[Social reimbursement-the Spanish-German ENT Society's (SDGHNO) Latin America project]. / Soziale Rückvergütung ­ das Projekt "Lateinamerika" der Spanisch-Deutschen HNO-Gesellschaft (SDGHNO).

Nowadays, social projects are usually oriented in such a way that after a given period of time, they can either support themselves independently or even allow a pecuniary reimbursement. In the latter case, experts speak of a profit-oriented reimbursement. On the other hand, there is so-called social reimbursement, which in contrast to the abovementioned form is not profit oriented, but, for example, considers its task fulfilled by the fact of successful knowledge transfer. The Spanish-German Society for ENT Medicine and Head and Neck Surgery (SDGHNO) launched the Latin America project in 2001 under the patronage of the then President Prof. Dr. Wolfgang Draf (Fulda). The goal of the SDGHNO was and is to create a professional as well as cultural platform for Spanish- and German-speaking ENT doctors. This platform can and should be used for professional purposes, e.g., for knowledge transfer. Since the beginning of its existence, the Latin America project has thus brought numerous scientific events into being and created specific contacts which have lasted until today or have even been continued and further developed. Particularly successful examples are Chile, Colombia, and Peru. This is a vivid example of social reimbursement, because the participating German-speaking members/speakers carried out their tasks on an entirely voluntary basis. Thus, the SDGHNO did not bear any travel, catering, or accommodation costs. The activities of the SDGHNO within the framework of the Latin America project are explained.

Induction chemotherapy (IC) followed by radiotherapy (RT) versus cetuximab plus IC and RT in advanced laryngeal/hypopharyngeal cancer resectable only by total laryngectomy-final results of the larynx organ preservation trial DeLOS-II.

Background: The German multicenter randomized phase II larynx organ preservation (LOP) trial DeLOS-II was carried out to prove the hypothesis that cetuximab (E) added to induction chemotherapy (IC) and radiotherapy improves laryngectomy-free survival (LFS; survival with preserved larynx) in locally advanced laryngeal/hypopharyngeal cancer (LHSCC). Patients and methods: Treatment-naïve patients with stage III/IV LHSCC amenable to total laryngectomy (TL) were randomized to three cycles IC with TPF [docetaxel (T) and cisplatin (P) 75 mg/m2/day 1, 5-FU (F) 750 mg/m2/day days 1-5] followed by radiotherapy (69.6 Gy) without (A) or with (B) standard dose cetuximab for 16 weeks throughout IC and radiotherapy (TPFE). Response to first IC-cycle (IC-1) with ≥30% endoscopically estimated tumor surface shrinkage (ETSS) was used to define early responders; early salvage TL was recommended to non-responders. The primary objective was 24 months LFS above 35% in arm B. Results: Of 180 patients randomized (July 2007 to September 2012), 173 fulfilled eligibility criteria (A/B: larynx 44/42, hypopharynx 41/46). Because of 4 therapy-related deaths among the first 64 randomized patients, 5-FU was omitted from IC in the subsequent 112 patients reducing further fatal toxicities. Thus, IC was TPF in 61 patients and TP in 112 patients, respectively. The primary objective (24 months LFS above 35%) was equally met by arms A (40/85, 47.1%) as well as B (41/88, 46.6%). One hundred and twenty-three early responders completed IC+RT; their overall response rates (TPF/TP) were 94.7%/87.2% in A versus 80%/86.0% in B. The 24 months overall survival (OS) rates were 68.2% and 69.3%. Conclusions: Despite being accompanied by an elevated frequency in adverse events, the IC with TPF/TP plus cetuximab was feasible but showed no superiority to IC with TPF/TP regarding LFS and OS at 24 months. Both early response and 24 months LFS compare very well to previous LOP trials and recommend effective treatment selection and stratification by ETSS. Clinical trial information: NCT00508664.

Predictors of the outcome of nasal surgery in terms of olfactory function.

