[Excisional surgery of orbital tumors]. / Exzisionale Chirurgie orbitaler Tumoren.

The indications for orbital tumor surgery are an incisional biopsy to confirm the diagnosis or in malignant operable tumors a complete excision or a debulking to avoid complications in large invasively infiltrating tumors. In the case of benign tumors, the indications for surgery depend mostly on the clinical symptoms and cosmetic esthetic disfigurement. In the present article the preoperative examinations as well as surgical access approaches to different orbital regions, endoscopic procedures and methods of intraoperative navigation are presented. Magnetic resonance imaging is the instrument of choice, whereby in many cases computed tomography (CT) adds further information. Depending on the indications, diffusion-weighted sequences, CT angiography and digital subtraction angiography (DSA, catheter angiography) are added to the preoperative diagnostics. For space-occupying lesions located anterior to the bulbar equator, an anterior orbitotomy can be performed transconjunctivally or transpalpebrally. A lateral orbitotomy is used to reach lateral, laterocranial, and lateroinferior orbital segments, whereas transcranial approaches are suitable for processes located far posterior and for those with retro-orbital intracranial extension as well as for processes in the optic foramen/superior orbital fissure. The indications for an endonasal access approach are processes medial to the bulb or optic nerve and up to the orbital apex. A transantral access can be chosen for caudal, mediolateral, and medioinferior space-occupying lesions. Modern orbital surgery is complemented by endoscopic procedures and intraoperative navigation. Orbital tumors belong to the interdisciplinary relevant diseases. Therefore, an optimal management takes place at specialized multidisciplinary centers.

Erratum to: Hearing preservation in children with electric-acoustic stimulation after cochlear implantation : Outcome after electrode insertion with minimal insertion trauma.

The article Hearing preservation in children with electric-acoustic stimulation after cochlear implantation-Outcome after electrode insertion with minimal insertion trauma, written by T. Rader, A. Bohnert, C. Matthias, D. Koutsimpelas, M­A. Kainz, S. Strieth was originally published electronically.

[Is routine histopathologic examination of middle ear cholesteatoma necessary?] / Ist die routinemäßige histologische Untersuchung von Mittelohrcholesteatomen nötig?

BACKGROUND: In the course of restoration of the middle ear, routine histological examinations are initiated in many cases, although a middle ear cholesteatoma can be clinically diagnosed in a normal case. AIM OF THE STUDY: To determine the correlation between the surgeons' diagnosis and that of the pathologist and comparison with literature data. To check the rate of unexpected diagnoses. Analysis of the costs and survey of German ENT clinics with regard to handling of the histological examination in suspected cholesteatoma. MATERIALS AND METHODS: Retrospective evaluation of cholesteatoma cases of the ENT Department of the University Medical Center of Mainz from 2010-2015. Germany-wide postal survey of ENT clinics. RESULTS: In all, 449 operations for the rehabilitation of a middle ear cholesteatoma, of which there were 312 (69.5%) first diagnoses and 137 (30.5%) recurrences. A histological examination was conducted in 78.6%. For first diagnoses, the sensitivity of the clinical assessment was 97.9%, specificity 10%, positive predictive value 96.3%, and negative predictive value 16.7%. Recurrences showed values of 100%, 40%, 97.1% and 100%. Unexpected malignant findings did not occur. This routine histological examination was performed by 58.6% of German ENT hospitals. A total of 80% of those questioned this to be useful. A histological examination cost an average of 14.06 €. CONCLUSION: Intraoperatively, there is a high degree of accuracy in diagnosing cholesteatoma. The cost factor of the histological examination is low and should not be the basis for the decision. The ear microscopy and the experience of the ear surgeon should be decisive for the decision for histological examination.

Hearing preservation in children with electric-acoustic stimulation after cochlear implantation : Outcome after electrode insertion with minimal insertion trauma.

BACKGROUND: Cochlear implantation in patients with functional residual low-frequency hearing is performed according to an established hearing-preserving surgical technique in order to cause minimal trauma of inner ear structures. Due to the increasing number of cochlear implants in children, the preservation of residual hearing is becoming increasingly important in this patient collective. OBJECTIVES: Short- and mid-term hearing preservation outcome in pediatric patients is investigated. MATERIALS AND METHODS: A group of 9 children (12 ears) between 5 and 12 years of age were examined after hearing-assisted cochlear implantation with respect to the pure tone audiometric thresholds. Retrospectively, short-term hearing preservation (up to 3 months after surgery) was examined. In a subgroup of 5 children, mid-term hearing preservation (7.5 to 16 months after surgery) was also analyzed. The mean values of hearing preserved (HL%) and hearing loss (HL) due to electrode insertion were calculated as measured values. RESULTS: In the whole group, the mean values of the preoperative PTAlow were 29.8 dB and the short-term postoperative PTAlow 42.6 dB. The mean value of the HL% was 73.6%, corresponding to an HL of 9.4 dB. In the subgroup, the mean PTAlow postoperatively was 46.0 dB in the mid-term and the HL% at 80.7% with a HL of 6.6 dB. CONCLUSIONS: The results in children are consistent with the results in adults. Electric-acoustic stimulation (EAS) should be used in the treatment of children with existing low-frequency residual hearing, as good residual hearing preservation can also be achieved in children after implantation.

