Article

Recurrent olfactory neuroblastoma treated by radiation, surgery, and chemotherapy: “A case report”

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Abstract

Olfactory neuroblastoma is a rare tumor. The role of CT Scan, morphological details and the electron microscopic findings have been highlighted in the diagnosis of the lesion. The present patient, in which the tumor had recurred following surgical excision, was treated successfully with a combination of radiation, further surgery, and maintenance chemotherapy. We also feel that such a planned multidisciplinary approach is justified as the initial treatment for all advanced lesions in view of high recurrence rates reported following use of radiotherapy or surgery alone.

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... Chemotherapy has been suggested as palliative therapy and may have a beneficial effect on olfactory neuroblastoma in approximately 62% of patients when combined with other treat- ment modalities. 18 Combination chemotherapy with cyclophosphamide (150 mg/m2 daily for 7 days), doxorubicin (35 mg/m 2 ) and vincristine (1 mg/m 2 ) has previously been administered with radiation therapy. 19 Four of five patients with Kadish stage C olfactory neuroblastoma either died (3) or still had residual tumour (1) after treatment with supralethal high dose chemotherapy followed by cryopreserved autologous bone marrow transplantation. ...
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The course of a patient with an olfactory neuroblastoma treated with conventional doses of chemotherapeutic agents and radiotherapy is presented. The patient survived 34 months after tumour necrosis with resultant pneumocephalus requiring surgical intervention.
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Olfactory neuroblastoma (esthesioneuroblastoma) is an extremely rare tumour arising from the olfactory epithelium of the nasal cavity close to the cribriform plate. Most institutions will have little experience in recognisi the clinical and histological features, or management of this tumour and reliance is placed on researching the literature when the individual patient presents. This study reviews seven patients with olfactory neuroblastoma treated at the Queensland Radium Institute from 1971 to January 1990. The overall local contrd rate in this series is 57% (four of seven patients) and 43% of patients (three of seven) remain alive. Conservative surgery and post-operative radiotherapy is recommended for early disease and more radical surgery with post-operative radiotherapy for advanced disease.
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There is no Class I evidence to guide the appropriate management of esthesioneuroblastoma (EN). Most data currently guiding treatment come from small- or modest-sized series gathered at individual centers that have concluded that surgery with radiotherapy is the preferred treatment. In this study, the authors summarize the published literature on treatment outcomes in patients with EN. The objective was to ascertain what variables predict prognosis in these patients and to determine the relative effect of different therapies. The authors identified 205 published studies containing treatment outcomes for surgery, radiotherapy, chemotherapy, or multimodal treatment. Using Kaplan-Meier analysis, the survival of patients who received surgery was compared with that in those who received surgery and radiotherapy. Additionally, Kadish staging was compared with low- and high-grade Hyams criteria to assess for subgroup prognostic significance in survival differences. Nine hundred fifty-six patients met the inclusion criteria, with a median follow-up time of 3 years. Kaplan-Meier analysis demonstrated no difference in survival between patients who underwent surgery alone and those who underwent surgery plus radiotherapy at 5 years (78 vs 75%) or 10 years (67 vs 61%, respectively) (p = 0.3). Univariate analysis demonstrated worse survival in cases involving Kadish Grade C tumors, Hyams Grade 3 and 4 tumors, and in patients older than 65 years of age. Multivariate analysis demonstrated that Hyams Grade 3 and 4 lesions carried significant risk (proportional hazard = 4.83, p < 0.001) with 5- and 10-year survival of 47 and 31%. A biopsy should always be obtained in cases suspected of EN because histology is a strong prognostic indicator and will help guide appropriate treatment. Unimodal surgery and combined surgery/radiotherapy appear to be of equivalent efficacy with respect to survival in patients with EN. Chemotherapy should be considered in high-grade EN.
