Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hepatobiliary neuroendocrine carcinoma was diagnosed in 17 cats in a period of 10 years. Seven tumors were of intrahepatic origin, one of which was a composite containing components of epithelial and neuroendocrine carcinoma. Nine tumors were of extrahepatic origin, and one tumor was located in the gall-bladder. The cats were adult and geriatric, and the male : female ratio varied according to tumor group. Hepatomegaly, anorexia, weight loss, and vomiting were the most common clinical signs observed in the cats with hepatic neuroendocrine carcinoma. The cats with extrahepatic neuroendocrine carcinoma showed these signs plus icterus (5/9) and high concentrations of hepatic enzymes. Histologically, the hepatic neuroendocrine carcinomas had two patterns, one with acinar structures separated by vascular stroma lined by cuboidal or columnar cells and the other solid with groups of anaplastic cells separated by vascular stroma. The composite tumor consisted of both bile duct carcinoma and neuroendocrine carcinoma. The extrahepatic neuroendocrine carcinomas and the gallbladder neuroendocrine carcinoma were characterized by solid sheets or groups of round to oval cells with vascular or fibrovascular stroma. Immunohistochemical examination of 10 of the neuroendocrine carcinomas revealed that all 10 stained with neuron-specific enolase; one bile duct carcinoma and the gallbladder carcinoma stained with chromogranin; four of five bile duct carcinomas and the gall bladder carcinoma stained with synaptophysin; and one bile duct carcinoma stained with gastrin. One cat with hepatic carcinoma had duodenal ulcer; in this cat, ultrastructural studies showed neurosecretory granules leading to the diagnosis of Zollinger-Ellison syndrome. In four cats in which necropsy was permitted, carcinomatosis (4/4), lymph nodes (4/4), lungs (2/4), and intestines (1/4) were the metastatic sites. Fourteen of the 17 cats were euthanatized during or immediately after surgery.
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PMID:Hepatobiliary neuroendocrine carcinoma in cats: a clinicopathologic, immunohistochemical, and ultrastructural study of 17 cases. 1587 79

An adult castrated male Golden Retriever of unknown age was presented with a history of weight loss and progressive left thoracic limb lameness. On physical examination, a solid mass was palpated on the left scapula that had areas of lysis on radiographs and an area of cortical bone loss on ultrasound. Hepatomegaly, abdominal distension, and numerous intra-abdominal soft tissue masses were also found. Fine-needle aspirates of the scapula and several abdominal masses contained numerous free nuclei mixed with fewer individualized, intact cells that were round in shape and rarely formed small sheets. The cells had high nuclear to cytoplasmic ratios, central nuclei, coarsely stippled chromatin, 1-2 prominent nucleoli, and basophilic cytoplasm with indistinct cell borders. The cytopathologic interpretation was neuroendocrine neoplasia, either metastatic or multicentric. The dog was subsequently euthanized and based on gross and histologic findings at necropsy, a diagnosis of pheochromocytoma with multiple metastases was made. The neoplastic cells stained positive with Grimelius stain and were immunoreactive for synaptophysin and chromogranin A. Pheochromocytomas are rare tumors in dogs and uncommonly undergo distant metastasis, especially to bone.
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PMID:What is your diagnosis? Shoulder mass in a dog with lameness. 1949 May 68

Ewing sarcoma/primitive neuroectodermal tumor is a rare tumor of soft tissues of thoraco-pulmonary regions, pelvis and lower extremities. Involvement of visceral organs by primitive neuroectodermal tumor is even rarer, with the kidney being the most commonly involved organ. Involvement of the liver has been reported in the form of metastasis from other primary sources presenting as liver abscess. We report a 20-year-old lady presenting with massive hepatomegaly, with computed tomography scan evidence of diffuse hepatomegaly and a normal porta and intrahepatic biliary radicles. She subsequently underwent ultrasonography-guided true-cut needle biopsy of the liver. Histopathology of the liver revealed nests of small round blue tumor cells in the background of hepatocytes infiltrating the liver, which expressed Mic-2 and Fli-1, and were negative for cytokeratin, desmin, hepatocyte-specific antigen (OCHIE5), synaptophysin, chromogranin A and CD-20. Immunohistochemistry revealed CD-99-positive. Extensive search regarding any possible different site of involvement by the tumor was negative. The patient responded to a combination therapy of vincristine, adriamycin and cyclophosphamide alternating with ifosfamide and etoposide 3 weekly over 43 weeks and has been doing well even after 1 year of diagnosis. The clinical presentation, the macroscopic aspect, together with the histological pattern, the cytological characteristic and the cellular immunophenotype lead to the diagnosis of primary primitive neuroectodermal tumor of the liver which responded well to combination chemotherapy.
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PMID:Primitive neuroectodermal tumor of the liver: a case report. 1999 88

New information has demonstrated that there are few long-term disease-free survivors after a liver transplant for neuroendocrine tumors. All studies have limited follow-up to 10 years after a transplant. We present the case of a recurrent metastatic carcinoid in a patient 16 years after an orthotopic liver transplant. The subject initially presented with worsening chronic diarrhea, hypoglycemia, and confusion with massive hepatomegaly. The postoperative pathology report showed 80% to 90% of the liver tissue replaced by biopsy-proven synaptophysin-positive intrahepatic tumor with neuroendocrine differentiation. At the time of his liver transplant, he also underwent a distal pancreatectomy and splenectomy. Nuclear medicine tumor location studies, ultrasound, and computed tomography studies were performed at regular yearly intervals for 8 years on follow-up. Sixteen years after his orthotopic liver transplant, a retroperitoneal mass was detected showing neuroendocrine differentiation. Older studies focusing on an orthotopic liver transplant for highlighted clinical features would positively predict long-term survival. Older studies found the following features to be predictive of long-term survival in liver transplant for neuroendocrine tumors: age < 55 years, < 50% replacement of liver with metastatic neoplastic tissue and carcinoid type. These features were identified on multiple studies as positive predictors of disease-free survival. These studies were limited to, at most, 10-year follow-up. Newer studies have examined molecular features such as expression of E-cadherin and Ki-67 as positive predictors of long-term survival. However, no study has determined the full natural history of these tumors and for how long these patients should be followed. This anecdotal report highlights that late recurrence can occur.
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PMID:Liver transplant for neuroendocrine tumor metastatic to the liver: literature review and report of extirpation at 16-year recurrence. 2534 64

An 18-year-old male Yorkshire Terrier was admitted with a history of neurological signs including dullness and progressive tetraparesis. Physical examination revealed bilaterally symmetrical alopecia and pot-bellied abdomen. Computed tomography and necropsy examination showed a mass across the frontal sinus and cerebral frontal lobe, bilateral adrenocortical hyperplasia, and hepatomegaly. Histopathologically, the tumor lesions consisted of sheets, nests, or cords of small- to medium-sized round-to-polyhedral cells. Adrenal cortex showed bilateral diffuse cellular proliferation, and some hepatocytes showed intracytoplasmic glycogen accumulation. Immunohistochemically, the tumor cells were positive for pancytokeratin, chromogranin-A, neuron-specific enolase, S100, synaptophysin, and thyroid transcription factor-1 but negative for microtubule-associated proein-2 and neurofilament, leading to the diagnosis of neuroendocrine tumor. These tumor cells were also positive for adrenocorticotropic hormone.
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PMID:Canine adrenocorticotropic hormone-producing sinusoidal neuroendocrine tumor associated with Cushing's disease. 3165 39