[Hearing preservation in children with electric-acoustic stimulation after cochlear implantation : Outcome after electrode insertion with minimal insertion trauma (German version)]. / Hörerhalt bei Kindern mit elektrisch-akustischer Stimulation nach Cochleaimplantation : Ergebnisse nach Elektrodeninsertion mit minimalem Insertionstrauma.

BACKGROUND: Cochlear implantation in patients with functional residual low-frequency hearing is performed according to an established hearing-preserving surgical technique in order to cause minimal trauma of inner ear structures. Due to the increasing number of cochlear implants in children, the preservation of residual hearing is becoming increasingly important in this patient collective. OBJECTIVES: Short- and mid-term hearing preservation outcome in pediatric patients is investigated. MATERIALS AND METHODS: A group of 9 children (12 ears) between 5 and 12 years of age were examined after hearing-assisted cochlear implantation with respect to the pure tone audiometric thresholds. Retrospectively, short-term hearing preservation (up to 3 months after surgery) was examined. In a subgroup of 5 children, mid-term hearing preservation (7.5 to 16 months after surgery) was also analyzed. The mean values of hearing preserved (HL%) and hearing loss (HL) due to electrode insertion were calculated as measured values. RESULTS: In the whole group, the mean values of the preoperative PTAlow were 29.8 dB and the short-term postoperative PTAlow 42.6 dB. The mean value of the HL% was 73.6%, corresponding to an HL of 9.4 dB. In the subgroup, the mean PTAlow postoperatively was 46.0 dB in the mid-term and the HL% at 80.7% with a HL of 6.6 dB. CONCLUSIONS: The results in children are consistent with the results in adults. Electric-acoustic stimulation (EAS) should be used in the treatment of children with existing low-frequency residual hearing, as good residual hearing preservation can also be achieved in children after implantation.

[Changes in Incidence and Mortality Trends of Head and Neck Cancer in Rhineland-Palatinate, 2000-2009]. / Veränderung von Inzidenz und Mortalität von Kopf-Hals-Malignomen in Rheinland-Pfalz, 2000-2009.

BACKGROUND: Epidemiological data on HNC are often reported aggregated despite their anatomical and histological heterogeneity. In Germany, few studies have analyzed incidence and mortality trends separately for specific anatomic sites. Furthermore, little is known about whether the incidence of HPV-associated tumour entities of the head and neck region has increased. METHODS: Based on cancer registry data from Rhineland-Palatinate from 2000 to 2009, age-standardized incidence and mortality rates were calculated for all HNC sites and localisation groups that might be HPV-associated according to the literature. Trends were analyzed by Joinpoint regression and reported as the annual percentage change (APC). RESULTS: Throughout the study period, 8 055 incident cases and 3 177 deaths were identified. The incidence rates of overall HNC increased among women (APC:+2.2%) and declined slightly among men (- 0.9%). Significantly increasing incidence rates among women were seen for tumours of the oral cavity (+2.7%) and the oropharynx (+3.6%). Among men, a significant decrease in incidence rates for tumours of the hypopharynx (-3.4%) and the larynx (-2.7%) are noteworthy. Cancers at HPV-associated sites showed increased incidence rates in men (+3.3%) and women (+4.3%). A decrease in mortality was found for tumours of the larynx in both sexes (-5.8% men,-9.1% women). CONCLUSIONS: A detailed analysis by localisation of HNC showed significant and often opposing trends for men and women regarding incidence and mortality.

[Primary plexiform ameloblastoma in the sinonasal tract of a 49-year-old female patient. Case report]. / Primäres sinunasales Ameloblastom bei einer 49-jährigen Frau. Fallbericht.

Sinonasal ameloblastoma is a rare tumor, which most frequently affects male patients in the 6th-7th decade. We report on the case of 49-year-old female patient presenting with nasal obstruction. Computed tomography revealed a solid and hypodense mass filling the maxillary sinus and the nasal cavity. The histological examination showed a plexiform ameloblastoma without significant bone erosion. Viewing this together with the literature review, we suggest that sinonasal ameloblastoma in female patients becomes apparent approximately one decade earlier than in male patients. This tumor should be considered in the differential diagnosis of unilateral sinonasal symptoms.

Lack of neurofibromatosis type 2 gene promoter methylation in sporadic vestibular schwannomas.

BACKGROUND: Vestibular schwannomas (VS) are benign tumors of the nervous system that are usually sporadic but also occur in the inherited disorder neurofibromatosis type 2 (NF2). In VS, losses of chromosomal material and mutations of the NF2 gene have been established to be causative. For a subset of VS without detectable gene alterations, promoter inactivation by hypermethylation has been suggested. However, published data are very limited and contradictory. METHODS: We analyzed NF2 gene promoter methylation in 35 sporadic VS by methylation-specific PCR. RESULTS: Twenty-three of the tumors were informative, showing no promoter methylation. In the remaining 12 tumors, promoter methylation could neither be verified nor excluded. CONCLUSIONS: Our study suggests that NF2 gene inactivation by promoter hypermethylation is a rare or very uncommon mechanism of NF2 gene inactivation in sporadic VS. Other mechanisms destabilizing the NF2 gene product, yet to be identified, might play a role in the genesis of VS apart from the loss or mutation of the NF2 gene.

