ABSTRACT
Sclerosing extramedullary hematopoietic tumors (SEMHTs) are associated with chronic myeloproliferative neoplasms. These extremely rare mass lesions were first described in kidney and peritoneum. On histopathology, they are characterized by sclerosis, entrapped fat, atypical megakaryocytes with myeloid and erythroid elements. Only approximately ten cases have been subsequently reported in orbit, lacrimal system, liver, omentum, and skin. The authors present a case of SEMHTs as incidentally detected omental nodules, while the patient was undergoing splenectomy for Janus kinase-2 negative myelofibrosis. The authors postulate their origin in omentum-associated lymphoid tissue; and highlight the diagnostic dilemma presented by SEMHTs at frozen section.
ABSTRACT
Extragonadal germ cell tumors are rare and comprise 2-5% of all germ cell tumors. Seminoma/dysgerminoma, teratoma, yolk sac tumor (YST) and mixed germ cell tumors are few of the extragonadal germ cell tumors arising within the brain. Of these, primary pure YSTs arising in the brain are extremely rare and arise in the pineal (62%) and suprasellar (31%) regions. Primary YST occurring in the cerebellum is exceedingly rare, associated with a very poor prognosis and has been reported in less than 10 cases in literature. We herein report a case of primary pure yolk sac tumor in the cerebellar vermis in a 2-year-old child.
ABSTRACT
Focal nodular hyperplasia (FNH) is a benign condition of the liver often discovered incidentally on radiological investigation. Although FNH is a well-described lesion in the literature considerable diagnostic problems regarding this entity still remains. We report a case of multiple FNH in a 23-year-old male patient detected as an incidental finding in autopsy. On gross examination FNH was not suspected because of the multiple lesions and the lack of central scar which is characteristically described in FNH. The diagnosis was established on histopathology after examination of multiple sections of the lesions.
Subject(s)
Autopsy , Focal Nodular Hyperplasia/diagnosis , Focal Nodular Hyperplasia/pathology , Histocytochemistry , Humans , Incidental Findings , Liver/pathology , Male , Microscopy , Reticulin/analysis , Silver Nitrate , Staining and Labeling/methods , Young AdultABSTRACT
Syndromic paucity of bile ducts or "Alagille syndrome" is characterized by peculiar facies, chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects and peripheral pulmonary artery hypoplasia or stenosis. We present a two-year-old female child with the 'partial' or 'incomplete' Alagille syndrome. The child had three of the five major features of the syndrome. A brief review of literature of the syndrome is presented.