Article
26 Patienten mit histologisch gesicherten Ästhesioneuroblastomen wurden hinsichtlich Therapiemodalitäten, Überlebenszeit und Rezidiv- und Metastasierungsrate untersucht. Die mediane Nachbeobachtungszeit betrug 64 Monate mit einer Spanne von 2 bis 214 Monaten. Das mittlere Alter der Patienten bei Diagnosestellung betrug 53 Jahre mit einer Spanne von 24 bis 73 Jahren. Die Geschlechterverteilung war mit 13 Männern zu 13 Frauen ausgewogen. Drei Patienten zeigten zu Therapiebeginn eine Tumorausdehnung entsprechend Stadium Kadish A, 7 entsprechend Kadish B und 16 entsprechend Kadish C. 20 Patienten wurden primär chirurgisch versorgt, 5 Patienten nach Diagnose einer primären Radiotherapie zugeführt. Bei 17 Patienten erfolgte eine postoperative Bestrahlung. Ein Patient verstarb vor Einleitung einer definitiven Primärtherapie infolge Tumorprogress nach 2 Monaten. Eine Chemotherapie wurde primär adjuvant oder neoadjuvant bei 5 respektive einem Patienten durchgeführt. Ergebnisse: Die mediane Überlebenszeit aller Patienten lag bei 64 Monaten, im Stadium A bei 150,5 Monaten, im Stadium B bei 59 Monaten und im Stadium C bei 40 Monaten. 7 Patienten (27%) entwickelten Lokalrezidive, 4 weitere (15%) im Verlauf zervikale Lymphknotenmetastasen. Bei 3 Patienten (11,5%) bestanden zum Diagnosezeitpunkt Lymphknotenmetastasen. In 6 Fällen (23,1%) kam es zu einer Fernmetastasierung. Die 5-Jahres-Überlebensrate aller Patienten lag bei 48%, davon im Stadium A bei 100%, im Stadium B bei 71% und im Stadium C bei 25%. Die 10-Jahres-Überlebensrate betrug für alle Patienten 15%, stadienspezifisch entsprechend 33%, 33% und 15 %. Multimodal behandelte Patienten schnitten bezüglich Überlebenszeit und Rezidivrate am besten ab. Schlußfolgerungen: Bei multimodaler Therapie sind in Übereinstimmung mit der Literatur Langzeitüberlebensraten bis zu 214 Monaten möglich. Auch Rezidive sind erfolgreich therapierbar. Entscheidendes Prognosekriterium ist die Tumorausdehnung zu Therapiebeginn
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Olfactory neuroblastoma (esthesioneuroblastoma) is an extremely rare tumour arising from the olfactory epithelium of the nasal cavity close to the cribriform plate. Most institutions will have little experience in recognising the clinical and histological features, or management of this tumour and reliance is placed on researching the literature when the individual patient presents. This study reviews seven patients with olfactory neuroblastoma treated at the Queensland Radium Institute from 1971 to January 1990. The overall local control rate in this series is 57% (four of seven patients) and 43% of patients (three of seven) remain alive. Conservative surgery and post-operative radiotherapy is recommended for early disease and more radical surgery with post-operative radiotherapy for advanced disease.
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A retrospective review of the Memorial Sloan Kettering Cancer Center experience (MSKCC) with esthesioneuroblastoma was performed. From 1975 to 1985 14 cases were identified. Overall 5- and 10-year survival was 86% and 70%, respectively. Four local failures were observed and 4 neck failures were observed. A review of the literature since 1966 revealed an unexpectedly high incidence of neck failure, and of the 21 of 110 patients with neck failures that were identified through the literature, there were 6 subsequent deaths.
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Article
Full-text available
Esthesioneuroblastoma (ENB) arises from the neuroepithelium in the olfactory rim of the nasal cavity. It accounts for about 3% of all intranasal tumours. Reviews since the first description by Berger and Luc in 1924 never reported more than a hundred cases, stressing the rarity of the tumour. However, a thorough literature review revealed a total of 945 reported cases. In our search we found a total of 1,457 cases chronicled in the literature of which perhaps 487 were cited in more than one paper, bringing the total of reported cases to 945. Author cases accounted for 198 and therefore collaborative efforts accounted for 747 cases. Sex distribution was 53.36% male and 46.64% female. Kadish classification was applied to 553 cases revealing 103 (18.29%) class A cases, 182 (32.33%) class B and 278 (49.38%) class C cases. This distribution was generally stable through the decades. Treatment could be classified in 898 cases. It consisted of surgery alone in 25.17% (226 cases), radiotherapy alone in 18.37% (165 cases), combined surgery and radiotherapy in 43.21% (388 cases) and chemotherapy in 13.2% (119 cases), followed in 11 cases (1.22%) by bone marrow transplant. In the reported cases an overall follow up could be evaluated in 477 cases, while in only 234 cases a five-year follow up was done. The outcome was 68.38% alive and disease free, 12.82% alive with disease and 18.80% dead. From these 20.51% had surgery only, 11.11% radiotherapy and 68.38% combined surgery and radiotherapy. The best survival rates were obtained by combined therapy (72.5% vs. 62.5% surgery alone and 53.85% radiotherapy alone). Death rates were highest after radiotherapy alone (30.77% versus 18.75% in combined therapy and 12.50% after surgery alone). In conclusion, ENB is a rare but not exceptional tumour. It is best treated with combined surgery and radiotherapy. Unfortunately early diagnosis is still uncommon and no significant changes to the proportions of Kadish classes at first diagnosis have been noted in recent decades. A greater awareness of the tumour and earlier diagnosis seems the major focus for future research.