The VEGF/VEGF-R axis in sporadic vestibular schwannomas correlates with irradiation and disease recurrence.

BACKGROUND/AIMS: The molecular mechanisms downstream of mutated neurofibromatosis type 2 (NF2) gene resulting in the growth and development of vestibular schwannoma (VS) are controversial. Several lines of evidence suggest the involvement of the vascular endothelial growth factor (VEGF) pathway in VS development. Given that recent studies of VEGF blockade in patients with NF2-associated VS showed positive effects on VS growth control, we initiated this comprehensive study of the VEGF pathway in sporadic VS. METHODS: A tissue microarray analysis of 182 sporadic VS was conducted. The expression of VEGF and its receptors as well as the proliferative activity of the tumors were quantified. The expression data were correlated to tumor volumes and diameters as well as to tumor recurrence and previous irradiation. RESULTS: All studied tumors expressed VEGF and its receptors. Proliferative activity was related to the growth characteristics of the tumors. Moreover, we found significantly higher VEGF levels in recurrent tumors (p = 0.0387) and in preoperatively irradiated tumors (p = 0.0213). CONCLUSION: Our data suggest a relevant role of the VEGF pathway in VS growth and therapy outcome. Therefore, targeting this pathway using antiangiogenic compounds might be beneficial for patients with sporadic VS, especially those with recurrent or irradiated tumors.

Multifocal adult rhabdomyoma of the head and neck: a case report and literature review.

Adult rhabdomyoma is a rare, usually solitary, primary tumour of striated muscle origin that almost exclusively presents in the head and neck region with predilection for male. We herein present a rare case of a multifocal adult rhabdomyoma in a 72-year-old woman. The lesions were located, the first one in the area of the left aryepiglottic fold and the second one right cervical arising from oesophagus. The diagnosis of the cervical mass was obtained prior to resection through cytological examination (FNAC). We discuss the clinical, cytological and histological findings and we provide a brief review of the literature on this entity.

Proangiogenic effects of ionizing irradiation on squamous cell carcinoma of the hypopharynx.

OBJECTIVE: There is experimental evidence that ionizing irradiation affects a proangiogenic response. However, the relevance of this effect on tumour growth in vivo is not in detail investigated yet. The present objectives were to examine the influence of ionizing radiation on the expression of the vascular endothelial growth factor (VEGF) and its receptors (flt-1 and flk-1), the microvessel density and the tumour proliferation, in head and neck squamous cell carcinoma (HNSCC). METHODS: We used a HNSCC-cell line, derived from a hypopharyngeal tumour, for subcutaneous injection in 16 athymic nude mice. After reaching an average diameter of 12-14 mm the xenografts were randomised and 8 out of the 16 animals (therapy group) were irradiated with a single fraction of 6 Gy while the control group remained without any intervention. The irradiated and the respective control tumours were prepared after 7 (T7) and 70 days (T70) for immunohistochemical analysis. The expression of VEGF, its receptors flk-1 and flt-1, the vessel density (CD31) and the proliferation rate (Ki67) were quantified. RESULTS: At the point of time T7 we observed a reduction of the tumour growth rate, of the proliferative activity and of the VEGF- as well as of the VEGF-R-expression. At the point of time T70 we found increased values for proliferation, microvessel density, VEGF- and flk-1 expression in the therapy group compared to the therapy group at T7 as well as to the control group at T70. CONCLUSION: These changes might suggest a long-term proangiogenic effect of irradiation, which might result in growth promotion of the remaining tumour after the end of therapy.

Cervical adult rhabdomyoma presenting as a rapidly growing mass in a patient with diffuse large B-cell non-Hodgkin's lymphoma.

BACKGROUND: Adult rhabdomyoma is a rare mesenchymal tumor, which generally grows slowly and is mainly localized in the head and neck area. PATIENT AND METHODS: We report the extraordinary case of a rapidly growing adult rhabdomyoma in a 73-year-old man. The patient was treated for diffuse large B-cell non-Hodgkin's lymphoma with CHOP therapy (doxorubicin, cyclophosphamide, vincristine, and prednisone). Comparison of the respective computed tomography scans showed prominent enlargement of 35% in the tumor mass volume on the right side of the neck within 3 months. The tumor was highly suspicious for lymphoma. Surgical resection was performed. RESULTS: Histological examination revealed a tumor which was composed of tightly packed polygonal cells with a PAS-positive granular or vacuolated cytoplasm, occasionally with cross-striations. Immunohistochemically, the cells were positive for desmin, myogenin, Myo-D1, but negative for S-100. Due to these characteristic morphologies, adult rhabdomyoma was diagnosed. CONCLUSION: This is the first report on an adult rhabdomyoma with a proven rapid enlargement. The possible pathomechanisms are discussed.