Liver tumour of mixed type

Williams, A. Wynn

doi:10.1002/bjs.18004116506

Cited by 25 publications

(4 citation statements)

References 8 publications

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“…38). This feature has been documented in the older literature, first in a 6-year-old girl (Sheehan 1930), in 2.25-year-old boy (Williams (1953), and in 14-year-old boy (Pack and Miller 1956), illustrating that this phenomenon occurs in a wide age range. One case of mixed tumor occurring in 4-year-old girl and containing tumor cells with a typically striated cytoplasm is intriguing insofar as the neoplasm also contained components indistinguishable from fibrosarcoma, a finding that is not known to occur in typical HB-MEM (Watanabe et al 1975).…”

Section: Mixed Epithelial and Mesenchymal Hepatoblastoma (Mem-hb)supporting

confidence: 57%

Hepatoblastoma and the Hepatoblastoma Family of Tumors

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Hepatoblastoma (HB) is a rare malignant blastomatous liver tumor characterized by various combination of epithelial and mesenchymal cell lineages that recapitulate early phases of liver ontogenesis. HB is typically a pediatric liver neoplasm; most cases are diagnosed before the age of 5 years. Very rarely, HB develops in older children and adults. HB accounts for less than 5 % of pediatric malignancies. The tumor occurs more commonly in low birth weight children and is associated with several molecular and cytogenetic abnormalities, mutations in the Wnt/beta-catenin signaling pathway being frequent. Morphologically, HBs are divided into epithelial and mixed epithelialmesenchymal types. Epithelial tumors are broken down into fetal, embryonal, macrotrabecular, and small cell undifferentiated subtypes, while mixed epithelialmesenchymal HBs are grouped into those without or with teratoid features. A subset of small cell undifferentiated HB exhibits rhabdoid features associated with loss of INI1 expression, similar to malignant rhabdoid tumors. Although HBs are aggressive lesions with high recurrence rate and metastatic disease, modern therapies now produce excellent results. In order to improve treatment, molecular profiles are developed to refine risk stratification.

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Section: Mixed Epithelial and Mesenchymal Hepatoblastoma (Mem-hb)supporting

confidence: 57%

Hepatoblastoma and the Hepatoblastoma Family of Tumors

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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“…However, there are also pediatric liver tumors with a rhabdomyoid component that cannot easily be allocated to mixed hepatoblastomas. Williams (1953) described, in 2-year-old boy, a neoplasm which he termed liver tumor of mixed type. The tumor presented as a cystic mass located in the right liver lobe.…”

Section: Other Liver Tumors With a Rhabdomyocytic/ Rhabdomyoblastic Dmentioning

confidence: 99%

“…This RMS is almost always an embryonal RMS. The lesion has first been described in 1875 (Wilks and Moxon 1875) and has later also been described as embryonic tumor with striated muscle (Sheehan 1930), a malignant mixed tumor of the choledochal duct (Goeters 1941), and a cystic liver tumor of the mixed type (Williams 1953). The case of Sheehan (1930), a 6-year-old girl, was described in great detail, and the author already pointed out that this type of tumor may present in the form of intrahepatic cysts representing dilated bile ducts obstructed by the intraluminally growing tumor.…”

Section: Introductionmentioning

confidence: 99%

Tumors of the Striated Muscle Cell Lineage: Hepatobiliary Rhabdomyosarcoma and Rhabdomyoma

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Rhabdomyosarcomas (RMS) are a group of aggressive malignancies derived from a skeletal muscle cell lineage. These neoplasms occur in several distinct body regions in children and adults. One subset of RMA develops in the hepatobiliary tract, mainly in the pediatric age group, associated with a distinct clinical and radiological syndrome. Hepatobiliary RMS is defined as a mucosal-type RMS arising from the wall of intrahepatic or extrahepatic bile ducts. This neoplasm is the most common biliary tract tumor in children, but is overall rare, accounting for only 0.8 % of all RMS and 1.3 % of malignant liver tumors in childhood. The neoplasm can form polypoid masses that protrude into the bile ducts. Histologically, most hepatobiliary RMS are embryonal RMA, whereas alveolar RMS is a very rare hepatic malignancy. Histologically, embryonal RMA of the liver shows a loose neoplastic tissue with desmin-positive stellate cells and cells resembling rhabdomyoblasts. Frequently, a cellular layer is found underneath the biliary tract epithelium, the so-called cambium. RMS of the hepatobiliary tract can rarely also occur in adults.

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“…Mixed liver and bile duct forms also occur as do mixed carcinoma and sarcoma. In children, hepatoblastomas, mixed tumours containing osteoid, and true teratomas are also seen (Froboese, 1952;Milman and Grayzel, 1951;Sheehan, 1930;and Williams, 1953). The maximum incidence is between 45 and 6o years of age (Behrend and Harberg, 1952), but liver tumours are also common in infancy.…”

Section: Primary Malignant Tumoursmentioning

confidence: 99%