Cholesteatoma of the cerebellopontine angle

Congenital cholesteatoma is the third most common tumor found in the cerebellopontine angle. It must be differentiated from acoustic neuromas, meningiomas, metastatic tumors, arachnoid cysts and lipomas. Symptoms include hemifacial spasm, progressive facial paralysis, hearing loss, tinnitus, vertigo, pain and otorrhea. Radiologic and magnetic resonance imaging frequently can be useful to establish a preoperative diagnosis. The treatment of choice is total removal of the lesion. Complete removal with preservation of normal structures is the most difficult and technically exacting procedure performed by the neurotologic surgeon. The clinical features and results from a series of 19 cases, nine of which extended into the cerebellopontine angle, are discussed.     

Epithelial cyst in the cerebellopontine angle

Aase-Smith syndrome (ASS) is usually defined as a dominantly inherited combination of arthrogryposis, Dandy-Walker malformation and cleft palate. We describe a sporadic case of foetal akinesia with abnormal fossa posterior, fitting the diagnosis of ASS, and discuss the nosology of this entity among syndromes with distal arthrogryposis. ASS shows a "hybrid" phenotype: adults with mild ASS could be classified as distal arthrogryposis, whereas severely affected newborns overlap with the Marden-Walker phenotype, which is recessively inherited. The specificity of the disorder comes from the coexistence of both forms in the same pedigree, so that ASS appears impossible to diagnose with certitude in sporadic cases. We suggest that the severe expression of ASS is only the extreme but aspecific expression of a dominantly inherited form of distal arthrogryposis. Implications for genetic counselling in distal arthrogryposis are outlined.     

Cholesteatoma of the cerebellopontine angle. A case report

In this paper are recalled the most important features of this sinusal condition, its behaviour, complications and so on. Regarding the management the A. recall to the classic surgery, well done, orderly and systematically planed, which give him always good outcomes. By the way accordingly to the promising it could be, he refers to endoscopic functional sinusal approach.     

Lipomas of the internal auditory canal and cerebellopontine angle

OBJECTIVE: To evaluate lipomas of the internal auditory canal (IAC) and cerebellopontine angle (CPA). STUDY DESIGN: Retrospective review. METHODS: Review of a multi-institutional series of 17 lipomas of the IAC/CPA, combined with a Medline review of the 67 cases reported in the world literature. RESULTS: This series of 17 IAC/CPA lipomas is the largest reported series to date, bringing the total number of documented cases to 84. There appears to be a nearly 2:1 male to female predominance. Sixty percent were left-sided lesions, and three were bilateral. Hearing loss, dizziness, and tinnitus were the most common presenting symptoms. Surgical resection was performed in 52 (62%) of these lesions; however, total tumor removal was accomplished in only 17 (33%), which is most likely because of the fact that these tumors tend to have a poorly defined matrix and a dense adherence to neurovascular structures. Sixty-eight percent of patients experienced a new deficit postoperatively, 11% were unchanged, and only 19% improved with no new deficit. Only one documented case of tumor growth was identified; however, the reported follow-up was short (average, less than 3 years). CONCLUSION: With the magnetic resonance imaging techniques now available, lipomas can be reliably differentiated from other masses within the CPA and IAC, so histopathologic diagnosis is rarely necessary. Because of the potential for significant morbidity with resection of these lesions, we believe that conservative follow-up is the best treatment option for patients with these rare lesions. Surgery is indicated only when significant progressive or disabling symptoms are present.     

Treatment of lung metastases from malignant melanoma with IL-2 inhalation therapy

Seven patients with pulmonary metastases of malignant melanoma were treated with inhalation therapy with 36 million IU interleukin-2 for six months. Inhalation therapy was combined with four bolus infusions of DTIC at a dose of 850 mg/m2 once every four weeks. Response rates were 71.4% with 2 patients achieving a complete remission (CR), 2 partial remissions (PR), 1 stable disease (SD), and 2 progressing disease (PD). Therapy was well tolerated with low toxicity. Six of the patients developed cough; one patient was slightly feverish. We conclude that inhalation therapy of lung metastases is a promising addition to the therapeutic arsenal against malignant melanoma.     

A case of facial nerve neurinoma originated from cerebellopontine angle

Both the activation of protein kinase C (PKC) and the facilitation of depolarization-evoked glutamate release have been implicated in the induction of hippocampal long-term synaptic potentiation. These observations may be functionally related, since stimulation of PKC activity enhances evoked glutamate release. Recently, it was shown that arachidonic acid and the diacylglycerol analog oleoyl-acetyl-glycerol activate brain PKC in a synergistic fashion. We report the facilitation of depolarization-induced glutamate efflux from hippocampal mossy fiber synaptosomes due to a combination of arachidonic acid and oleoyl-acetyl-glycerol. The potentiating effects appeared to depend on the activation of PKC since they were attenuated by staurosporine. In addition, the effects of arachidonic acid and oleoyl-acetyl-glycerol appeared to be limited to calcium-dependent processes.     

