[ rare case report of endobronchial actinomycosis with severe hemoptysis]

Endobronchial actinomycosis is an uncommon disease with symptoms of cough, hemoptysis and endobronchial obstruction. It can imitate the presentations of pulmonary tuberculosis or cancer. A 68 year-old male was referred with the complaint of fever, cough and severe hemoptysis. His weight loss during the last 3 months was 8 kgs. Blood tests indicated mild leukocytosis and increased sedimentation rate. In his chest radiography, opacity was observed in the left lower pulmonary lobe. Fiberoptic Bronchoscopy revealed a nodular lesion in the lateral segment of left lower lobe. Pathologic diagnosis confirmed the presence of actinomycosis. Although endobronchial actinomycosis is uncommon, it is included in the differential diagnosis of endobronchial obstructive lesions, especially bronchogenic malignant tumors and bronchial tuberculosis. In areas with high incidence of tuberculosis, endobronchial actinomycosis can mimic its symptoms

[Abetalipoproteinemia: report of two cases]

Abetalipoproteinemia is a rare disorder of lipoprotein metabolism, which causes low levels of cholesterol and absence or very low levels of LDL and VLDL. This rare autosomal recessive disorder is characterized by lipid malabsorption, spinocerebellar degeneration, acanthocytosis and retinopathy. In this study two cases of this disorder presented with severe malabsorption and abdominal distention, which mimicked celiac and fibrocystic diseases were reported

[Sinus histiocytosis with massive lymphadenopathy [Rosai-Dorfman disease]: a case report]

Sinus histiocytosis with massive lymphadenopathy [SHML] or Rosai-Dorfman disease [RDD] is a rare benign histiocytic disease of unknown etiology, usually associated with lymph node enlargement in various superficial or deep sites. It usually shows a prolonged clinical course with occasional exacerbation and remission phases. In this article case of RDD in a 5-year-old male with large right inguinal and bilateral cervical lymphadenopathies were, occasional fever and anemia was described. The clinical presentation, histopathologic characteristics, and treatments of RDD were discussed

Immunohistochemical expression of P53 protein in histologically favorable Wilms tumor and its relationship to tumor stage at presentation

Wilms tumor, as the most common renal tumor of children, has been associated with chromosomal abnormalities. Although a correlation between anaplasia and mutations of P53 tumor suppresser gene has been found in Wilms tumor, significance of these mutations in different clinical stages of favorable- Wilms tumor, remains largely unresolved. The goal of this study was to determine the frequency of P53 expression in histologically favorable Wilms tumors and its correlation to tumor-stage at presentation. In this retrospective study, 48 cases of confirmed Wilms tumor with favorable-histology were retrieved from the files of departments of pathology in three hospitals in Mashhad University of Medical Sciences between 1990 and 2004. Histological characteristics and clinicopathological staging were in accordance with National Wilms Tumor Study guidelines. P53 expression was determined by the immunohistochemical method. For each section, the proportion of neoplastic cells exhibiting nuclear positivity was broadly quantified and their intensity of staining was charted, based on visual impression by two pathologists. A total of 48 cases of histologically favorable Wilms tumor were assessed. Eleven cases [23%] showed positivity for P53 which were 3 [27.3%] with stage II, 3 [27.3%] with stage IV, 2 [18.2%] with stage I, 2 [18.2%] with stage III and 1 case [9.1%] with stage V. The P53 immunopositivity was seen in 1-25% of tumor cells in 9 cases [18.8%], in 26 to 50% of tumor cells in 1 case [2.1%] and in >75% of tumor cells in the other one case [2.1%]. The intensity of staining was moderate in 6 cases [12.5%], weak in 4 [6.3%] and strong only in one case 4.2%]. The most common component with P53 immunoreactivity was blastemal in 11 cases [100%]. In 4 cases [36.4%] there was also positivity in epithelial and in 2 cases [18.2%] in mesenchymal components. We found no correlation of P53 immunoreactivity and its intensity to tumor stage at presentation in individuals with histologyically favorable Wilms tumors [p=0.66, p=0.52 respectively]

[Juvenile granulosa cell tumor of the testis in a 3-month-old infant]

Juvenile granulosa cell tumor [JGCT] is a rarely diagnosed subset of benign sex cord-stromal testis tumors. Although it accounts for only 1.2% of all prepubertal testis tumors, the JGCT is one of the most common congenital and infantile testicular neoplasms. In contrast to other sex cord-stromal tumors in boys and to the ovarian homologue of the JCGT, there are no clinically evident endocrine manifestations. In this article we report a case of juvenile granulosa cell tumor of the left testis ina 3-month-old infant