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Olfactory esthesioneuroma: a report of 40 cases.

Olfactory esthesioneuroma is a rare malignant tumor arising in the olfactory epithelium. Forty cases observed at the Institut Gustave-Roussy from 1956 to 1987 are reported. This tumor usually grows slowly and is usually local, but it is important to be aware of the possibility of lymph node involvement (17%) and, particularly, of rapid development of distant metastases (25%), usually within 6 months. CT scan, and more recently, NMR have proved to be of value in choosing the surgical approach. In view of the usual point of departure, a combined neurosurgical and transfacial approach seems to be a satisfactory approach for obtaining oncological control of the lesion. The role of chemotherapy is discussed. The main prognostic factors seem to be the size of the lesion, the intracranial extension, and the lymph node involvement.

Adult↗

Olfactory neuroblastoma: clinicopathologic and immunohistochemical characterization of four representative cases.

Olfactory neuroblastomas are rare tumors whose clinical prognosis is not predictable by assessment of initial stage or grade. The pathologic diagnosis is often difficult because of the wide range of the patient's age and histologies. In this report, we document that the diagnosis of olfactory neuroblastoma can be clarified by immunohistochemical demonstration of a unique antigenic profile that can be obtained in routinely processed biopsies. We describe four cases of olfactory neuroblastoma diagnosed and treated from 1979 to 1989, each confirmed by immunohistology. One of our patients was misdiagnosed twice at an outside institution, first as having nasopharyngeal carcinoma and then as having small-cell, undifferentiated "oat cell" carcinoma. Despite accurate tumor diagnosis and appropriate therapy, we found that there was no apparent correlation of clinical outcome with Kadish clinical stage or histologic grade of tumor.

Adult↗

Esthesioneuroblastoma: surgical treatment without radiation.

This study compares the treatment of two groups of patients diagnosed with esthesioneuroblastoma limited to the nasal cavity without evidence of cribriform plate erosion. Five patients were treated with radiation and extracranial excision, and eight patients were treated by craniofacial resection only. The incidences of local recurrence were 60% and 12%, respectively. The paper also presents a new staging classification for these tumors.

Adolescent↗

Esthesioneuroblastoma: the UCLA experience 1970-1990.

A retrospective review was conducted of all esthesioneuroblastoma cases treated at UCLA Medical Center from 1970 through 1990. Patients were staged according to the staging systems of Kadish, et al., Biller, et al., and a new staging system proposed by the authors. Of 26 patients treated, 74% were alive at 5 years and 60% were alive at 10 years. Combined treatment with surgery and radiation is advocated since a recurrence-free status was achieved in 92% of the patients, compared with 14% for surgery alone and 40% for radiation alone. A craniofacial resection was performed in 7 patients, all of whom have remained disease free. Negative prognostic factors included: age over 50 years at presentation, female sex, tumor recurrence, and metastasis. The proposed new staging system predicted disease-free status better than the other staging systems.

Adolescent↗

Olfactory neuroepithelioma in a dog: an immunohistochemical and electron microscopic study.

A case of olfactory neuroepithelioma was investigated electron microscopically and immunohistochemically. The tumor mass was found in the nasal cavities of a 10-year-old female dog, which showed epistaxis, nasal discharge and facial swelling. The tumor tissue consisted of tubular structure of cuboidal to columnar cells and compactly arranged nests of small cells surrounded by a fibrovascular stroma. Mitotic figures were frequently observed. Immunohistochemically, the tumor cells frequently showed positive for neurofilament protein, synaptophysin and/or carnosine in addition to keratin. Ultrastructurally, tight junction was observed between the tumor cells. No dense-cored secretory granules were shown in the tumor cells. These findings indicated that the present tumor had neuronal and epithelial features probably originating from the olfactory epithelium.

Animals↗

[Dysembryoplastic neuroepithelial tumor. An epidemiological study from a single institution].

The true incidence of dysembryoplastic neuroepithelial tumor (DNT) is unknown. Variable estimations have been advanced according to the type of recruitment of patients. In series of patients with tumors who were operated on for pharmacoresistant epilepsy, the rates varied widely, from 5% to 87%. Among 600 pediatric tumors in a series, 9 DNT (1.5%) were found. Since DNT was identified only in 1988, we reviewed our cases (1975-1991) of gangliogliomas (n = 25), oligoastrocytomas (n = 9), temporal oligodendrogliomas (n = 11), temporal astrocytomas grade II (OMS 1993) (n = 44) irrespective to age, as well as all astrocytomas grade II (n = 61) and oligodendrogliomas (n = 10) in patients up to 20 years of age. Seven DNT were encountered. Four cases had been formerly diagnosed as gangliogliomas and 3 as astrocytomas grade II. From 1992 on, 4 more DNT were diagnosed, making up a total of 11 cases. Eight patients were under age 17 (6 y to 17 y, mean 10.3 y) and 3 were 27, 42 and 51 year-old. Eight tumors were temporal, 1 frontal and 2 occipital. All patients have had pharmacoresistant epilepsy. Among all neuroepithelial tumors diagnosed in our Division in the last 22 years, DNT comprises 1.2% in patients under age 20 (n = 660), 0.24% in patients over 20 years (n = 1254), and 0.63% all ages considered (n = 1914).

Adolescent↗

Small round blue cell tumor of seminal vesicle in a young patient.

Seminal vesicle tumor is a rare disease with unclear origin. Generally, it is presented as a pelvic mass that can be detected by sonography and digital rectal exam. The authors report a 25-year-old patient with a pelvic mass which the magnetic resonance and surgical specimen reveal a seminal vesicle tumor. Immunohistochemical findings favored a primitive neuroectodermal tumor of the seminal vesicle. Herein, the treatment, histological and histochemical findings of this entity are discussed.

