Liver Mesenchymal Hamartoma

Preferred Label : Liver Mesenchymal Hamartoma;

NCIt synonyms : Mesenchymal Hamartoma of Liver; Mesenchymal Hamartoma of the Liver; Liver MH; Hepatic Mesenchymal Hamartoma; HMH;

NCIt definition : A multicystic mesenchymal tumor that arises from the liver during fetal development. It is the third most common pediatric liver tumor following hepatoblastoma and infantile hemangioma. Cytogenetic and molecular studies have revealed the presence of chromosomal rearrangements involving chromosome 19q13.4 or androgenetic-biparental mosaicism. These findings support the evidence that it is a neoplastic process and not only a developmental process. Clinically, it usually presents as an abdominal mass associated with abdominal distention. Morphologically, it consists of hypocellular fibromyxoid stroma and bland spindle cell proliferation. It generally has a benign clinical course. Following resection, the prognosis is good.;

Neoplastic status : Benign;

Détails

Origin ID

C5751;

UMLS CUI

C1333971;

Automatic exact mappings (from CISMeF team)
- Hemifacial myohyperplasia [OMIM Phenotype]
- Hepatic cystic hamartoma [ICD-11 More detail]
- Hepatic cystic hamartoma [ORDO Disease]
- Mesenchymal hamartoma of liver (disorder) [SNOMED CT concept]
Disease excludes abnormal cell
- Malignant Cell [NCIt concept]
Excludes anatomical site(s)
- Extrahepatic Bile Duct [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
- Digestive System [NCIt concept]
- Hepatobiliary System [NCIt concept]
- Liver [NCIt concept]
- Soft Tissue [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Mesenchymal hamartoma of liver (disorder) [SNOMED CT concept]
concept_is_in_subset
- Cellosaurus Disease Terminology [NCIt concept]
disease_excludes_finding
- Malignant Cellular Infiltrate [NCIt concept]
disease_excludes_normal_tissue_origin
- Lymphoid Tissue [NCIt concept]
disease_has_abnormal_cell
- Neoplastic Cell [NCIt concept]
- Neoplastic Connective and Soft Tissue Spindle Cell [NCIt concept]
disease_has_finding
- Benign Cellular Infiltrate [NCIt concept]
- Childhood Lesion [NCIt concept]
- Cystic Lesion [NCIt concept]
- Fibromyxoid Stroma Formation [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- Multilocular Lesion [NCIt concept]
disease_has_primary_anatomic_site
- Connective and Soft Tissue [NCIt concept]
- Digestive System [NCIt concept]
- Liver [NCIt concept]
- Soft Tissue [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Androgenetic-Biparental Mosaicism [NCIt concept]
- Rearrangement of 19q13.4 [NCIt concept]

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