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

Neuroendocrine tumors are uncommon cancers characterized by a slow grow rate. Unresectable liver metastases are the main cause of death in patients with these tumors. This is the first Argentine report of a liver transplantation as an indication for the treatment of unresectable liver metastases from a pancreatic neuroendocrine tumor. We present a 48-year-old woman with diagnosis of a pancreatic neuroendocrine tumor with multiple bilobar unresectable liver metastases. A splenopancreatectomy was performed after a complete staging revealed absence of extrahepatic disease. Six months later, a follow-up performed with thoracoabdominal CT scan and octreo-scan was consistent with no tumor recurrence or extrahepatic disease. As the huge hepatomegaly caused a notorius deterioration in the patient's quality of life, we decided to include her in the waiting list for liver transplantation. Priority points were requested to the MELD (model for end stage liver disease) Exceptions Experts Committee with a positive response. Twelve months after the primary surgery, with a MELD score of 23 points, a deceased donor liver transplantation was performed without evidence at that moment of residual disease. Eighteen months after liver transplantation, the patient required the surgical repair of a stenosis in the biliary anastomosis. At the surgery peritoneal tumor recurrence was diagnosed. Now, 24 months after liver transplantation the patient has an excellent quality of life and a well functioning graft. We report this case of a liver transplantation as an indication for the treatment of liver metastases from a neuroendocrine tumor and we review the literature on this controversial issue.
 ...
PMID:[Liver trasplantation for the treatment of non-resectable metastases of neuroendocrine tumors: first report in Argentina]. 2223 4

A 36-year-old male who carried a diagnosis of irritable bowel syndrome presented with chronic watery diarrhea and was found to have hepatomegaly on physical exam. A computed tomography (CT) scan of the abdomen revealed hepatomegaly with lesions suspicious for metastatic disease. A colonoscopy revealed a polypoid lesion in the terminal ileum, which was biopsied, revealing a neuroendocrine tumor (NET). He was treated with palliative octreotide and chemoembolization of liver metastases until disease progression. The case highlights the importance of considering functional NETs, especially carcinoid syndrome, in patients with chronic unresolving diarrhea, since early diagnosis allows for further treatment options that can prolong survival.
...
PMID:A cause to consider for chronic unresolving diarrhea. 2532 16

Introduction: Situs inversus totalis is an uncommon anomaly which exist a complete transposition of organs and it's occasionally associated with congenital heart diseases, such as tetralogy of fallot. Pheochromocytoma is a rare neuroendocrine tumor with an annual incidence of 2-8 cases per million people and for years has been studied its relationship with the hypoxic pathway. Case Report: A 29 year old male with a history of tetralogy of fallot corrected at 10 years and situs inversus totalis. He was admitted to hospital with a progressive story of four months of constipation, palpitations, headache, dyspnea and sweating. Physical examination revealed a thinned man with peripheral cyanosis, clubbing and signs of decompensated congestive heart failure as hepatomegaly, legs edema, multifocal systodiastolic murmurs, abdominal distension and jugular venous distention. The echocardiogram shows severe right ventricular dysfunction and severe pulmonary hypertension. Furthermore, abdominal computed tomography shows right adrenal mass. Elevated metanephrines and catecholamines confirmed the diagnosis of pheochromocytoma. Surgical removal is decided and preoperative management begins with alpha-adrenergic blockade, however the patient had a hemodynamic decompensation with an unfavorable evolution. Discussion: In conclusion, there are few reports of cyanotic congenital heart disease with pheochromocytoma. Several studies show a significant association between both of them due to chronic hypoxia leads sustained hyperresponsiveness in adrenal medulla and it would cause the tumor. Special preoperative management of pheochromocytoma is recommended when there underlying heart disease and congestive heart failure. We present the first international report of tetralogy of fallot and pheochromocytoma in a patient with situs inversus totalis.
 ...
PMID:Tetralogy of Fallot and pheochromocytoma in a situs inversus totalis: An unusual association. 2777 99

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.
 ...
PMID:Canine adrenocorticotropic hormone-producing sinusoidal neuroendocrine tumor associated with Cushing's disease. 3165 39