Nasal surgery is frequently performed. Predictors of surgical outcome in terms of chemosensory function are not clear. A total of 64 patients were included in this prospective study (41 men, 23 women; age range 22-67 years). Prior to surgery, patients received a detailed otorhinolaryngological examination including nasal endoscopy, and CT scans used to establish the Lund-Mackay score. Olfactory function was analyzed using a custom-built odor identification test which allowed separation of chemosensory function into "olfactory" and "trigeminal" portions. Retest was performed 6 months after surgery. In terms of the sense of smell, nasal surgery produced the highest success rates in women, high degree of polyposis, and aspirin-intolerance. Neither age, presence of asthma, nor the number of preoperative surgical interventions had a significant impact on the outcome of surgery in terms of chemosensory function. Although "trigeminal scores" changed to a lesser degree than "olfactory scores", the present results indicated that nasal surgery may also improve trigeminal function, although this needs further corroboration. Improvement of olfactory function following nasal surgery appears to last, on average, for at least 6 months. While the present results may be seen as an encouraging step towards the description of more detailed prognostic factors related to nasal surgery, results from the present investigation also point towards the idea that nasal polyposis is due to a multifactorial process that, so far, is not adequately addressed by current research. Future work is needed to identify further predictors of postoperative outcome in terms of olfactory function.

[Endonasal micro-endoscopic resection of sinonasal inverted papilloma]. / Endonasale mikro-endoskopische Resektion invertierter Papillome der Nase und ihrer Nebenhöhlen.

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.

[Extraosseous osteosarcoma of the thyroid gland]. / Extraossäres Osteosarkom der Schilddrüse.

Extraosseous osteosarcomas are very rare malignant neoplasms that have been published in 300 cases so far. Manifestation within the thyroid gland was described in 6 cases only. We report a 69 year old female who suffered from enlarged recurrent struma after primary resection and radiotherapy 5 years ago. Histology revealed a high grade extraosseous osteosarcoma. Presenting the patient's clinical, radiological and histological findings the therapeutical options of this rare tumor entity are discussed.

[Paraganglioma of the head and neck--tumor control, functional results and quality of life]. / Paragangliome des Kopf-Hals-Bereiches--Tumorkontrolle, funktionelle Ergebnisse und Lebensqualität nach Operation.

BACKGROUND: Surgical therapy for paragangliomas (PG) of the head and neck is, due to the alternatives of radiation therapy and wait-and-scan strategy and because of postoperative morbidity, under ongoing discussion. MATERIAL AND METHODS: Between 1981 and 2004, 79 patients with 94 PG of the head and neck were treated at our department. These patients had follow-up examination within a clinical trial considering tumor control, functional results and for the first time neuropsychologically evaluated postoperative quality of life. Mean follow-up time was 65 months (1 to 228 months). RESULTS: Among the 94 PG there were 19 carotid body (GCP), 12 vagal nerve (GVP) and 63 jugular-tympanal paragangliomas (JTP). Of these, 87 tumors underwent surgery. In 68 patients (78.1 %), the tumor could be removed completely. In particular, complete resection of GCP was achieved in 100 %, of GVP in 90.9 %, of JTP type A in 100 %, of JTP type B in 83.3 %, of JTP type C in 66.6 % and of JTP type D in 61.5 %. During the follow-up period, residual or recurrent tumors were diagnosed in 17 patients (19.5 %). Six of the seven residual PG were observed by magnetic resonance tomography and did not show growth. One residual PG and 6 recurrencies were resected completely. One recurrent tumor was radiated and 3 others are under observation without showing growth tendencies. Two patients died postoperatively due to borderline operations of extended tumors. The quality of life after PG surgery showed a SIP of 4.8, which is comparably much better than after acoustic neuroma surgery (SIP 10.3). CONCLUSION: Whereas complete tumor resection of GCP and JTP types A and B is almost ever possible without cranial nerve palsies, surgery of GVP and advanced JTP causes often severe functional deficits. However, postoperative quality of life is mostly good. Nevertheless, advanced PG require an individualized therapeutic regime also including radiation and observation of tumor growth.

