Genetic analysis revealed the same novel mutation (K317M) in exon 11 of the MAPT gene in both pedigrees. A common haplotype between members of the two pedigrees suggests that they belong to the same family.
The clinical and pathologic features of 6 cases of intestinal-type adenocarcinoma of the sinonasal region are presented. These cases were collected in a 17 year period (1972-1988) and account for less than 4% of malignancies of this region in our records for this period. All of the patients were men aged 48 to 82 years (mean, 54 years). Previous exposure to wood dust was reported in 1 case. Radiographic studies, especially computerized tomography, were of critical importance to delineate the extent of tumors. Nasal obstruction was the most common complaint. Duration of symptoms prior to diagnosis is available in 5 cases and ranged from 5 to 36 months (mean 18 months). Surgical treatment was performed in 4 patients (of palliative type in 2) followed by radiotherapy in 3. Histopathology revealed tubulo-papillary (5 cases) and mucinous (1 case) patterns. Follow-up is available in all patients (range 0 to 108 months), 50% of whom are still alive. In our series, only 1 patient has survived more than 5 years. Data pooled from the literature reveal that 53% of patients have experienced local recurrences following therapy, and 60% have died of their disease. Of these deaths, 80% occurred within 5 years of diagnosis.
Adenomyoepithelioma of the breast is a rare neoplasm. We present a case of a benign adenomyoepithelioma of the breast in an asymptomatic 60-year-old woman. This report illustrates the mammogram, ultrasound, and magnetic resonance appearances of this unusual lesion, with histopathological correlation.
The radiologic and histologic features of an inflammatory pseudotumor of the liver in a 62‐year‐old man are presented. The lesion was coincidentally discovered during clinical work‐up of an acute pancreatitis caused by choledocholithiasis. Malignancy was suspected in the light of the radiologic presentation. To our knowledge, only 23 cases of inflammatory pseudotumor of the liver have appeared in the international literature. Its etiology remains unknown, although reactive‐inflammatory or infectious mechanisms have been suggested. The present case seemed to be associated with bile flow obstruction, as the mass partially regressed following cholecystectomy and Vaterian sphincterotomy. Therefore, a hypothetical obstructive pathogenetic mechanism is considered. We conclude that this tumor‐like condition must be considered in the differential diagnosis of solid masses in the liver.
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