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Five cases of histologically confirmed olfactory neuroblastoma (esthesioneuroblastomas) have been studied by computed tomography (CT). Both the clinical symptoms (in particular, unilateral nasal obstruction and recurring epistaxis) and radiological findings (opacity of the paranasal sinuses) are nonspecific. Computed tomography shows a contrast enhancing masslesion and is more useful than conventional tomography in estimating its extension and the associated bone destruction, secondary reaction of sinusitis, and tumoral calcification. In the two cases in which the tumor was associated with exophthalmos, CT demonstrated that the muscular cone acts as a barrier preventing tumoral invasion. In cases with intracranial involvement, it appears that the attenuation values can be useful for differentiating between invasion by contiguity (two cases) versus metastasis (one case). Computed tomography is valuable in the follow-up and during and after radiotherapy of the tumor.
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Esthesioneuroblastoma is an uncommon tumor of neural crest origin arising in the nasal cavity. Since 1966, 97 cases have been reported in the world literature. In this report an analysis is presented of the following aspects of these 97 patients: age and sex distribution, disease staging, treatment results, interval to recurrence, and survival. Staging was according to a system proposed by Kadish et al. There is a bimodal age distribution with peaks in age groups 11-20 years and 51-60 years. Prognosis is favorable in early stage disease (A and B) with 3-year crude survivals of 88.9% and 83.3%, respectively. Stage C patients have a poor prognosis, with 52.9% surviving 3 years. Treatment consisted of radiotherapy, surgery, or a combination of radiotherapy and surgery. In stage A, prognosis is favorable in all three treatment groups with one 1 of 24 patients dying of recurrent disease. In stage B, recurrence rates and 3-year survivals are also similar in the three treatment groups. Thus, single modality treatment is as effective as combined treatment for early stage disease. Local recurrence is the predominant site of failure occurring in 68% of recurrences. The overall crude survival for the 97 patients was 95.4%, while the determinate survival (corrected for intercurrent disease) was 70.8%.
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Olfactory neuroblastoma is a malignant neoplasm with a varied biological behavior. Its clinical course is unpredictable and there is no correlation between its microscopic features and biological behavior. The present study deals with light and ultrastructural characteristics of two cases of olfactory neuroblastoma of the nasal cavity. In one patient, the definitive diagnosis was established on the basis of ultrastructural features of the lesion. The most consistent fine structural findings were the presence of intracytoplasmic densecored neurosecretory granules, "true" and "pseudo-" rosettes, and the neuritic processes emanating from the tumor cells. On the basis of their biochemical, histochemical, and ultrastructural characteristics, olfactory neuroblastomas are similar to neuroblastomas arising from the adrenals or sympathetic nervous system. These findings, therefore, support the hypothesis that olfactory neuroblastomas are most likely of neural crest origin and thus belong to a group of neoplasms collectively known as "apudomas" or neurocristomas. The literature review strongly favors combined surgery and postradiation as the most effective treatment of olfactory neuroblastoma.
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Esthesioneuroblastoma is a nasal tumor which arises from cells of neural crest origin. It is a difficult tumor to diagnose clinically and histopathologically. First described in 1924, approximately 160 cases have been reported with over 125 of these in the last 15 years. This reflects an increased awareness of the tumor by physicians rather than an icreased incidence. In the past 17 years, 12 cases of esthesioneuroblastoma have been treated at the Department of Otolaryngology and Maxillofacial Surgery of the University of Virginia Medical Center. Reviewing these cases and the literature leads us to make the following recommendations for diagnosis and treatment: The diagnosis of esthesioneuroblastoma can be made by 1) the clinician who suspects it in any patient with a nasal mass causing unilateral obstruction; 2) the finding of plexiform intercellular fibrils by light microscopy (rosettes and pseudorosettes are not as common as reported); 3) the finding of secretory granules and neurites by electron microscopy of the highly undifferentiated tumors; and 4) formaldehyde-fume-induced fluorescence. Combined therapy with preoperative irradiation followed by craniofacial resection of the tumor to include the cribriform plate is recommended. This treatment should result in a five-year survival in excess of 50% of patients.