[Modern anesthesiologic concepts supporting paranasal sinus surgery]. / Moderne Anästhesieverfahren in der endonasalen Nasennebenhöhlenchirurgie.

BACKGROUND: Optimal vision is essential for successful endonasal sinus surgery. Beside topical vasoconstriction general anaesthesia can contribute to reduce intraoperative bleeding. METHODS AND RESULTS: For many years deliberate hypotension was used to prevent intraoperative bleeding. The intentional reduction of systolic blood pressure to 50-60 mm Hg was achieved by the use of Sodium Nitroprusside alone or in combination with other vasoactive agents. However, intraoperative bleeding is not affected by this technique unless the systolic blood pressure falls below 60 mm Hg which can cause serious side effects for the patient. Recently, there is growing evidence that not only systolic blood pressure but also a low heart rate (< 60 beats per minute) can minimize surgical bleeding. With the introduction of total intravenous anaesthesia (TIVA) by the use of Propofol and Remifentanyl an anaesthetic technique has been established which fulfils the haemodynamic requirements in endonasal sinus surgery in many regards. The inhibiting effects on the cardiovascular system of these drugs alone can lead to a reduced bleeding. TIVA allows the reduction of the systolic blood pressure to 60 mm Hg as well as the heart rate below 60 beats per minute. If necessary it can be supported by vasoactive agents of which betablockers have a theoretical advantage. CONCLUSION: For general anaesthesia in endonasal sinus surgery Sodium Nitroprusside is no longer recommended. Instead a TIVA using Propofol and Remifentanil should be used.

[Glottic laryngeal carcinoma. Tis, T1 and T2--long term results after laser resection]. / Glottische Larynxkarzinome. Tis, T1 und T2--Langzeitergebnisse nach Laserresektion.

BACKGROUND: Endoscopic laser resection is one treatment modality for early glottic carcinoma. Benefits are the preservation of laryngeal structure without tracheotomy, the short duration of treatment, low traumatisation and good functional results. METHODS: From 1989 to 1999, 143 patients with an isolated and previously untreated glottic squamous cell carcinoma were treated by CO2 endoscopic laser resection. The tumors were classified as carcinoma in situ (Tis) in seven cases, T1 tumor (T1N0M0) in 91 patients and T2 tumor (T2N0M0) in 45 cases. Median follow-up was 5 years. RESULTS: For the group of Tis and T1 carcinomas, 86 of 98 patients were free of recurrences. The 12 recurrences (12.2%) were treated by repeated laser surgery and laryngectomy (four patients). None of these patients died of tumor related causes. For patients with T2 carcinomas, the overall recurrence rate was 28.9% (13 patients). In six patients, a total laryngectomy had to be performed and one patient died because of tumor recurrence. The relapse free survival estimate using the Kaplan-Meier method was 87% for Tis and T1 carcinomas and 70% for T2 carcinomas. The overall laryngeal preservation rate was 95% for Tis and T1 carcinomas and 85% for T2 tumors. All recurrences occurred within 4 years of primary surgery. CONCLUSION: The results suggest that the oncological outcome after endoscopic laser surgery is comparable to conventional open partial resections.

[Endonasal micro-endoscopic tumor surgery: state of the art]. / Stellenwert der endonasalen mikro-endoskopischen Tumorchirurgie.

