Merlin: the neurofibromatosis 2 tumor suppressor.
In recent years, it has become clear that the ERMs occupy a crucial position as protein linkers that both respond to and participate in reorganization of membrane-cytoskeletal interactions. With the identification of new binding partners, the ERMs are also implicated in linked regulation of the activities of particular membrane proteins. Thus, they reside at a junction in a complex web of interactions that must respond to stimuli from both outside and inside the cell. As expected from its structural motifs, merlin behaves in a manner similar to the ERM proteins, but with some notable differences. Chief among these is the absence of intramolecular interaction to mask intermolecular interaction domains in isoform 2. The full range of merlin's intermolecular interactions remains to be delineated, but it can be expected from the comparison to ERMs that merlin also sits within a web of interactions that may involve multiple partners and signaling pathways, some of which it shares with the ERMs. Defining merlin's tumor suppressor function will likely require identifying those differences that are peculiarly important in the target cell types of NF2. However, the fact that inactivation of merlin in the mouse by targeted mutagenesis produces a variety of malignant tumors with a high rate of metastasis  suggests that merlin's suppression of tumor formation may involve different partners and pathways in different cell types and genetic backgrounds. Consequently, the disruptions due to merlin inactivation in the progression of malignant mesothelioma may represent a tumor suppressor role operating by a different pathway than that in schwannoma or meningioma. (+info)
Nitrogen intake and tumorigenesis in rats injected with 1,2-dimethylhydrazine.
Tumor incidence was studied in 1,2-dimethylhydrazine (DMH) injected male rats assigned at weaning to isoenergetic casein-sucorse deits containing 7.5%, 15%, or 22.5% protein with or without 2.5% urea. Twenty rats fed each diet were given weekly intraperitoneal injections of DMH (15 mg/kg body weight/week) for the first 24 weeks and 20 were given saline. Of 96 DMH-injected rats necropsied after 28 weeks, 88 were necropsied during the 32nd or final week of the experiment. Adenocarcinomas of the small and large intestine were larger and significantly more numberous in rats fed 15% and 22.5% dietary protein. Keratin producing papillomas of the sebaceous glands of the external ear were observed first at 21 weeks in DMH-injected rats fed 22.5% protein. These were subsequently observed in some rats from all DMH-treated groups. As time progressed, the ear tumors increased in size and number in all groups but the greatest incidence was in the group fed 22.5% protein. No tumors were observed in saline-injected rats. Urea feeding did not increase the number of tumors nor cause changes in pH, urease activity or ammonia concentration of contents of the colon or cecum, or blood cholesterol. As dietary protein increased, cecal ammonia concentrations rose while both colon and cecal pH dropped. Portal blood urea and cholesterol reose as dietary protein was increased. DMH-treated rats had significantly higher concentrations of colon and cecal ammonia and lower blood cholesterol. Altough the rats fed 7.5% protein gained significantly less weight during 0 to 6 weeks of feeding, their weight gain was significantly higher during 6 to 26 weeks. No tumors were found in rats necropsied at 16 weeks. (+info)
Trends for invasive squamous cell neoplasia of the skin in Norway.
Over the period 1966-1995, based on 11,662 patients, the incidence of squamous cell carcinoma of the skin increased three to four times in Norway mainly as a result of an increased number of localized tumours. In men, cancer of the auricle was the second most common site; in women the incidence was low. (+info)
Cavernous angioma of the internal acoustic meatus--case report.
A 39-year-old female presented with an intrameatal cavernous angioma manifesting as hearing loss and tinnitus in the right ear which progressed over 8 months. Magnetic resonance (MR) images revealed an intrameatal lesion as ultra-high intensity, nearly as bright as cerebrospinal fluid, on the T2-weighted images, and isointensity on the T1-weighted images. Computed tomography (CT) showed the mass accompanied by stippled patterns of calcification. The patient underwent surgery under a diagnosis of calcified acoustic neurinoma. Histological studies were compatible with cavernous angioma. Intrameatal cavernous angioma is a rare disease which requires differential diagnosis from the more common neurinoma or meningioma in this location. Intrameatal lesion with ultra-high intensity on T2-weighted MR imaging and stippled patterns of calcification on CT is more likely to be cavernous angioma than acoustic neurinoma. These neuroimaging features are important information in deciding the treatment strategy. (+info)
Rapid and selective induction of erythroleukemia in female Donryu rats by continuous oral administration of 1-ethyl-1-nitrosourea.
Three groups of female Donryu rats were given continuously 1-ethyl-1-nitrosourea, 400, 200, or 100 mg/liter, in their drinking water. Leukemias developed in 94 of 104 (92%) rats surviving more than 6 experimental weeks. Of the leukemias, the erythroleukemias were induced most frequently, rapidly, and selectively. Other types of leukemias were found in a few rats of the high-dose group and, in some cases, in rats of the low-dose group. Tumors were also induced in the digestive tract, mammary glands, ear duct, and other organs, but their incidences were lower than 24%. (+info)
Malignant melanoma with liver and spleen metastases: case report.
CONTEXT: The diagnosis of primary melanoma is easily confirmed after histological analysis of the lesion, whereas it is rarely diagnosed when the patient even has distant metastases. DESIGN: Case report CASE REPORT: Malignant melanoma is responsible for about 1% of all deaths caused by cancer in the USA and only 3% of all malignant skin diseases. Malignant melanoma is a rare disease, although it corresponds to 65% of all deaths caused by skin cancer. The liver and spleen are rarely the first sites of melanoma metastases. This paper reports on the clinical picture of a patient with fatal malignant melanoma and hepatic and spleen metastases. As this was an un-usual presentation, the melanoma diagnosis could only be made after pathological analysis of the skin and hepatic lesions. (+info)
Jacobson's nerve schwannoma presenting as middle ear mass.
Schwannoma is one of the common benign middle ear space tumors. Middle ear space schwannomas may originate from the nerves of the tympanic cavity or by extensions from outside the middle ear space. In the English-language literature, the facial nerve and chorda tympani nerve, but not yet the tympanic branch of glossopharyngeal nerve (Jacobson's nerve), have been reported as the origins of intrinsic middle ear space schwannomas. We present the clinical and radiologic features of a middle-space schwannoma originating from Jacobson's nerve, and suggest that such a tumor be included in the differential diagnosis of middle ear tumors. (+info)
Undefined complications of parathyroid adenoma, parathyroid hyperplasia (primary hyperparathyroidism), thyroid follicular adenoma, thyroid papillary carcinoma, temporal astrocytoma, cerebellar meningioma, and hemangioma of external auditory meatus and oral papilloma.
A 59-year-old woman who had parathyroid adenoma, parathyroid hyperplasia, thyroid follicular adenoma, thyroid papillary carcinoma, astrocytoma of the right temporal lobe, cerebellar meningioma, capillary hemangioma of the left external auditory meatus and papilloma of the left upper gingiva is reported. Dynamic magnetic resonance imaging, computed tomography with contrast-enhancement and gastrofiberscopy revealed no remarkable findings in the pituitary, pancreas, adrenals, stomach or duodenum. Similar lesions were not found in any family members. Defect of the causative genes of multiple endocrine neoplasia types I and IIa, MENIN and RET was not detected. Further follow-up of this patient and family members is needed. (+info)