Article
Five cases of histologically confirmed olfactory neuroblastoma (esthesioneuroblastomas) have been studied by computed tomography (CT). Both the clinical symptoms (in particular, unilateral nasal obstruction and recurring epistaxis) and radiological findings (opacity of the paranasal sinuses) are nonspecific. Computed tomography shows a contrast enhancing mass-lesion and is more useful than conventional tomography in estimating its extension and the associated bone destruction, secondary reaction of sinusitis, and tumoral calcification. In the two cases in which the tumor was associated with exophthalmos, CT demonstrated that the muscular cone acts as a barrier preventing tumoral invasion. In cases wtih intracranial involvement, it appears that the attenuation values can be useful for differentiating between invasion by contiguity (two cases) versus metastasis (one case). Computed tomography is valuable in the follow-up and during and after radiotherapy of the tumor.
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Seventeen patients with olfactory neuroblastoma seen at the Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary from 1941 to 1971 are presented. Data on age, sex, symptoms, physical, and roentgenographic findings, and therapeutic results are evaluated. A system of pretherapy staging is proposed in which for Group A, the tumor is limited to the nasal cavity; in group B, the tumor is localized to the nasal acvity and paranasal sinuses; and in group C, the tumor extends beyond the nasal cavity and paranasal sinuses. Olfactory neuroblastoma is a radioresponsive, and, to a limited extent, radiocurable tumor and varies in aggressiveness. Of 17 patients in this series, 13 or 76% were alive without disease following treatment by surgery, irradiation, and combination of these two methods. Uncontrolled primary lesions with or without metastases accounted for all therapeutic failures. A treatment policy for this disease is presented.
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Olfactory esthesioneuromas are rare tumors of the nasal fossae. They originate from the epithelium and nerves of the olfactory mucosa. From the histogenesis and morphology, two types can be distinguished: the olfactory esthesioneuroepithelioma and the olfactory esthesioneurocytoma. A third type, the olfactory esthesioneuroblastoma, is still insufficiently characterized. Errors in the microscopical diagnosis occur frequently, particularly in regard to the olfactory esthesioneuro-cytomas, which are often taken for lymphosarcomas or anaplastic carcinomas, and the olfactory esthesioneuroblastomas, which are sometimes taken for neuroblastomas. The microscopical classification of the olfactory esthesioneuromas is important, because a prognosis from the histological features is possible up to a point. The olfactory esthesioneuroepithelioma appears most liable to give rise to local recurrence and metastasis.
Article
SINCE the first description of olfactory neuroepithelioma by Berger et al1 in 1924, there have been 102 subsequent cases reported in the world literature.That the tumor is not as uncommon as once believed is evidenced by more frequent recent reports of it. The main problem seems to have been one of recognition, and the literature is replete with instances of mistaken diagnoses. The olfactory neuroepithelioma is a notorious masquerader and has been mistaken for transitional cell carcinoma, malignant melanoma, plasmacytoma, myxosarcoma, reticulum cell sarcoma, and lymphosarcoma.By definition, the esthesioneuroepithelioma olfactif, according to Berger et al,1 is a malignant neoplasm of the olfactory apparatus with a histologic pattern similar to that of tumors of sympathetic ganglia, the adrenal medulla, and the retina.Previously unreported case histories of two patients and a comprehensive systematic review of the significant aspects of this lesion are presented in an endeavor
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Five patients with advanced stage or metastatic esthesioneuroblastoma treated with chemotherapy are reported, and another eight cases found in the literature are reviewed. In this collective experience with chemotherapy in this disease, the authors found that 8 of 13 patients (62%) had an objective response to chemotherapy. The agents which may be active in this disease and the role of chemotherapy, particularly adjuvant therapy for patients presenting with advanced disease, are discussed. Cancer 53:1036-1041, 1984.
