1/104. Olfactory esthesioneuroblastoma.Esthesioneuroblastomas are malignant tumours, usually of slow, invasive growth and low metastatic rate. Skeletal destruction must be assumed to be common, but is often demonstrable only by tomographic sections. Clinically these tumours do not differ from others of the same site, so that the diagnosis has to be based upon the histological appearances. In the light microscope the presence of neurofibrils is considered a specific differential diagnostic factor against other small-cell malignant tumours in this region. There seems to be no basis for a morphological classification into previously described sub-groups, neither according to histogenetic, light, nor ultra-microscopic findings. The general degree of differentiation and the number of mitoses appear to be the main factors of prognostic significance. Combined irradiation and surgical excision is considered the best treatment.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
2/104. Sinonasal teratocarcinosarcoma ("mixed olfactory neuroblastoma-craniopharyngioma") presenting with syndrome of inappropriate secretion of antidiuretic hormone.Sinonasal teratocarcinosarcoma (SNTC) is a rare, aggressive, histologically heterogeneous neoplasm of the paranasal sinuses and nasopharnyx of adults that is composed of variably benign or malignant neuroepithelial, epithelial, and mesenchymal elements. Occasional cases show intracranial extension and may be operated on by neurosurgeons and encountered by neuropathologists who may not be familiar with the entity. STNCs have not previously been associated with functional hypersecretory status. We report a 59-year-old male who presented with headache and syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and was subsequently found to have a bulky tumor of the frontal and ethmoid sinuses with focal dural invasion. The tumor was predominantly composed of olfactory neuroblastoma areas (90% of tumor) admixed with unusually well-developed craniopharyngioma-like mature squamous epithelium and ghost cells ( 10% of tumor). Scattered neuroblastoma tumor cells showed strong immunoreactivity with antibodies to arginine vasopressin, supporting ectopic hormone secretion by the tumor. While the coexistence of neuroectodermal and oral ectodermal-like differentiation in SNTCs is characteristic, in our case it was developed to an extreme functional and morphologic degree and was unassociated with other mesenchymal or epithelial elements often found in these complex tumors. SNTCs with limited differentiation have prompted controversy in classification.- - - - - - - - - - ranking = 1.2keywords = neuroblastoma (Clic here for more details about this article) |
3/104. High uptake of 123I-metaiodobenzylguanidine related to olfactory neuroblastoma revealed by single-photon emission CT.We studied a case of olfactory neuroblastoma by noting 123I-metaiodobenzylguanidine (MIBG) uptake revealed by single-photon emission CT (SPECT). MR imaging revealed an enhancing tumor extending from the left nasal cavity to the bilateral anterior frontal cranial fossae. SPECT revealed high 123I-MIBG uptake in the enhancing tumor. SPECT-revealed 123I-MIBG uptake appears to be clinically useful for distinguishing olfactory neuroblastomas from other tumors, especially suprasellar meningiomas.- - - - - - - - - - ranking = 1.2keywords = neuroblastoma (Clic here for more details about this article) |
4/104. 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.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
5/104. Olfactory neuroblastoma metastatic to the breast.Olfactory neuroblastoma (Esthesioneuroblastoma) is a rare malignant tumour arising from olfactory epithelium. It has a predilection for cervical lymph node metastasis and also has potential for distant metastasis to unusual sites like scalp, face, aorta, spleen, liver, adrenal gland and ovary. We report here a rare case of olfactory neuroblastoma in an adolescent girl with metastatic deposits in the breast. A poor prognosis due to rapidly progressive disseminated disease was observed. The relevant literature regarding metastatic olfactory neuroblastoma and metastasis in the breast from non-mammary malignant neoplasms is reviewed.- - - - - - - - - - ranking = 1.6keywords = neuroblastoma (Clic here for more details about this article) |
6/104. Olfactory neuroblastoma (esthesioneuroblastoma): report of six cases treated by a novel combination of endoscopic surgery and radiosurgery.Microsurgical techniques have considerably improved the results of surgical treatment for esthesioneuroblastoma (olfactory neuroblastoma). Nevertheless, these rare tumours of the frontal skull base are still associated with high rates of tumour recurrence and mortality, thus remaining a challenge even for experienced surgeons. A novel therapeutic approach that combines endoscopic sinus surgery and radiosurgery (gamma knife) is presented here. Six patients (3 males, 3 females) aged between 27 and 75 years (median 38 years) were treated between August 1993 and July 1999. Following paranasal and nasal endoscopic sinus surgery, marginal irradiation doses ranging from 16 to 34 Gy were applied radiosurgically involving up to 7 isocentres. At present, the median follow-up period is 57 months (range: 9 - 79 months). Without mortality, tumour control was achieved in all patients. One patient, who had to undergo additional craniotomy because of extensive neoplastic infiltration, developed postoperative liquorrhea. In another case the clinical course was complicated by a bilateral frontal sinusitis. All patients complained of nasal discharge and crusts. However, a preoperative Karnovsky Index ranging from 80 to 100 % remained stable in four patients whereas an improvement was observed in two patients. Based on the favourable results observed so far, the combination of endoscopic sinus surgery and radiosurgery can be considered as promising new option for the treatment of esthesioneuroblastoma that merits further investigation.- - - - - - - - - - ranking = 2.2keywords = neuroblastoma (Clic here for more details about this article) |