BACKGROUND: The aim of the present study was to establish the efficacy of endonasal micro-endoscopic surgery for removal of benign and malignant neoplastic lesions of the paranasal sinuses and the anterior skull base. MATERIAL AND METHODS: This retrospective study evaluated 350 patients with tumors of the paranasal sinuses and the anterior skull base (215 benign and 135 malignant tumors) that were treated surgically at the ENT-Department of the Hospital Fulda gAG between 1993 and 2003. Median follow-up was 65 months. RESULTS: Most frequent entities of benign tumors were osteomas, inverted papillomas and juvenile angiofibromas. Adenocarcinomas, squamous cell carcinomas and esthesioneuroblastomas were most frequently treated in the group of malignomas. 54 % of the benign (n = 118) and 41 % of the malignant tumors (n = 54) were resected exclusively via the endonasal micro-endoscopic approach. Within the follow-up period 3 recurrencies were observed, two inverted papillomas (one was operated endonasally) and one juvenile angiofibroma. In the malignoma group 34 patients died because of tumor disease (16 cases due to recurrencies, 18 cases due to metastases formation). The 5 year survival rate was 66.4 % with respect to the disease-specific survival. The Kaplan-Meier analysis revealed statistically significant differences for the pT stage: pT2 and pT3 tumors have had a 5-year disease-specific survival of 92.3 % and 83.8 %, respectively, compared to 61.5 % of the pT4 tumors. Disease-specific survival also showed differences dependent on histology, tumor site and occurrence, but was without proven significancy. In the patient subgroup who suffered from adenocarcinoma, squamous cell carcinoma or esthesioneuroblastoma the 5-year disease-specific survival was 78.4 % of 29 patients after endonasal resection compared to 66.4 % in 51 patients operated via an external approach. CONCLUSION: The advances in endonasal micro-endoscopic surgery also allow a safe and effective removal of benign and malignant anterior skull base tumors with low morbidity. Thereby, indication is dependent on tumor site and size as well as histology.

[Paranasal sinus mucoceles: surgical management and long term results]. / Mukozelen der Nasennebenhöhlen: Chirurgisches Therapiekonzept und Langzeitergebnisse.

BACKGROUND: The aim of this study was to determine the best surgical approach in the treatment of paranasal sinus mucoceles according to their localization. MATERIAL AND METHODS: A retrospective analysis was carried out in 255 patients with 290 sinus mucoceles who were treated surgically at the ENT-Department of the Hospital Fulda gAG between 1983 and 2001. This series include 125 frontal sinus, 23 frontoethmoid, 41 ethmoid, 72 maxillary sinus and 26 sphenoid mucoceles. The patients' history, presenting symptoms, radiological findings, and surgical management were reviewed. Of them, 185 patients were followed by endoscopic and CT or MRI control during a period of up to 19 years, median follow-up was 12 years. RESULTS: In 168 out of 255 patients (66 %) the mucoceles were arisen due to previous sinus surgery, in 37 cases (14 %) after traumatic lesions, in 5 patients (2 %) due to chronic sinusitis and in 2 cases (<1 %) according to tumors. In 43 cases (17 %) no causes were found. In 78.8 % the previous operation was performed via an external approach, either according to Jansen/Ritter or Caldwell-Luc, contrary to 1.5 % after endonasal surgery. The median period until mucocele appearance was 15 years for maxillary sinus, 13 years for frontoethmoid, 10 years for ethmoid, and 8 years for frontal and sphenoid sinus celes, respectively. 201 mucoceles (69.3 %) have been operated endonasal micro-endoscopically, 18.6 % via the osteoplastic approach, 10 % endoscopically combined with an osteoplastic procedure and only 2 % according to Jansen/Ritter. Thereafter, recurrence of mucoceles was found in 4 patients only ( = 2.2 %; related to the endonasal approach = 1.6 %). CONCLUSION: Frontoethmoidal, ethmoidal, sphenoidal and maxillary sinus mucoceles are excellent indication for exclusively endonasal micro-endoscopic surgery. The osteoplastic approach combined with endonasal surgery is suitable in far lateral located frontal or maxillary sinus mucoceles.

[Reconstruction of the frontoorbital frame using split-thickness calvarial bone grafts]. / Tabula externa zur Rekonstruktion des frontoorbitalen Knochengerüstes.

