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)

An acutely ill 6-month-old female infant presented with massive hepatomegaly, accompanied by severe anemia with peripheral normoblastemia and thrombocytopenia. Bone marrow examination revealed erythroid hyperplasia with gross erythroid dysplasia, reduced granulocytic precursors, and virtually absent megakaryocytes. The bone marrow also contained completely necrotic cells occurring in clumps as well as singly. The appearances suggested bone marrow involvement by neuroblastoma. Accordingly, combination chemotherapy was instituted and laparotomy was performed as soon as her clinical condition had improved. Left adrenalectomy was carried out, because a small adrenal nodule of ganglioneuroma was present. Liver biopsy showed expansion of portal tracts by loose fibrous connective tissue containing hemosiderin deposits and some degenerate cellular debris, consistent with areas of involuted metastatic neuroblastoma. Complete recovery followed, and subsequent bone marrow examination was entirely normal. It is thought that the dyserythropoiesis probably resulted from the release of toxic metabolites from regressing neuroblastoma.
 ...
PMID:Transient dyserythropoiesis occurring during the involutionary phase of stage IV-S neuroblastoma. 271 38

Neuroblastoma, the most common malignant tumor in the neonatal period is metastatic in 25 to 50% of cases. While the prognosis of antenatal neuroblastoma is often considered as favorable, included in the most common metastatic stage (Stage IV S), it can lead to fetal or neonatal death. We report a case of a fetus with a stage IV neuroblastoma who died in utero. The most important prognostic factor is tumor stage, making sonographic detection of metastasis essential. Nevertheless, accurate staging remains difficult by sonography. When metastatic neuroblastoma is suspected, sonographic survey has to be reinforced, and if serious criteria such as massive hepatomegaly, placentomegally or hydrops appear, delivery must be considered.
 ...
PMID:[Antenatal metastatic neuroblastoma: prognostic criteria. A case report]. 1506 99

Stage 4S metastatic neuroblastoma (NB) has a favorable prognosis due to a high rate of spontaneous regression. Young infants risk lethal complications arising from hepatomegaly, which can develop rapidly despite treatment. MYCN oncogene amplification confers a significantly worse prognosis. We describe a 4-week-old neonate with MYCN-amplified stage 4S NB complicated by gross hepatomegaly causing rapidly progressive respiratory, hepatic, and renal failure. The child remains in remission 3 years after hepatic artery embolization, radiotherapy, standard, and high-dose chemotherapy. Embolization of the hepatic artery, with classical treatment, is feasible and safe at this age and may contribute substantially to the management of high-risk patients.
 ...
PMID:Successful treatment of MYCN amplified, progressive stage 4S neuroblastoma in a neonate with hepatic artery embolization in addition to multimodality treatment. 1592 57

In this article, we report the case of a newborn who presented a life-threatening hepatomegaly with respiratory distress at 12 days of life, complicating a metastatic neuroblastoma. Low-dose liver radiotherapy was performed in emergency in order to decompress. Chemotherapy has also been delivered due to a tumoral relapse 1 month after radiotherapy. After a follow-up of 20 years, this young woman is still in complete remission, with no long-term sequelae.
 ...
PMID:[Neonatal neuroblastoma complicated with a threatening tumoral hepatomegaly treated by irradiation: No sequel 20 years later]. 2656 72

We aim to provide an up-to-date summary of infantile hepatic hemangioma (IHH) and its misnomers and to dialectically present the differential diagnosis of these rare entities of the liver. Eligible peer-reviewed articles on hepatic infantile hemangiomas, published between 2000 and 2015, were reviewed for this study. IHH is the most common hepatic vascular tumor in children. Once a liver mass is identified in an infant, the differential diagnosis ranges from vascular malformations to benign and malignant tumors including mesenchymal hamartoma, hepatoblastoma, metastatic neuroblastoma, so careful physical examination, imaging studies, and, if indicated, tumor markers and biopsy, are of pivotal importance to ascertain the correct diagnosis. Despite the benign nature of IHHs, some of these lesions may demand medical and/or surgical intervention, especially for multiple and diffuse IHH. Complications can include hepatomegaly, hypothyroidism and cardiac failure. Therefore, a close follow-up is required until complete involution of the lesions. We propose an algorithm to guide the physicians towards the proper management of hepatic lesions.
...
PMID:History of the infantile hepatic hemangioma: From imaging to generating a differential diagnosis. 2761 Mar 42