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 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An esthesioneuroblastoma, a rare malignancy of the olfactory nerve, was identified with bone imaging in a four year old male who presented with a history of headaches and proptosis of the right eye. Correlation with CT is provided.
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PMID:Esthesioneuroblastoma imaged with bone scintigraphy. 376 67

Thirty-nine patients with esthesioneuroblastoma are reviewed. The presentation of the tumor, symptomatology, investigation, and treatment are discussed. A recommended treatment regimen is outlined. Histologic typing is valueless in predicting tumor behavior. An illustrative and difficult case of recurrent base of skull esthesioneuroblastoma is presented. The resection performed is described, and the problem of extradural oropharyngeal communication is discussed. The solution was to use a temporalis and galeal frontalis flap. Reconstruction was with an external and intraoral prosthesis. Optimal treatment in a fresh lesion is radical surgery with or without radiation therapy. Esthesioneuroblastoma is a rare and often misdiagnosed malignant tumor of the olfactory epithelium. Originally described by Bergen et al. in 1924 as "esthesioneuroepithelioma olfactif," it was introduced into the North American literature by Schall and Lineback in 1951. Since then, fewer than 200 cases have been collected. The various terms used to describe it--olfactory esthesioneuroblastoma, esthesioneurocytoma, and olfactory neuroblastoma--all denote origin from the neural crest. The sensory nerves of smell are short bundles of fibers that originate in the olfactory bulb and pass through the cribriform plate to the olfactory area of the nasal mucosa. This mucosa is located in the most superior part of both nasal fossae. Thus the usual primary sites of occurrence include the superior nasal cavity or nasal septum, and turbinates, the ethmoid, or the cribriform plate, although an extranasal site of origin has been suggested. Symptoms are usually progressive and range from nasal obstruction or epistaxis to diplopia, ocular pain, and headaches in the more advanced disease state.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Esthesioneuroblastoma: treatment of skull-base recurrence. 402 92

Esthesioneuroblastomas (EN) exhibit problems in early diagnosis and therapy due to their localization at the frontal skull base. Analysis of six cases with EN (four male, two female; average age, 34.8 years) showed atypical initial symptoms, beginning as nasal bleeding, hyposmia and frontal headache. CT scans demonstrated hypo- to isodense tumors at the anterior skull base with extension to the sinuses and orbits. Five patients were operated on by an extranasal approach; one patient required orbital exenteration with later reconstructive surgery of the orbit by a microvascularly adapted forearm flap. One patient underwent a neurosurgical procedure first that was followed by chemotherapy and stereotactically guided radiation. One patient died 1 year after onset of therapy due to intracranial tumor. One patient developed lung metastasis 5 years after treatment. Four patients remain in clinical remission and receive regular follow-ups. Our analysis shows the guarded prognosis of EN despite multimodality therapy. This includes the problems of advanced disease with complications of surgery and radiation. All therapeutic procedures should be planned in collaboration with otolaryngologists, neurosurgeons and radiotherapists. New computer-aided and stereotactically guided radiation procedures can be helpful, especially in patients with extensive disease.
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PMID:[Diagnosis and therapy of olfactory neuroblastoma]. 792 33

A 51-year-old man presented with headache, vomiting and exophthalmus. Neurological examination revealed anosmia, papilledema, decrease in visual acuity, and disability in ocular movement. MRI showed a huge mass which occupied the whole nasal cavity and compressed the frontal lobe upwards and the eyes laterally. CT revealed an extensive bony destruction of the frontal base and bilateral orbits. The mass was biopsied transnasally, and was histologically diagnosed as olfactory neuroblastoma. It was highly radiosensitive and disappeared with a local irradiation of 40 Gy. Three months later the patient complained of a pain radiating from the neck to the right arm. MRI demonstrated a metastasis at the vertebral body of C5. Local irradiation of 30 Gy was performed. The metastatic lesion was removed, and a bone graft taken from the iliac bone was transplanted via an anterior cervical approach. Three weeks later, however, a hard mass appeared in the right of his neck and was surgically removed. By histological examination, it was also identified as a metastatic neuroblastoma to the cervical lymph node. A week after the removal of the cervical metastatic lesion, the metastasis extended rapidly to the left cervical and the bilateral hilar lymph nodes of the lungs. Chemotherapy was performed with a total doses of 800mg of cyclophosphamide, 1.5mg of vincristine, 40mg of pirarubicin, and 80mg of cisplatin. The lesions disappeared within 7 days. However, the patient died from disseminated intravascular coagulation 10 months after the onset. Olfactory neuroblastoma is usually an intranasal neoplasm, but it rarely extends intracranially and intraorbitally as is shown in our case. Basically, olfactory neuroblastoma is a relatively slow-growing tumor though it has a tendency to develop local recurrences over long periods even after aggressive primary treatment, and accompanied with distant metastases. However, our patient showed a very short survival time. Invasive extension and multiple metastases occurred during a short period, followed by disseminated intravascular coagulation. Combined chemotherapy at the initial treatment may be recommended in such an extensive case.
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PMID:[A case of olfactory neuroblastoma with intracranial, intraorbital extension and multiple metastases]. 902 94

