Esthesioneuroblastoma: a Danish demographic study of 40 patients registered between 1978 and 2000.

OBJECTIVE: A retrospective review of all diagnosed cases of esthesioneuroblastoma registered in Denmark between 1978 and 2000 was carried out in order to obtain epidemiological data and optimize national treatment guidelines. MATERIAL AND METHODS: Forty cases were verified histologically and included in the analysis Epidemiological and histopathological data were evaluated in relation to the clinical outcome. RESULTS: The 40 cases represent an incidence rate of 0.4 cases/million inhabitants per year. Eight (20%) patients were classified as Kadish stage A, 13 (32.5%) as stage B and 19 (47.5%) as stage C. The histopathological findings were classified according to the grading system of Hyams The median follow-up time was 2.3 years (range 0.3-11.1 years). The 5-year crude survival rate was 61%, with a median survival of 3.1 years (range 0.3-19.2 years). The 5-year disease-free survival rate was 50%, with a median survival of 1.7 years (range 0-19.2 years). Only 3 (7%) patients had positive cervical lymph nodes at presentation. A nationwide consensus regarding treatment was seen in patients classified as Kadish stages A and B. The longest duration before the first recurrence of esthesioneuroblastoma was 5(1/2) years. CONCLUSIONS: The following therapeutic guidelines are suggested: Kadish stage A patients, surgical tumour resection and radiotherapy; Kadish stage B, surgical tumour resection and radiotherapy; Kadish stage C, surgical tumour resection via a craniofacial resection and radiotherapy combined with chemotherapy. Long-term follow-up of esthesioneuroblastoma patients is mandatory.

Implication of 18F-fluoro-2-deoxy-D-glucose positron emission tomography on management of carcinoma of unknown primary in the head and neck: a Danish cohort study.

OBJECTIVES: To demonstrate the efficacy of whole-body 18F-fluoro-2-deoxy-D-glucose positron emission tomography (18F-FDG PET) in the detection of a carcinoma of unknown primary after conventional diagnostic workup in patients with a metastatic neck lesion and to demonstrate how the treatment policy of wide-field irradiation can be safely modified in relation to the findings from PET scanning. STUDY DESIGN: Prospective cohort study of consecutive patients. METHODS: Forty-two consecutive patients with squamous cell or undifferentiated metastatic disease in the neck from a carcinoma of unknown primary were enrolled after standard clinical workups. These patients underwent extensive clinical investigations including endoscopy under anesthesia with multiple mucosal biopsies and diagnostic imaging as well. If no primary site was indicated at this stage, a whole-body 18F-FDG PET scan was performed. RESULTS: Potential focal pathological uptake indicated a primary tumor in 20 of 42 cases (48%). After PET, this was confirmed by additional investigations in 10 patients (24%). Of these, seven primaries were found in the head and neck region (hypopharynx [three], base of tongue/vallecula [two], nasopharynx [1], floor of mouth [1]), and three primaries were found below the clavicles (lung [1], esophagus [1], and abdomen [1]. Positron emission tomography resulted in significant modifications of radiation treatment fields or fractionation prescriptions in all the patients who were diagnosed with a primary tumor after PET. CONCLUSION: With our present strategy of wide-field irradiation in patients with neck node metastases from a carcinoma of unknown primary, whole-body 18F-FDG PET had treatment-related implications in 24% (10 of 42) of the patients.

Esthesioneuroblastoma: a Danish clinicopathological study of 40 consecutive cases.

Aims. To review all cases of esthesioneuroblastoma in Denmark from 1978 to 2000 with respect to staging, grading, histopathological and immunohistochemical evaluation, and prognostication. Methods and results. Possible cases of esthesioneuroblastoma were retrieved from Danish oncology departments. Patients were included on the basis of review of their files or pathology reports, and/or on the basis of histopathological and immunohistochemical examination. Forty-nine possible cases were retrieved. Nine cases were excluded. Esthesioneuroblastoma is a malignant neuroendocrine tumour originating in the olfactory mucosa. It is a small blue cell neoplasm with a characteristic lobular architecture. It has a neuroendocrine immunophenotype and a sustentacular S-100 staining pattern. The tumours were staged according to Kadish and graded according to Hyams. Kaplan-Meier survival analysis was used to identify prognostic factors. Conclusion. The Kadish staging system was able to group the patients into prognostically relevant groups. Intracranial involvement and metastases at the time of diagnosis were found to be poor prognostic factors. Hyams grading system is difficult to work with and it was not possible to divide patients into prognostically relevant groups. Presence of necrosis, a diffuse growth pattern and a high proliferation index proved to be equally poor prognostic factors.