Adult↗

[Olfactory neuroblastoma with intracranial extension. Report of two cases].

Two cases of olfactory neuroblastoma with intracranial extension are described. The radiological features, including those of magnetic resonance (MR) imaging, are emphasized. On plain X-rays, the nasal cavity, ethmoid, sphenoid, and/or frontal sinuses were opacified. Erosion of the bony confines of the sinuses and orbit was seen in Case 1, but no bony destruction was observed in Case 2 despite intracranial tumor invasion via the seemingly intact cribriform plate. Angiography showed tumor staining in the ethmoid sinus, and in Case 1 tumor vessels were also found in the base of the frontal lobe. Computed tomography (CT) disclosed a large, soft, enhanced tissue mass occupying the nasal cavity and paranasal sinuses and extending into the anterior cranial fossa. In Case 1, MR images (0.5 Tesla) with a short spin echo sequence (repetition time [TR], 600 msec; echo time [TE], 26 msec) and a long spin echo sequence (TR, 2100; TE, 30, 60, 90) were obtained. T1-weighted images delineated the tumor accurately in relation to the normal architecture. T2-weighted images revealed widespread brain edema, with the tumor recognizable as a mass of low signal intensity within the high-intensity region of brain edema. The CT and MR imaging findings are by no means specific to olfactory neuroblastoma. However, both modalities, particularly MR imaging, are of special value in demonstrating the precise extent of the tumor in three dimensions.

Humans↗

[Olfactory neuroblastoma complicated by postirradiation pneumocephalus. Case report].

A 56-year-old male was admitted with the complaints of nasal bleeding, gait disturbance, and disturbance of consciousness. Neurological examination revealed drowsiness, right hemiparesis, and choked discs. Computed tomography scan showed an enhanced mass at the frontal base, which extended to the left nasal and paranasal cavities. Angiography showed a tumor stain with a mass sign. The intracranial part of the tumor was removed completely and he was discharged ambulatorily. Two months after surgery, however, he was admitted again for the regrowth of the tumor. Ventriculoperitoneal shunting was placed and radiation therapy was given to the brain and nasal cavity. After 3000 rad irradiation the clinical condition suddenly became worse because of pneumocephalus. The cranial tumor disappeared after irradiation but he died of metastases and general prostration. Clinically this case was diagnosed as an olfactory groove meningioma at first, but immunohistochemical diagnosis was olfactory neuroblastoma.

Brain Neoplasms↗

Radiation-induced bilateral cystic frontal lobe necroses demonstrating a fluid-blood level--case report.

A 41-year-old male developed radiation-induced bilateral cystic frontal lobe necroses after irradiation for an olfactory neuroblastoma. Computed tomography (CT) and magnetic resonance (MR) imaging revealed the lesions, one containing a fluid-blood level on CT scans and niveau formation on MR images. It was proved to be a coagulated hematoma within the cyst at surgery. Such a fluid-blood level in a radiation-induced cyst has never been reported, although hemorrhage frequently accompanies delayed radiation necrosis. Positron emission tomography with multiple tracers may be useful in differentiating cerebral radiation necrosis from tumor recurrence, because of absence of abnormal tracer accumulation.

Adult↗

Surgical treatment of intractable epilepsy originating from the primary sensory area of the hand--case report.

A 14-year-old right-handed girl presented with intractable epilepsy originating from the primary sensory area of the hand, manifesting as sensory partial seizures in the left hand with secondary generalization. Neurological examination showed no abnormal findings. Magnetic resonance imaging, magnetoencephalography, and cortical stimulation using chronic subdural electrodes demonstrated a lesion located in the primary sensory cortex of the hand, in which the ictal onset zone was identified by 24-hour intracranial electroencephalographic recording. Surgical resection of the lesion and multiple subpial transections of the adjacent cortices were performed. The histological diagnosis was dysembryoplastic neuroepithelial tumor (DNT). She was completely free of seizures without permanent sensory deficits postoperatively. DNT located in the primary sensory hand area may be resectable without causing postoperative sensory deficits, if accurate functional mapping and surgical resection are performed.

Adolescent↗

Radiographic features of olfactory neuroblastoma.

Olfactory neuroblastomas are malignant, slowly growing neurogenic tumors originating from the olfactory mucosa of the nasal cavity. Fourteen patients with this tumor were evaluated and showed variable extension into adjacent structures such as the ethmoid and sphenoid sinuses, orbit, and anterior cranial cavity.

Adolescent↗

Olfactory neuroblastoma.

A case of olfactory neuroblastoma, type 1 (olfactory neurocytoma), in a 44-year-old man is described. The tumour grew extensively in the right nasal cavity with involvement of the maxillary sinus and ethmoidal region, but was radically removed at operation. The prognosis and treatment are discussed in the light of earlier literature.

Adult↗

An ultrastructural study of intranuclear rodlets in a malignant extracranial neuroepithelial neoplasm.

This report describes for the first time the presence of intranuclear rodlets in a malignant extracranial neuroepithelial neoplasm. This malignant neoplasm appeared poorly differentiated by light microscopy and presented initially as a diagnostic problem. Electron microscopy revealed neoplastic cells joined by macula adherens-type junctions and containing numerous interdigitating cell processes diagnostic of a neuroepithelial neoplasm. A quantitative analysis of 500 cells revealed the presence of intranuclear rodlets in 5% of the neoplastic cells. The rodlets were composed of individual filaments that measured 7-9 nm in width. An extensive review of the literature revealed the high frequency of reported rodlets in normal neurons, normal paraneurons, neoplasms of the nervous system, and paraneuromas. The significance of this interesting structure to the physiology of the cell is discussed.

Aged↗