Ectomesenchymal chondromyxoid tumor (ECMT) is a rare tumor. Only 26 cases of ECMT have been reported, all occurred in the anterior tongue. We present a case of a 30-year-old male with a nodule in the hard palate, which was reported as ECMT on histopathology. The differential diagnosis considered included are extraskeletal myxoid chondroma, ECMT, pleomorphic adenoma, oral focal mucinosis, chondroid choristoma, and ossifying fibromyxoid tumor. After serial sections no other component was observed and a diagnosis of ECMT was made by exclusion. The patient is asymptomatic on follow up. A review of existing literature is also presented here.
Background: Malignant peripheral nerve sheath tumor is a rare soft tissue sarcoma of ectomesenchymal origin. It is the malignant counterpart of benign soft tissue tumors like neurofibromas and schwannomas and may often follow them. Common sites include deeper soft tissues, usually in the proximity of a nerve trunk. Breast is an extremely rare location of this lesion and presentation as a breast lump in the absence of pain or previous benign neural tumor is even rarer.
Hyper cellular marrow, increased lymphocytes and plasma cells, marrow granulomas, hemophagocytosis, myelofibrosis, myelodysplasia and gelatinous transformation of the marrow were notable features the presence of which together or individually should caution a pathologist to search for Leishman Donovan (LD) bodies in patients especially in a non-endemic zone in a tropical country.
Evans syndrome is an uncommon condition characterised by simultaneous or sequential development of immune thrombocytopenia (ITP) and autoimmune haemolytic anaemia (AIHA) with a positive direct antiglobulin test (DAT) in the absence of a known underlying aetiology. The great majority of patients with Evans syndrome have a chronic relapsing course despite treatment, which is associated with significant morbidity and mortality. We reviewed the clinical and laboratory features of six patients with Evans syndrome. All patients had thrombocytopenia, bleeding symptoms and haemolytic anaemia with positive direct Coombs test at presentation. We discuss the aetiopathogenic, clinical, therapeutic and natural history of Evans syndrome.
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