Olfactory neuroblastoma in an old patient. Report of a case]. 1631 85

Sinonasal disease is one of the most common clinical head and neck pathologies. The majority of sinonasal pathology is inflammatory with neoplasms comprising approximately 3% of all head and neck tumours. Although sinus tumours are rare, they portend a poor prognosis, often due to advanced disease at diagnosis. Like most neoplasms, early detection improves prognosis, therefore clinicians and radiologists should be aware of features separating tumours from inflammatory sinus disease. This article reviews the anatomy, clinical features, imaging findings, treatment and histopathology of selected sinonasal tumours. Benign neoplasms reviewed include osteoma, inverting papilloma, and juvenile nasal angiofibroma. Malignant neoplasms reviewed include squamous cell carcinoma, the minor salivary gland tumour, adenoid cystic carcinoma, adenocarcinoma, melanoma, lymphoma, and olfactory neuroblastoma (esthesioneuroblastoma).
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PMID:Imaging of lumps and bumps in the nose: a review of sinonasal tumours. 1636 Nov 46

Olfactory neuroblastoma is an uncommon intranasal tumor originating from olfactory neuroepithelium. Despite the development of electron microscopy and immunohistochemical testing, the pathological diagnosis of this tumor is still difficult because of the wide range of histological features. Magnetic resonance imaging (MR) of this tumor and the pattern of contrast enhancement have not been well described. The purpose of this report was to analyze the MR characteristics of olfactory neuroblastomas. The MR signal, pattern of contrast enhancement, and correlation with high-resolution computed tomography (CT) imaging were examined. Seventeen patients with olfactory neuroblastoma were treated at Hokkaido University Hospital and a related hospital during the past 25 years. MR images taken in 12 patients and CT images taken in 9 patients with histologically confirmed olfactory neuroblastoma were retrospectively reviewed. Compared with brain gray matter, 11 tumors were hypointense on T1-weighted images, 9 homogeneously and 2 heterogenously. Eight tumors were hyperintense on T2-weighted images, 3 homogeneously and 5 heterogeneously, although their appearance was less intense than that of sinusitis. Gadolinium enhancement was moderate in one case and marked in 10 of the 11 cases, 9 homogeneously and 2 heterogeneously. Nine of the 11 tumors showed smooth regular shaped margins; 2 of these tumors exhibited irregular infiltrating margins on gadolinium-enhanced images, compared to the pre-contrast T1-weighted images. Eight of the 11 tumors had clearly demarcated margins, while 3 of the 11 tumors did not exhibit gadolinium enhancement. Six of the 12 cases (50%) exhibited intracranial cysts on the gadolinium-enhanced images. T2-weighted or gadolinium-enhanced images successfully distinguished sinusitis from tumors in 4 cases whereas the CT images failed. Gadolinium enhancement, particularly in the tangential plane, demonstrated intracranial extension not apparent on the CT images in one case. In most cases, olfactory neuroblastomas are hypointense on T1-weighted images, hyperintense on T2-weighted images, and show marked homogeneous enhancement with well-demarcated regular margins upon gadolinium enhancement. Although the definite diagnosis is based on histopathology findings and MR features are nonspecific, they may suggest an imaging diagnosis of olfactory neuroblastoma when seen in the superior nasal cavity. MR is superior to CT both in delineating the extent of the tumor and in making an imaging diagnosis.
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PMID:[Magnetic resonance imaging of olfactory neuroblastoma]. 1661 28


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