7/104. Endoscopic resection of esthesioneuroblastoma.Esthesioneuroblastoma (olfactory neuroblastoma) is a rare malignant neoplasm originating from neuroepithelial cells. Treatment for this tumor has traditionally required a craniofacial resection through an external approach. The safety and efficacy of transnasal endoscopic resection is uknown. A series of five patients with Kadish stage A or B lesions of the anterior skull base were resected and immediately reconstructed endoscopically over a nine-year period. There were 2 males and 3 females with a mean age of 64 years. Three patients were treated primarily and two were treated secondarily for recurrences after failing a traditional external craniofacial resection and postoperative radiotherapy. All primary lesions received adjuvant radiotherapy. The mean follow-up time was 31 months. All patients were discharged within 4 days or less. Operative complications included temporary postoperative infraorbital anesthesia (1 patient), unplanned cerebrospinal fluid leak (1 patient), and can orbital hematoma (1 patient). None of these complications resulted in any permanent sequelae. Two patients had prolonged nasal crusting for up to one year due to a variety of factors; large cavities, radiation therapy, and exposed lyophilized dural graft. However, all skull base defects healed without any short- or long-term sequelae. There have been no local recurrences. Two patients (1 primary and 1 secondary) developed regional metastasis to the orbit or cervical lymph nodes managed with primary transorbital excision or modified neck dissection, respectively. All but one patient remain free of disease by clinical, endoscopic, and radiographic (CT or MRI) surveillance. The remaining patient recurred distally as an undifferentiated carcinoma in his temporal bone and lungs 12 months after the initial resection. In experienced hands select cases of esthesioneuroblastoma can be safely excised and reconstructed endoscopically with comparable degrees of tissue removal as with external approaches. Short-term oncologic results in this mall series of patients appear to be comparable to traditional methods. Longer follow-up on a larger series of patients is warranted.- - - - - - - - - - ranking = 1.4keywords = neuroblastoma (Clic here for more details about this article) |
8/104. Olfactory neuroblastoma visualized by technetium-99m-ECD SPECT.We describe a case of olfactory neuroblastoma diagnosed by 99mTc-ECD SPECT. Although MRI and CT are very important for delineating these tumors, they are, by no means, specific for neuroblastomas. 131I-MIBG scintigraphy, the standard method for imaging tumors of neural crest origin, also failed to detect a histologically proven esthesioneuroblastoma.- - - - - - - - - - ranking = 1.4keywords = neuroblastoma (Clic here for more details about this article) |
9/104. Fine needle aspiration cytology of metastatic olfactory neuroblastoma: a case report.BACKGROUND: Olfactory neuroblastoma (ONB) is an uncommon tumor, presenting as a polypoid mass arising from the upper nasal cavity. This tumor has been seldom diagnosed by direct fine needle aspiration (FNA). CASE: Metastatic ONB was diagnosed by FNA. The patient was a 40-year-old female with a polypoid mass in the nasal cavity and ipsilateral cervical lymphadenopathy. The punch biopsy of the nasal tumor revealed a smudged small round cell neoplasm with neuroendocrine differentiation, consistent with ONB. In FNA smears from the cervical lymph node, there were well-preserved, small, monotonous cells with hyperchromatic nuclei, fibrillary cytoplasm and indistinct cell borders. Also noteworthy were occasional pseudorosettes as well as rare true rosettes. By immunocytochemistry, tumor cells were positive for cytokeratin, chromogranin and synaptophysin. CONCLUSION: ONB, like adrenal neuroblastoma, shows distinctive cytologic features, including a rosette or pseudorosette and fibrillary network. FNA can accurately demonstrate these characteristic findings, and in some cases it may be a better diagnostic modality than incisional biopsy.- - - - - - - - - - ranking = 1.2keywords = neuroblastoma (Clic here for more details about this article) |
10/104. Olfactory neuroblastoma with epithelial and endocrine differentiation transformed into ganglioneuroma after chemoradiotherapy.We report a 56-year-old man in whom an olfactory neuroblastoma with epithelial and endocrine differentiation transformed into a mature ganglioneuroma after chemoradiotherapy. The tumor arising from the sphenoidal and maxillary sinuses showed rapid growth into the frontal lobe and metastasis to the cervical lymph nodes. The patient showed signs of a syndrome of inappropriate secretion of antidiuretic hormone (SIADH). A radical craniofacial resection of the primary tumor was performed after 16 Gy of local irradiation and systemic chemotherapy. Three months after the operation, the patient died of mediastinal metastasis. The biopsy before chemoradiotherapy showed a neuroblastoma with Homer-Wright rosettes, fibrillary matrix, Flexner-Wintersteiner rosettes and antidiuretic hormone production. After chemoradiotherapy, the histology changed to that of a ganglioneuroma consisting of large ganglion cells and schwann cells without immature neuroblastoma components. Although transformation to ganglioneuroma in an adrenal neuroblastoma is common, an olfactory neuroblastoma showing ganglioneuronal maturation after chemoradiotherapy has not been reported. The pluripotent progenitor cells of the olfactory neurons may be the origin and their existence explains why various neoplasms with neuronal and epithelial differentiation arise from the olfactory mucosa.- - - - - - - - - - ranking = 1.8keywords = neuroblastoma (Clic here for more details about this article) |
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