Extension of adrenocortical carcinomainto the right atrium

Adrenocortical carcinoma [ACC] is a rare and highly aggressive malignant neoplasm which can produce intravascular extension into the inferior vena cava [IVC] rarely extend into the right atrium [RA]. We report a case of a male patient with large ACC with extension into the IVC and RA. Computed tomography showed a large right adrenal mass with contiguous tumor thrombus extending into IVC and RA with extension to the level of tricuspid valve. Patient underwent combined cardiac and abdominal surgical intervention on cardiopulmonary bypass with removal of the mass

Peppered and rare - gastric and duodenal pseudomelanosis: a case series

Upper Gastrointestinal [GI] pseudomelanosis is an uncommon entity characterized by endoscopic visualization of speckled dark mucosal pigmentation. While described in the rectum and colon, 'melanosis' or more aptly 'pseudomelanosis' is rare in the duodenum and exceedingly rare in the stomach. Five cases of pseudomelanosis were encountered at our department. Four females and one male were diagnosed, with a mean age of 70 years. All patients exhibited duodenal pseudomelanosis, with one demonstrating gastric antral pseudomelanosis as well. Common features among these patients included iron deficiency anemia, hypertension, chronic kidney disease, hydralazine use and iron supplementation. Biopsy specimens stained at least partially positive for iron and stains for calcium and copper were negative. Histochemical analysis revealed the pigment of pseudomelanosis to be mainly iron sulfide, exhibiting unpredictable staining patterns, hypothesized to be secondary to varying sulfur content and iron oxidation. It is visualized as dark deposits in macrophages at the tips of the duodenal villi. Upper GI pseudomelanosis remains a poorly understood finding, weakly associated with chronic kidney disease, diabetes, hypertension, iron supplements and anti-hypertensive medications. While the pathogenesis, clinical and prognostic significance remains unclear, it is thus far considered a benign condition

Urbach-Wiethe disease: experience at a tertiary care hospital in Abbottabad, Pakistan

Urbache-Wiethe disease [Lipoid Proteinosis] is a rare autosomal recessive disorder characterized by the deposition of an eosinophilic hyaline-like material in the skin, larynx, mucous membranes, brain, and other internal organs. A survey of one year duration was carried out prospectively at the Department of Dermatology, Ayub Teaching Hospital Abbottabad to document cases of lipoid proteinosis. Cases were selected from the outpatients department on the basis of clinical presentation and were subjected to detailed examination and investigations after admission. Five cases were diagnosed as suffering from Lipoid Proteinosis over the study period. All had typical features of hoarseness, skin lesions and tongue involvement. All were born of consanguineous parents. Three [60.0%] cases also gave a history of involvement of other family members, particularly cousins. This rare disease occurs in Hazara Division of North West Frontier Province of Pakistan with an as yet undetermined frequency and clinical suspicion is warranted to diagnose cases with the typical presentation