[Malignant transformation of a juvenile papilloma in a 11 year old boy]. / Maligne Entartung einer juvenilen Kehlkopfpapillomatose bei einem 11-jährigen Jungen.

BACKGROUND: The juvenile laryngeal papilloma is the most common benign neoplasm in children. Often the entity shows an elongated recurrent course of disease with an expansion into the tracheo-bronchial system. Sporadic malignant transformation in adults with a papilloma was reported after treatment with radiotherapy alone or in combination with the intake of additional toxins (e. g. nicotine). Similar reports of a malignant transformation of juvenile papillomas without additional risk factors is very rarely reported. CASE REPORT: We report about an 11 year old boy, who suffered from a juvenile laryngeal papilloma. The multiple laser surgical procedures and a therapy with interferon resulted in a short-term remissions. A tracheotomy was inevitable. Seven months after the first diagnosis of the papilloma a regional metastatic squamous cell carcinoma was found. In spite of combined radiotherapy and chemotherapy the boy died 11 months later. CONCLUSIONS: The spontaneous malignant transformation of a juvenile papilloma in a squamous cell carcinoma is extremely rare. The surgical intervention as well the radiotherapy and chemotherapy using interferon was unsuccessful due to the high grade of malignancy. In view of the very short time interval between first diagnosis of juvenile papilloma and the subsequent malignant transformation, one must consider either the potential presence of a very aggressive form of papilloma or alternative two coincident independent diseases.

[Long term results in adenoidcystic carcinoma]. / Langzeitergebnisse beim adenoidzystischen Karzinom.

BACKGROUND: Due to the discreet initial symptoms and the locally aggressive infiltration with perineural spread the adenoidcystic carcinoma (ACC) presents a special diagnostical and therapeutical challenge. PATIENTS: : In a retrospective study the forms of 54 patients were analysed, whose average age was 55.5 (24 - 77) years. RESULTS: The sex ratio showed a slight female preponderance with 57 % to 43 %. The major salivary glands were affected in 26 cases. The exact distribution of the ACC was: parotid gland (n = 18), submandibular gland (n = 8), oral cavity (n = 10), paranasal sinuses (n = 11), nasopharynx (n = 4) and larynx (n = 3). The most common symptoms were a tumor-related swelling and pain which persisted for a duration of several months after final diagnosis could be established. Facial palsy was observed in 4 patients. Histological examination revealed a tubular subtype in 4 cases, in 28 cases a cribriforme and in 12 cases a solid subtype. No definitive differentiation was possible in 10 specimens. The skull base was infiltrated in 16 patients. Except one patient all 54 underwent surgical therapy. Postoperative radiotherapy was additionally given in 25 cases which was combined with a chemotherapy in 6 patients. Nevertheless ACC recurred in 60 % of our patients. Lymphnode metastases were observed in 13 patients after a latency of 3.3 years in average, but predominantly pulmonary metastases as distant spread developed in 18 patients after 5.8 years significantly later. The overall survival rate was 84.38 % after 2 years, 75.90 % after 5 years, 50.49 % after 10 years and 20.11 % after 20 years. Male sex, infiltration of the skull base and histological evidence of perineural and perivascular spread proved to be statistically significant factors for an unfavourable prognosis. CONCLUSIONS: Due to the uncommon biological behaviour with a slow growth on the one hand side and an aggressive local invasion on the other hand side the ACC can be regarded as a challenging malignant disease for the clinician whose adequate therapy does not allow any standardized regime. The tendency for recurrence even after a period clinically free of symptoms makes a life long follow-up mandatory.

Adenoid cystic carcinoma of the skull base.

Adenoid cystic carcinoma (ACC) is a slowly growing tumor with a particular tendency to infiltrate the surrounding tissue by perineural spread. The clinical diagnosis may prove difficult due to the submucons extension of the tumor, especially at the skull base. This article outlines the clinical characteristics, diagnostics, and treatment modalities in a series of 56 patients with an ACC in the head and neck diagnosed between 1970 and 1998 in 32 females and 24 males. The youngest patient was aged 24 years, the oldest 77 years. The average age was 54 years. In 16 patients the tumor originated in the paranasal sinuses or the nasopharynx and involved the skull base. As a rule, several months passed between the manifestation of the first symptoms such as pain, blocked nose, epistaxis, or diplopia and the initial clinical diagnosis. All patients received surgical treatment, however, complete microscopical resection could only be achieved in approximately one third of the cases. Therefore, nine patients were postoperatively treated with radiotherapy. The average survival rates of the patients with an ACC of the skull base were only 99 months as compared to 144 months in the patients without skull base involvement.

[Neonatal hearing screening with the Echosensor automated device for otoacoustic emissions. A comparative study]. / Neugeborenenhörscreening mit dem automatisierten Messgerät Echosensor für otoakustische Emissionen. Eine vergleichende Untersuchung.

Following the recommendations of the United States National Institutes of Health Consensus Conference in 1993, otoacoustic emissions (OAE) are now used internationally for hearing screening. The use of recording systems as well as the interpretation of results requires specially trained personnel although measurements are easier to perform than other recording methods available. To date, no objective method for general hearing screening, has been introduced in Germany, for neonates or children at risk, even though it would be desirable to detect and rehabilitate children with congenital hearing loss as early as possible (incidence, 1-6 per 1000). The Echosensor provides the means for carrying out OAE recordings within a short period of time and includes an automatic evaluation of results. Recordings can also be made by trained personnel. As a comparison test, audiologically trained staff recorded otoacoustic emissions in 111 new-born children in order to compare the results of the Echosensor with the results of a conventional OAE measurements device (the ILO88). The aim of this pilot study was to determine the reliability and validity of the recordings in comparison with available standards in brainstem audiometry. Our study showed that the results of the Echosensor corresponded well with the ILO88 results. Consequently, an OAE measuring method is now available is also provides high sensitivity and specificity and is easy to use. Our findings show that the Echosensor can meet the demands of systematic hearing screening in Germany.