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Tumours affecting the nose, paranasal sinuses and adjacent skull base are fortunately rare. However, they pose significant problems of management due their late presentation and juxtaposition to important anatomical structures such eye and brain. The increasing application of endonasal endoscopic techniques to their excision offers potentially similar scales of resection but with reduced morbidity. The present document is intended to be a state-of-the art review for any specialist with an interest in this area 1. to update their knowledge of neoplasia affecting the nose, paranasal sinuses and adjacent skull base; 2. to provide an evidence-based review of the diagnostic methods; 3. to provide an evidence-based review of endoscopic techniques in the context of other available treatments; 4. to propose algorithms for the management of the disease; 5. to propose guidance for outcome measurements for research and encourage prospective collection of data. The importance of a multidisciplinary approach, adherence to oncologic principles with intent to cure and need for long-term follow-up is emphasised.

Background: The aim of this retrospective study was to assess the potentials and limitations of endonasal micro-endoscopic sinus surgery in the management of sinonasal inverted papilloma (IP) and to demonstrate long-term results., Methods: Eighty-seven patients underwent resection of an IP either via an endonasal, an osteoplastic maxillary or frontal sinus or a combined approach. Charts were reviewed for presenting symptoms, tumour stage according to the Krouse classification, surgical management and follow-up status., Results: Most tumours were staged as T2 or T3 (42.5% each). Sixty-eight (78.2%) patients were referred for primary surgery. Nineteen (21.8%) patients presented with recurrent disease. The majority of IP (70%) were removed via an endonasal micro-endoscopic procedure. In 20 (23%) patients a combined approach was performed. The overall recurrence rate was 10.3%. Referring to endonasal surgery the incidence of recurrent IP was 10% in contrast to 15% after a combined procedure., Conclusion: Our data show that endonasal micro-endoscopic surgery offers an effective and safe treatment modality of IP with insignificant morbidity. Strict application of selection criteria, wide removal of the tumour origin along the subperiosteal plane as well as drilling the underlying bone and close follow-up of patients are mandatory for success.

Background: The goal of this study was to assess the potentials and limitations of endonasal micro-endoscopic surgery in the treatment of sinonasal inverted papilloma (IP) and to demonstrate long-term results., Patients and Methods: From 1989 to 2005, 64 patients underwent resection of IP via an endonasal approach using either the endoscope or microscope. Charts were reviewed retrospectively for presenting symptoms, radiological and intraoperative data. All patients were followed by endoscopic and MRI control during a period of up to 174 months, median follow-up was 78 months., Results: Our study group consisted of 26 male and 38 female patients with an average age of 54.3 years. The majority of the patients (67 %) complained of unilateral nasal obstruction. 52 patients (81 %) were referred for primary surgery. In 12 cases (19 %) recurrent tumors were operated. According to the Krouse classification for IP the tumors were staged as T1 = 11 (17 %) cases, T2 = 37 (58 %) and T3 = 14 (22 %). In two patients a squamous cell carcinoma was associated with an IP ( = T4 stage). Most tumors were localized within the nasal cavity (72 %) or the anterior ethmoid (62 %). In 10 patients an infiltration of the bony skull base was present. During the follow-up period 6 patients developed recurrencies corresponding to an overall recurrence rate of 9.4 %., Conclusions: The advances in endonasal micro-endoscopic surgery allow both safe and effective removal of IP with low morbidity, and therefore it should be the approach of the first choice. The osteoplastic approach combined with endonasal surgery is suitable in far lateral located IP. Close follow-up is mandatory to ensure the surgical success.

Objective: To characterize the cytogenetic alterations of esthesioneuroblastoma (ENB)., Methods: Comparative genomic hybridization (CGH) was performed on genomic DNA extracted from 12 patients with primary ENB, 4 patients with tumor recurrence and 7 with metastasis. Equal amounts of biotin-labeled tumor DNA and digoxigenin-labeled normal reference DNA were hybridized to normal meta phase chromosomes. Tumor DNA was visualized by fluorescein (FITC) and normal DNA by rhodamin (TRITC ) and detected by fluorescence microscopy. The signal intensities of the different fluorochromes were quantitated as gray levels along the single chromosomes. The over-and under-represented DNA segments were determined by computation of FITC/TRITC ratio images and average ratio profiles., Results: Consensus deletion regions were most frequently observed on chromosomes 1p, 2q, 3p/q, 4p/q, 5p/q, 6q, 8p/q, 9p, 10p/q, 11p, 12q, 13q, 18q, and 21q. DNA over-representations were identified on chromosomes 1p, 7q, 9q, 11q, 14q, 16p/q, 17p/q, 19p/q, 20p/q and 22p/q. The genetic pattern of ENB was distinct from that of other small round-cell tumor types and neuroblastomas. The deletion on chromosome band 1p21-p31 was associated with bad prognosis. In particular, all patients died whose tumors had combined 1p21-p31 deletion, with tumors in clinical stage C or D, and of low differentiation (grade III or IV). Clonality analysis revealed a high concordance between pairs of primaries and metastases., Conclusion: CGH analysis identifies characteristic cytogenetic aberrations of esthesioneuroblastoma associated with its malignant phenotype.

