RESUMO
Well-differentiated papillary mesothelioma is a rare tumor occurring predominantly in the peritoneum of young women, a few with history of asbestos exposure. A 28-year-old woman presented with ascites and pain abdomen. Ultrasonography and computed tomography scan of the abdomen revealed a mass in the retroperitoneum measuring 15 cm × 12 cm. Histopathological examination along with immunohistochemistry (IHC) confirmed it to be a papillary mesothelioma in the peritoneum. It is difficult to differentiate from more common malignant mesothelioma and papillary adenocarcinoma, which also have poorer prognosis. The difficulty can be resolved by clinico-radiological correlation along with histopathological examination and IHC.
RESUMO
Fanconi anemia (FA), commonly inherited as an autosomal recessive trait, is the commonest cause of inherited bone marrow failure syndrome often accompanied by skeletal deformities, genitourinary abnormalities and an increased risk of malignancies especially acute myeloid leukemia. Haemoglobin E ((ß26 GluâLys)) is common haemoglobin variant in the north-eastern part of India and the northern part of West Bengal. Here, we report a case of FA with concomitant haemoglobin E-trait in a 5-year-old child who presented with weakness, epistaxis, malaena and intermittent fever. She had a hyper-pigmented patch over the nape of neck and polydactyly. Complete blood counts revealed pancytopenia. The bone marrow aspiration and trephine biopsy showed marrow hypoplasia. Chromosome analysis showed chromatid and chromosome breaks in 22 % cells suggestive of FA. The results were corroborated with MMC stress test. Thus, we present the first reported case of FA with haemoglobin E-trait.