Cases reported "Hepatomegaly"

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1/5. Peripheral T-cell lymphoma arising in the liver.

    We report 3 cases of primary hepatic peripheral T-cell lymphoma (PTCL). All patients were men, 50 to 57 years of age, who sought care because of systemic symptoms including fever, fatigue, and weight loss. physical examination revealed hepatomegaly in 2 patients, associated with jaundice in 1. Two patients had abnormal serum liver enzyme levels and coagulation profiles. Imaging studies demonstrated marked hepatomegaly without focal lesions in 1 patient and multiple discrete tumor masses in 2 patients. Tumor infiltrates in biopsy specimens were heterogeneous with a large cell component in 2 cases. An inflammatory background was present in all cases, complicating the histologic recognition of PTCL Immunohistochemical studies showed that all tumors were of T-cell lineage, and 2 cases had monoclonal T-cell receptor gamma chain gene rearrangements. One patient died of disease shortly after diagnosis, and 2 patients treated with multiagent chemotherapy are in clinical remission with 12 and 84 months of clinicalfollow-up, respectively. PTCL may rarely arise in the liver. These neoplasms respond to chemotherapy, suggesting that this disease is curable if diagnosed at an early stage.
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2/5. hepatoblastoma in a 15-month-old male: cytomorphology, electron microscopy, and differential diagnosis.

    The authors describe a case of hepatoblastoma in a 15-month-old male and discuss the differential diagnosis and electron microscopic features of small round cell tumors. The patient was found to have an enlarged liver and was admitted to the hospital for further investigation. Fine-needle aspiration of the liver revealed small. uniform cells with increased nuclear/cytoplasmic ratio and focal rosette formation. A diagnosis of small blue cell neoplasm favoring hepatoblastoma was made, but neuroblastoma could not be ruled out. Electron microscopic analysis performed on the liver aspirate showed features of hepatic differentiation as well as absence of neuroblastic differentiation. The diagnosis of hepatoblastoma was made. serum alpha-fetoprotein level of 33,250 mg/L confirmed the diagnosis. liver biopsy performed subsequently showed tumor cells arranged in nests, acini, and trabeculae with mitotic figures. Electron microscopy showed the same findings as described above. The patient underwent chemotherapy for 4 months and subsequently a partial liver resection was performed. This case illustrates the important role of electron microscopy in evaluating small round cell tumors in children.
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3/5. Case report: a common presentation of a rare disease-hepatosplenic T-cell lymphoma.

    Hepatosplenic T-cell lymphoma is a rare neoplasm characterized by systemic B-symptoms, hepatosplenomegaly, no lymphadenopathy, and lymphomatous infiltrates in the splenic red pulp, hepatic sinusoids, and bone marrow sinuses. The team presents the case of a healthy 30 year old man, active duty Marine, who presented with classic symptoms, yet obtaining a diagnosis took over three months from the onset of symptoms. This clinical entity initially described in 1990, is elusive, with vague and misleading symptoms. Despite aggressive conventional therapy with anthracycline-based regimens and stem cell transplant, prognosis is poor and median survival is less than one year
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4/5. Unusual hemangioendothelioma of the liver with epithelioid morphology associated with marked eosinophilia: autopsy case.

    vascular neoplasms characterized by epithelioid endothelial cells consist of several different entities from benignity to high-grade malignancy. Because of histological overlap between them, there is substantial difficulty in classifying them correctly. The present patient, a 33-year-old man, presented with hepatomegaly, striking eosinophilia and elevated serum interleukin-5 level. biopsy and autopsy revealed an unusual epithelioid vascular tumor in the liver, which is histologically distinct from epithelioid hemangioma, epithelioid hemangioendothelioma, or epithelioid angiosarcoma. The tumor cells had vasoformative and partly solid growth with no severe nuclear atypia and very low mitotic activity, and the histological features were similar to those of the entity recognized as hemangioendothelioma of bone. Organs other than the liver, for example the testes and bone, were also involved. This tumor should be considered in the differential diagnosis of severe eosinophilia.
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5/5. Occult hepatic sinusoid tumor of infancy simulating neuroblastoma.

    Two infants with hepatosplenomegaly and an occult tumor of hepatic sinusoids are reported. Although secretion of biogenic amines of neuroblastoma was not elevated, infrequent neurosecretory granules were observed by electron microscopy in the cytoplasmic processes of the tumor cells. The infants responded to vincristine and prednisone therapy and are tumor free 8 and 2 years later, respectively. The clinical, radiographic, biochemical, and microscopic findings of these cases are presented. The distinction from other infantile hepatic sinusoid small round cell tumors is based on the light and electron microscopic findings. This neuroepithelial tumor is either an unusual form of neuroblastoma or a neoplasm of APUD cell origin. If chemotherapy is utilized, it should be selective and limited.
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