BACKGROUND: There are many autogenous and allogenous grafts as well as alloplastic materials available for the reconstruction of craniofacial bony frame. We review our techniques and describe the advantages of using calvarial bone, especially split-thickness grafts for bone reconstruction in this area. PATIENTS AND METHODS: Between 1996 and 2003 the orbitocranial bony frames of 15 patients were reconstructed using split calvarial bone grafts at the ENT-Department of the Hospital Fulda gAG. In 12 patients the anterior frontal sinus wall or the entire Os frontale were affected. In 1 patient each the lateral wall, roof and floor of the orbit had to be reconstructed. The causes of the bone defects were trauma (n = 6), recurrent frontal sinusitis partly with osteomyelitis (n = 4), benigne tumors (n = 2) and malignancies (n = 3). Twelve patients have had multiple previous operations. In 3 patients the bone reconstruction was performed in the same operation as the tumor removal. Within the follow-up period between 2 and 8 years the split calvarial bone grafts remained stable in size and shape. Graft rejection, osteomyelitis or bone resorption did not occur. Furthermore, we have not experienced significant complications in harvesting cranial bone and have not seen major donor site morbidity. CONCLUSIONS: Our results demonstrate that split-thickness calvarial bone is an excellent graft not only for facial and forehead contouring but also for orbital and complex craniofacial reconstruction.

[Management of congenital nasal fistulas and cysts]. / Operatives Management von kongenitalen Nasenfisteln und -zysten.

BACKGROUND: Congenital nasal fistulas and cysts are uncommon craniofacial malformations. Mostly their symptoms appear already during childhood. In general, they are superficially located but they can reach the skull base or extend intracranially. PATIENTS AND METHODS: Between 1979 and 2004 fifteen patients (11 children and 4 adults) with nasal fistulas and cysts were treated surgically at the ENT-Department of the Hospital Fulda gAG. In 7 patients (46.6 %) the skull base was involved. An intracranial intradural extension was found in 1 child and therefore needed a combined ENT and neurosurgical procedure. In the other 6 cases the nasal fistulas or cysts were located extradurally. They were resected by removing the Christa galli and strenghten the dura. An additional septorhinoplasty was carried out in 4 of the 15 patients. Within the follow-up period of up to 25 years no recurrences were observed. CONCLUSIONS: The adequate therapy of nasal fistulas and cysts consists of complete resection. Magnetic resonance tomography (MRI) and computed tomography (CT) are essential to diagnose intracranial extension and to choose the proper surgical approach including a potential interdisciplinary procedure.

[Angiography of the carotid artery in centro-lateral skull base fractures?]. / Karotisangiographie bei zentro-lateralen Schädelbasisfrakturen?

BACKGROUND: In latero-basal, central or spheno-fronto-orbital skull base fractures the internal carotid artery is more frequently involved in severe lesions than expected. PATIENTS AND METHODS: Between 1996 and 2003 we examined 684 patients with Glasgow Coma Scales (GCS) between 2 and 15, median 7.2, using computed tomography (CT). In suspicion of a latero-basal, central or spheno-fronto-orbital fractur they got an additional high resolution skull base CT. If the bony canal of the internal carotid artery (ICA) was involved the patient underwent digital subtraction angiography (DSA). The ICA lesion was treated either interventional neuroradiologically, by surgery or only conservatively. RESULTS: Of the 684 patients 33 (4.8 %) had fractures of the ICA bony canal and therefore underwent DSA. Among them were 25 men and 8 women (mean age 35.3 years). Lesions of the ICA were seen in 1.9 % of the patients. A traumatic cavernous-carotid fistula was found in 7 patients (1 %) and in 6 patients (0.9 %) a dissection and/or an aneurysm of the ICA was diagnosed. Six of the patients had clinical symptoms. The lesions were treated primarily interventional neuroradiologically (n = 5) as well as surgically in two cases by clipping the aneurysm and closing the sphenoid sinus, respectively. CONCLUSIONS: Vessel lesions of the ICA in skull base fractures and involvement of the bony carotid canal are more frequent than mentioned in current literature. A solid diagnosis can only be achieved by DSA. Early diagnosis and treatment is important for improving the prognosis of these often multiply injured patients.