Olfactory neuroblastoma is a rare tumor originating in the upper nasal cavity. It rarely extends intracranially. We report a clinical case of olfactory neuroblastoma with intracranial extension and distant metastasis. A 35-year-old man complained of nasal stuffiness and bleeding, headache and vomiting. Neurological examination showed anosmia and papilledema. MRI showed a huge mass that occupied the right nasal and paranasal cavities, and extended into the right frontal base. The tumor was removed totally and was histologically diagnosed as olfactory neuroblastoma. About two months after surgery, however, MRI demonstrated a rapid recurrence of the tumor in the nasal and paranasal cavities and the frontal lobe. Metastatic lesions were also seen in the right cervical lymph nodes. Chemotherapy was administered using cisplatin and etoposide. The tumor in the frontal lobe shrunk markedly but the other lesion persisted. Whole brain irradiation and local irradiation of the cervical lymph nodes were performed. The tumors became smaller but did not disappear. MRI demonstrated spinal dissemination. Irradiation of the whole spinal cord was performed. The metastatic lesions disappeared. The patient was discharged without neurological deficits, but died of pneumonia 15 months after surgery. Olfactory neuroblastoma is a slow-growing tumor and is highly radiosensitive, but it rarely extends or develops multiple distant metastases and seldom shows a short survival time, as in our case. A review of the literature documented responses in patients treated with a cisplatin-based drug combination. We recommend systemic control using cisplatin-based chemotherapy in addition to irradiation to prevent local recurrence in cases of advanced or metastatic olfactory neuroblastoma.
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PMID:[A case of olfactory neuroblastoma with intracranial extension and distant metastasis]. 1106 69

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

Esthesioneuroblastoma is a rare tumour arising from the olfactory epithelium of the nasal vault which frequently invades the cranial base and orbit. Esthesioneuroblastoma has a bimodal age distribution between 11 and 20 years and between 51 and 60 years. Esthesioneuroblastoma accounts for approximately 1-5% of intranasal cancers. The case is reported of a 79-year-old female patient with a Kadish stage C tumour with a one-year history of headache, nasal obstruction, anosmia, rhinorrhoea and epistaxis. Aim of this study is to analyse the natural history, treatment and prognosis of this tumour, based on a review of the literature.
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PMID:Esthesioneuroblastoma treated with external radiotherapy. Case report. 1893 12

Esthesioneuroblastoma is a rare malignant tumour that originates from the olfactory region. Its incidence is about 0.4 per million per year. The most common symptoms include epistaxis, headache, nasal obstruction, hyposmia and eye symptoms. Diagnosis of this rare tumour is often delayed. We present a case in which a 61-year-old male was referred due to proptosis and loss of vision. Magnetic resonance imaging showed a tumour in the nasal cavity involving the orbit, the frontal lope of the brain and lymph nodes in the neck. The case was staged according to Kadish as a stage C. Surgery was performed and postoperative radiotherapy planned.
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PMID:[Metastasizing esthesioneuroblastoma]. 2126 77

INTRODUCTION. Esthesioneuroblastoma (ENB) is a very uncommon malignant tumor with a neuroectodermal origin that usually involves the anterior cranial fossa and nasal cavity. OBJECTIVES. To review our experience in the management of ENB and assess the validity of the histopathological diagnosis, modality of treatment and prognostic factors of the disease comparing our findings with the literature. METHODS. A retrospective study of 11 cases with the diagnosis of esthesioneuroblastoma treated in our hospital between 2000 and 2008. Statistical analysis was performed in search for prognostic factors. The bibliography about ENB published between 1990 and 2009 was reviewed RESULTS. There were 3 women and 8 men, with a mean age of 42 years old (range 20-71y). Their symptoms upon admission were nasal obstruction (81%), epistaxis (27%), visual loss (18%), headache and others. According to the Kadish Stage, 2 were stage B and 9 were stage C. Dulguerov and Calcaterra Classification was also used: 2 were T2, 3 were T3 and 6 were T4. The hystopathological result according to the Hyams classification was: 2 cases in stage I, 4 in stage II, 3 in stage III and 2 in stage IV. The two cases classified in stage IV changed the diagnosis to undifferenciate tumor in the second biopsy. A subcranial approach was performed in 8 cases combined with endonasal endoscopy to confirm the total removal, followed by radiotherapy in all and chemotherapy in one case, resulting on 62% (5 patients) being alive without disease, 12,5% (1 p) alive with disease, and 25% (2 p) dead of disease. Another patient was operated by a single endonasal endoscopic approach and a subtotal removal was achieved. This patient is alive without disease. The other 2 patients were treated by biopsy plus radiotherapy and chemotherapy, because they were considered unresectable, and one of them is alive with disease and the other one is dead of disease. Radiotherapy was performed in all cases and chemotherapy in 5 cases. The hystopathological grading system of Hyams was considered statistically significant as a prognostic factor of disease-free survival. CONCLUSIONS. When the hystopathological diagnosis of ENB is considered, the Hyams classification can be valid considering grade IV as an advanced stage that is sometimes difficult to differentiate from other undiferentiated tumors. The subcranial approach or craneofacial resection in advanced stages (Kadish C and some B) should be considered as the first treatment of choice. Radiotherapy is indicated in all cases and chemotherapy in selected cases. Hyams' classification was the only staging system that proved useful as a prognostic factor in our series.
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PMID:[A retrospective study about 11 cases of Esthesioneuroblastomas treated in Hospital Santa Creu i Sant Pau between 2000 and 2008 and literature review]. 2203 Nov 59

Esthesioneuroblastoma (ENB) is an extremely rare malignant neoplasm of the nasal cavity. We report the case of a middle aged man who presented with a 6-months history of progressive nasal obstruction, watering of the eye, headache and occasional epistaxis. A diagnosis of esthesioneuroblastoma was established by histopathology and confirmed by immunohistochemistry. On staging the mass was classified as a kadish stage C tumor. The mass was excised via a craniofacial resection through a bi-coronal approach and the tumor mass was peeled away completely from the frontal lobe dura. The patient underwent postoperative radiation, and he was free of recurrence at follow-up 3 months later.
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PMID:Esthesioneuroblastoma: A case report and review of the literature. 2312 Mar 21


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