Comparative genomic hybridization (CGH) was used to screen 22 esthesioneuroblastomas (ENB) from 12 patients including 12 primary tumors and 10 metastasis/recurrent lesions for chromosomal imbalances being the most extensive study so far. The analysis revealed a characteristic pattern consisting of deletions on chromosomes 3p and overrepresentations on 17q in up to 100% of cases. Other important alterations being detectable in more than 80% of cases were deletions on 1p, 3p/q, 9p, 10p/q along with overrepresentation on 17p13, 20p and 22q. Particularly striking was the pattern for chromosomes 3, 10 and 17q and 20 being affected almost exclusively by deletions or overrepresentations, respectively. Pronounced overrepresentations suggestive for high copy amplifications were seen on 1p34, 1q23-q31, 7p21, 7q31, 9p23-p24, 17q11-q22, 17q24-q25, 19, 20p, 20q13 and 22q13. Comparing tumor pairs from the same patient revealed a high concordance indicating clonality and confirming the genetic homogeneity of the tumor entity. The analysis of metastatic/recurrent lesions indicated a higher percentage of pronounced alterations, e.g., high copy DNA gains at 1q34-qter, 7q11, 9p23-p24, 9q34, 13q33-q34, 16p13.3, 16p11, 16q23-q24 and 17p13. The analysis furthermore suggested specific alterations, e.g., deletions of chromosome 11 and gains of 1p to be associated with metastasis formation and/or worse prognosis. Our results indicate that ENB is a distinct entity and provides criteria for its genetic distinction from other small round cell tumor types.

Objective: To summaries the treatment strategy of esthesioneuroblastoma (ENB)., Methods: Between 1988 and 2001, 17 patients with ENB were treated at the Department of Otorhinolaryngology of the Klinikum Fulda. All patients were monitored on an outpatient basis after completed therapy with a median follow-up of 44 months. In a retrospective review, the patients' charts, the computed tomography, and magnetic resonance imaging scans, the operation reports, and the follow-up data were analyzed, particularly with respect to the surgical approaches., Results: All tumors were staged according to Morita. One patient was classified as stage A, six stage B, nine stage C, and one stage D. All patients received surgical resection. Ten patients were disease free for at least 2 years, whereas 6 patients died because of ENB and one due to other disease. Of 10 patients who were free of disease, the tumors were removed via a transnasal approach in 6 patients using the microscope in combination with the endoscope. These tumors resected endonasally were staged as A (1 case) and B (5 cases). In ENB of stage C a craniofacial resection was performed using a subfrontal approach or the midfacial degloving. The lateral rhinotomy was applied only in cases in which an exenteration orbitae had to be carried out., Conclusion: ENB is best managed by complete surgical resection followed by adjuvant stereotactic radiation therapy. The Fulda surgical concept in management of anterior skull base tumors is also forwarded to resection of ENB. It classifies the following indications: 1) Endonasal approach in cases without tumor infiltration of the orbit and/or the brain; 2) Subfrontal approach in cases with extended tumor infiltration of the intradural space or the brain; 3) Midfacial degloving in cases with far lateral tumor spread, particularly fossa pterygoidea or pterygopalatina; 4) Lateral rhinotomy in all cases where an exenterative orbita is needed.

Surgery is the most common treatment for angiofibromas, but the approach is still a major point of discussion. Five cases of angiofibroma with typical localisation were treated surgically by an endonasal approach at the Fulda Academic Teaching Hospital from 1994 to 1997. This article presents an analysis of the clinical findings, computer tomography and magnetic resonance imaging, preoperative embolization, operative technique and complications. Endoscopic and radiologic follow-up ranging from 5 to 39 months excluded any residual tumour or recurrence. The endonasal microendoscopic approach with adequate preoperative embolization should be considered as an useful technique for removing tumours with considerable size without using an external incision.

Angiofibromas are usually characterized by typical location with involvement of the posterior nasal cavity and nasopharynx. They are most commonly found in adolescent males. Despite these facts, a case of an angiofibroma presented in a 15-month-old boy at an atypical site anterior and medial to the lacrimal sac. The tumor was resected via an endonasal, micro-endoscopic approach avoiding an external incision.

Angiofibromas are usually characterized by typical location with involvement of the posterior nasal cavity and nasopharynx. They are most commonly found in adolescent males. Despite these facts, a case of an angiofibroma presented in a 15-month-old boy at an atypical site anterior and medial to the lacrimal sac. The tumor was resected via an endonasal, micro-endoscopic approach avoiding an external incision.

Of the various procedures available for the reconstruction of nasal defects, examples of local flaps are demonstrated. These local flaps preserve the aesthetic and function of the nose since they prevent ugly scar retractions. An early surgical intervention minimizes the extent of the operation although radically performed and gives optimal results.