[Functional results, cognitive and effective quality of life disturbances after trans-temporal resection of acoustic neuroma]. / Funktionelle Ergebnisse, Lebensqualität, kognitive und affektive Konsequenzen nach transtemporaler Exstirpation von Akustikusneurinomen.

BACKGROUND: The aim of the study was to evaluate postoperative hearing, facial nerve function, quality of life (QOL), affective status and neuropsychological performance after trans-temporal removal of acoustic neuromas (AN). PATIENTS AND METHODS: A retrospective analysis was carried out in 89 patients with AN who were operated at the ENT-Department of the Hospital Fulda between 1988 and 2004. Median follow up was 57 months. Of these 41 patients were additionally examined by a psychologist for evaluation of QOL, neuropsychological functions and affective disorders using several questionnaires. RESULTS: The AN were classified as follows: Type A = 53 %, type B = 35 % and type C = 12 %. One year postoperatively facial nerve function was excellent in 93.3 % of the patients (grade I and II). In 53 % of cases hearing could be preserved. The subjective QOL was expressed through depression and social withdrawal associated with deafness. However, facial nerve dysfunction did not lead to QOL effect. Objective QOL (functional level) was reduced because of verbal memory disturbances and symptoms like dizziness. In 47.5 % of the patients affective and/or neuropsychological dysfunctions were diagnosed. Subsequent MRI evaluation showed lesions of the temporal lobe in 40.5 % of the cases. Of these 80 % suffered from cognitive and/or affective disturbances. CONCLUSIONS: The cognitive and affective disturbances after trans-temporal removal of AN could be to a certain degree due to the elevation of the temporal lobe during surgery. Further research, especially pre- and postoperative examination of QOL and psychological state as well as the comparison between different approaches, particularly trans-temporal vs suboccipital have to clear up specific morbidity of the different approaches.

[Management of occult malformations at the lateral skull base]. / Versorgung okkulter Missbildungen der lateralen Schädelbasis.

BACKGROUND: Occult malformations of the lateral skull base are rare anomalies, but can cause severe complications such as recurrent meningitis. Therefore, they need to be precisely delineated and sufficient surgical closure is mandatory. PATIENTS AND METHODS: Between 1986 and 2004 twenty patients (10 children and 10 adults) with occult malformations at the lateral skull base were treated surgically at the ENT-Department of the Hospital Fulda gAG. Of these 3 Mondini-malformations, 11 defects of the tegmen tympani or the mastoidal roof, 2 dural lesions to the posterior fossa and 4 malformations within the pyramidal apex have been found. Four patients have had multiple anomalies. Routing symptom was in all cases at least one previous meningitis. Radiological diagnostics included high-resolution computed tomography (CT) and magnetic resonance imaging (MRI) as well as CT- or MR-cisternography. Depending on type and localisation of the defect the following surgical algorithm was carried out: The trans-mastoidal approach was used in all cases of Mondini-malformation (including obliteration of the ear), in case of lesions to the posterior fossa as well as partly in anomalies at the tegmen tympani and mastoidal roof, respectively. Defects of the pyramidal apex should be explored via the trans-mastoidal way if the lesion is located caudally to the inner auditory canal (IAC), whereas the trans-temporal approach should be used if the lesion is situated ventral to the IAC and dorso-medially to the internal carotid artery (ICA). The trans-temporal approach was also performed in large defects of the tegmen tympani and mastoidal roof as well as in recurrences. CONCLUSIONS: In all cases of recurrent meningitis caused by agents of the upper airway tract the basic principle should be to search for occult skull base malformations radiologically as well as by sodium fluorescein endoscopy as long as the anomaly is detected.

[Clear cell chondrosarcoma of the larynx. A rare tumor in an uncommon location]. / Klarzellchondrosarkom des Larynx. Ein sehr seltener Tumor in einer aussergewöhnlichen Lokalisation.