Radical resection of primary malignant melanoma of the gallbladder with multiple metastases: report of a case

We present herein an usual case of primary malignant melanoma of the gallbladder in a 51-year-old man in whom an exploratory laparotomy for melena revealed six malignant melanoma lesions located in the gallbladder, main pancreatic duct, stomach, duodenum, jejunum, and a mesenteric lymph node. Total pancreatectomy was performed and histologically, junctional activity was seen only in the gallbladder, suggesting that this was the primary site. No melanotic lesions were found on the skin or eyes. The metastases to the main pancreatic duct and gastrointestinal tract appeared likely to have occurred as a consequence of the mucosal dissemination of the tumor cells shed into the bile. The post-operative course was uneventful and combined chemotherapy was administered for 16 months. No new metastatic lesions were found until 21 months postoperatively, when metastases were detected in the brain and thoracic spinal cord. These metastatic tumors were removed surgically, but the patient died from cerebral disturbance 26 months after the initial operation. Thus, we consider that aggressive surgical therapy was effective for extending the survival time and improving the quality of life of this patient.     

The accuracy of predicting lymph nodes metastases in malignant melanoma by clinical examination and microstaging

Since 1971, a prospective treatment regimen for primary cutaneous malignant melanoma performed by a single clinician has revealed the following early observations: 1) A significantly higher number of females with level II disease; 2) No recurrences or metastases to date in 29 patients with level II lesions treated by appropriate surgery; 3) The apparent clinical predictability of lymph node metastases in the group microstaged at level III. 4) An inability to predict lymph node metastases (or their delayed development) in patients with level IV disease; 5) A correlation between lymph node metastases and the development of disseminated disease.     

Prevention of malignant melanoma

Dermatologists and physicians of other specialties, as well as other health professionals have made tremendous efforts to improve the public education (primary prevention) and early detection (secondary prevention) of cutaneous malignant melanoma (CMM) especially during the last decade. In Australia, the country with the highest incidence of CMM in the world, the first public and effective campaigns were already carried out in the sixties. Through the public campaigns, the knowledge increased about skin cancer, and the attitude and behavior toward sun exposure changed in the population. In the USA and Great Britain too, effective public campaigns were carried out in great numbers and extensive experience was acquired. In Germany, prevention campaigns were first run in regional areas. In 1989, the Commission of Early Detection and Prevention of Melanoma of the German Dermatological Society launched nation-wide campaigns. These activities were complemented by regional campaigns in the 1990s. The analysis of previous campaigns demonstrates that single activities are less effective and repeated campaigns are necessary to increase knowledge about skin and to change attitudes and behavior towards UV-exposure. In addition, the development of sun protective clothing and structural changes, e.g. creation of shady places around open-air swimming pools, should be aimed for. Physicians of other specialties and other health professionals should also be included in prevention campaigns.     

Chemotherapy of malignant melanoma

Previous studies have shown the kidney to be an important site for the catabolism of secretin. We have investigated possible sites within the kidney for secretin uptake in four intact anesthetized dogs. The disappearance half-time of an intravenous infusion of secretin, measured by a sensitive and specific ratioimmunoassay in four intact anesthetized dogs was 2.84 minutes. After ureteral ligation (to arrest glomerular filtration), the half-time of a similar intravenous dose of secretin was 2.78 minutes. Finally, the renal vascular pedicles were ligated to totally exclude the kidneys from the circulation and the half-time was found to be 5.43 minutes. These findings demonstrate that the efficient renal mechanisms for secretin degradation are not dependent upon glomerular filtration but upon some other mechanism, presumably located in renal tubular cells.     

Immunology of malignant melanoma

Traditionally a homograft valve is used as a pulmonary replacement device for the Ross operation. Right ventricular outflow tract reconstruction during aortic valve replacement with an autograft was performed with stentless xenograft valves in nine patients. Hemodynamic performance is satisfying, but, long term evaluation is needed.     

Biochemistry of malignant melanoma

The biochemistry of malignant melanoma is reviewed. The biosynthesis of melanin from tyrosine is described and the role of tyrosinase and other enzymes in melanoma considered. Detailed methods for the assay of free catechols, their metabolites and urinary indole melanogens are included. Normal values for these constituents and their significance in the evaluation of melanoma patients are discussed.     

Primary malignant melanoma of the vagina

We report two cases of primary malignant melanoma of the vagina explaining the essential clinical pathological and therapeutic features. They themselves, added those represented in literature, might constitute a further contribution to best definition of the unusual genital pathology, especially as regards prognostic and therapeutic aspects.     

Primary malignant melanoma of the oesophagus

We report the case of a 74-year-old female patient in whom a primary malignant melanoma of the oesophagus was detected at the time of investigation of phlebothrombosis. Therapy of choice for this extremely rare tumour is radical surgical resection of the oesophagus. Even after surgical resection, primary oesophageal melanomas have a very poor prognosis. According to the present state of knowledge, it remains unclear to what extent the prognosis could be improved by adjuvant therapeutic procedures with radiotherapy or chemotherapy and immunostimulation.