Article
• The biologic behavior of olfactory neuroblastoma in 21 persons treated at the Mayo Clinic, Rochester, Minn, from 1960 to 1980 is reviewed. Follow-up data of five years or more are available for 19 cases. Findings showed the tendency of these tumors to spread submucosally and intracranially without obvious involvement of the anterior cranial fossa dura. The aggressive nature of this lesion has not been appreciated. Metastatic disease occurred in 62% and local recurrence in 57%. Long-term follow-up showed that recurrent or metastatic disease could occur up to ten years after initial diagnosis. The five-year survival rate was 58%. Only four patients who have been follow-up for more than five years were without local recurrence or metastasis. This review should prompt a change in the current attitudes toward treatment of this tumor. (Arch Otolaryngol 1983;109:797-802)
Article
Olfactory neuroblastoma is an uncommon malignant tumor arising from the nasal cavity. During the past 18 years from 1964 to 1982, only 24 cases have been in Japanese literature, including this case. The average age of these patients is 42.3 years, ranging from 4 to 75 years old. The sex ratio of male to female is 10:14, female being prevalent moderately. The peak distribution is from 30 to 40 years old. The majority of the patients died of distant metastasis within 2 years of diagnosis. Therefore, the overall prognosis in Japan is very poor, as compared with about a 50% 5 year survival rate in the western countries. This is probably because this tumor is difficult to diagnose correctly, and the chance to start treatment early is apt to be missed here, though some tumors behaved very aggressively from the beginning, metastasizing early.
Article
Estesioneuroblastomas are rare neoplasms that have sometimes been classified as tumors of amineprecursor uptake and decarboxylation (APUD) or neural-crest origin. To evaluate the validity of this histogenetic classification of esthesioneuroblastomas, we examined the intermediate filaments of one of these tumors with ultrastructural and immunohistochemical techniques. We found that these tumor cells contained both intermediate filaments and immunoreactive neurofilament proteins. On the other hand, immunoreactive vimentin and glial filaments (the other two classes of intermediate filaments found in the brain) were not found in these tumor cells. These observations provide strong evidence for the neuronal origin of esthesioneuroblastomas and suggest that antibodies against specific classes of intermediate filaments are useful for the diagnostic and and histogenetic evaluation of human neoplasms.
Article
The diagnosis and clinical management of olfactory neuroblastoma are discussed. Computerized tomography is a valuable means of assessing the pre-operative extent of disease and the efficacy of subsequent major surgery. The tissue diagnosis is facilitated by electron microscopy, demonstrating the presence of dense core vesicles and cytoplasmic filaments. Radiotherapy followed by craniofacial resection is the treatment of choice for olfactory neuroblastomas that are locally extensive at presentation.
Article
A retrospective review of the clinical records of 31 patients with esthesioneuroblastoma is presented. A bimodal age distribution was noted. The tumor is extremely rare among blacks. Most patients presented with locally advanced disease. However, regional and distant metastases at the time of initial diagnosis are uncommon. Local recurrence at the primary site was very common, and this reflects either the conservative initial surgical treatment employed or the multicentric nature of the tumor. Cervical lymph node metastasis is present in less than 10 percent of patients at the time of diagnosis. Survival rates were better in patients with early stages of disease. From this study we conclude that the current management of these tumors should consist of a combination of radiation and surgery. Radical resection followed by postoperative radiotherapy appears to be the treatment combination of choice. The 5 year survival rate in our series was 52 percent.
Article
Olfactory neuroblastoma is an uncommon malignant neoplasm whose clinical course varies and does not correlate with histologic features. A case is presented that was treated with radiotherapy and chemotherapy, since neither radical surgery nor radiation alone has successfully controlled such tumors. These lesions have a high incidence of local recurrence and are highly aggressive.
Article
Nine patients with olfactory neuroblastoma were treated at the Radiation Therapy Service of the University of Michigan Medical Center (UMMC); their case histories are presented. There was a slight female predominance and the peak age distribution was between 60 to 69 years (4 patients). One patient developed sudden visual loss in one eye and partial loss in the other resulting from a hematoma at the optic chiasmal region. We have ascribed it to radiation damage. Our results show that this tumor is radiocontrollable; control at primary site occurs in 66.6% of patients. We recommend postoperative irradiation in all tumors that are extensive initially.
Article
The cancericidal effect of irradiation on neuroblastoma is demonstrated in a case report.
Olfactory neuroepithelioma
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