Clear cell chondrosarcoma is a rare bone tumor of low malignancy. It constitutes only 2% of all chondrosarcomas and shows a particular predilection for the epiphyses of long bones. So far, there have only been three cases of clear cell chondrosarcoma of the larynx published in the literature. We report the case of a 46 year old man who suffered from slowly worsening dyspnoea. Diagnostic evaluation revealed a directly subglottic stenosis of the larynx, narrowing the lumen to 1/3. When we tried to resect the stenosis via splitting the ring cartilage from outside, tumorous infiltration of the whole ring cartilage was found. Extended biopsies were taken and histology revealed a clear cell chondrosarcoma. The therapy of choice for this tumor is wide surgical resection. Additional radio- and/or chemotherapy are not recommended. Therefore, the patient underwent laryngectomy. Histologically, the clear cell chondrosarcoma can be easily confused with the highly malignant osteosarcoma or the conventional chondrosarcoma, both requiring more aggressive treatment. Thus, clear cell chondrosarcoma should be kept in mind and, when necessary, a reference histology by a bone tumor register should be requested.

[Carcinoma of the external auditory canal and middle ear: therapeutic strategy and follow up]. / Therapeutisches Management von Gehörgangs- und Mittelohrkarzinomen.

BACKGROUND: Carcinomas of the external auditory canal (EAC) and the middle ear are rare and considered to have a poor prognosis. The recommended therapeutic strategy consists of surgical excision and postoperative radiotherapy. However, there are different opinions about the extend of the primary operation. PATIENTS AND METHODS: A series of 21 patients with carcinoma of the EAC and middle ear were treated at the ENT-Department of the Hospital Fulda from 1985 to 2003. Their records and radiologic findings were reviewed retrospectively with particular reference to tumor type and size, its relation to surrounding tissues, surgical procedures and radiation techniques. The tumors were staged according to the modified Pittburgh staging system for temporal bone carcinomas. The average follow-up time was 6.2 years (range 0.2 - 18.75). RESULTS: 17 patients suffered from carcinoma of the EAC, 4 carcinomas were primarily located in the middle ear. There were 15 squamous cell carcinomas, 3 adenoidcystic carcinomas, 2 adenocarcinomas and one mucoepidermoid carcinoma. 12 patients came primarily to our institution and were staged as follows: pT1 (n = 2), pT3 (n = 2), pT4 (n = 8). 8 patients showed up with recurrent or residual tumors (all of T3 or T4 stage). One patient could not be classified. In 5 cases the tumor was inoperable. These patients underwent combined chemoradiation therapy. All other 16 patients were operated and most of them received adjuvant radiation therapy. In the group of patients who were primarily operated overall 5-year survival rate was 100 %. In contrast, patients who's recurrent or residual tumors were resected had a 5-year survival rate of only 33 %. Patients who received combined chemoradiation therapy showed a 2-year survival rate of 75 %. CONCLUSION: Carcinoma of the EAC and middle ear should be treated primarily by a lateral or subtotal temporal bone resection stage dependent combined with a parotidectomy as well as a neck dissection. Local resection of the EAC is not sufficient, not even in T1 tumors. As from stage T2, in cases of recurrent tumor removal and questionable free margins as well as in cases with lymph node metastases an adjuvant radiation therapy should be added. The most important survival factor is removal of the primary tumor with histologically clear margins.

Visualization of inner ear dysplasias in patients with sensorineural hearing loss.

PURPOSE: We evaluated a data acquisition and post-processing protocol for inner ear (IE) assessment by MR imaging in patients, suffering from various labyrinth malformations. MATERIAL AND METHODS: MR IE studies of 158 consecutive patients (316 IEs) suffering from sensorineural hearing loss without evidence of an acoustic neurinoma were reviewed for pathologies of the IE and internal acoustic meatus. High-resolution MR data of all abnormal IE studies (n=45) were post-processed to previously standardized 3D volume rendered (VR) reconstructions. RESULTS: In 9 patients (5.7%) the following IE dysplasias were detected: malformation of the cochlea (6 IEs), vestibulum (4 IEs), semicircular canals (12 IEs) and vestibular aqueduct/endolymphatic sac (10 IEs). One patient showed evidence of an aplasia of the vestibulocochlear nerve. In 4 patients multiple IE dysplasias were encountered. Comprehensive 3D visualization of all labyrinthine dysplasias was achieved by the use of two VR reconstructions. The overall time for bilateral IE assessment amounted to 30-35 min. CONCLUSION: The imaging protocol allows for rapid and comprehensive visualization of various IE dysplasias, based on a limited number of VR reconstructions.

Cyclin D1 polymorphism and expression in patients with squamous cell carcinoma of the head and neck.

We have previously reported that the cyclin D1 (CCND1) GG870 genotype was associated with poorly differentiated tumors and reduced disease-free interval in patients with squamous cell carcinoma of the head and neck (SCCHN). We have now examined the association of this and a second CCND1 polymorphism with gene expression and outcome in SCCHN patients. Analysis of a CCND1 G/C1722 polymorphism revealed that CCND1 CC1722 genotype was associated with poorly differentiated tumors [P = 0.005; odds ratio (OR), 5.7; 95% CI, 1.7 to 19.2), and reduced disease-free interval (P = 0.003; Hazard Ratio (HR), 7.3; 95% CI, 1.1 to 27.2.) independently from the influence of CCND1 GG870 genotype. Patients whose tumors were negative for cyclin D1 were associated with reduced disease-free interval (P = 0.028; HR, 4.1; 95% CI, 1.4 to 14.2). Although G/C1722 genotypes were not associated with expression, we found a significant trend between reduced expression of cyclin D1 in patients with the CCND1 GG870 genotype (P = 0.04). Splicing of CCND1 mRNA in head and neck tissues was modulated by CCND1 A/G870 alleles, thus CCND1 transcript a was spliced equally from CCND1 A870 and G870 alleles, whereas CCND1 transcript b was spliced mainly from the CCND1 A870 allele. Our analysis has also identified differences in cyclin D1 genotype and protein expression and the pathogenesis of SCCHN in males and females. Thus, CCND1 CC1722 genotype was more common in female patients (P = 0.019; OR, 3.3; 95% CI, 1.3 to 10) and cyclin D1 expression was more frequent (chi-square1, 3.96; P = 0.046) and at higher levels (P = 0.004) in tumors from female patients. In summary, our data show that the two CCND1 polymorphic sites are independently associated with tumor biology and clinical outcome. CCND1 A/G870 alleles affect gene expression in head and neck tissues. We also provide preliminary evidence that the molecular genetics of SCCHN development may be influenced by patient gender.

High-resolution petrous bone imaging using multi-slice computerized tomography.

Multi-slice computerized tomography (MSCT) is considered to provide superior image quality. We defined a data acquisition protocol for high-resolution (HR) temporal bone imaging using MSCT and assessed its impact on data acquisition and post-processing (PP). The data acquisition protocol was defined in cadaveric phantom studies performed by MSCT and subsequently applied to 38 patients referred for temporal bone assessment. The parameters image quality and diagnostic value of MSCT data were assessed for the cross-sectional source images as well as for 2-dimensional (2D) reformations and 3-dimensional (3D) reconstructions by 3 radiologists by comparison with incremental HR scans of 17 patients with suspected middle ear disorders. The data acquisition protocol yielded HR images with an excellent detail resolution and a comparable image quality of cross-sectional scans and related orthogonal reformations. MSCT achieved higher scores for image quality and diagnostic value (p < 0.001, t-test) than incremental HR CT with regard to both 2D and 3D reconstructions. MSCT improves the image quality of HR cross-sectional scans as well as that of 2D and 3D PP techniques in petrous bone imaging. The radiation exposure of the eye lenses is increased by MSCT as gantry angulation is not yet possible in the helical scan mode.