Dataset Information

Hepatocellular Carcinoma in a 24-Year-Old Female with Beckwith-Wiedemann Syndrome: A Case Report and Review of the Literature.

ABSTRACT: In this report, the case of a 24-year-old female with Beckwith-Wiedemann Syndrome (BWS) who was diagnosed with well-differentiated hepatocellular carcinoma (HCC) is described. While BWS has been associated with childhood embryonal tumors, most commonly Wilms tumors and hepatoblastomas, this is the first case report to describe HCC in an adult with BWS. Although HCC typically occurs in elderly adults or those with underlying liver disease, in this case, we show that HCC can occur in a young adult with BWS without any underlying liver disease.

SUBMITTER: Ahlers CG

PROVIDER: S-EPMC7563044 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Hepatocellular Carcinoma in a 24-Year-Old Female with Beckwith-Wiedemann Syndrome: A Case Report and Review of the Literature.

Ahlers Carolyn G CG Trinh Quoc-Huy QH Montenovo Martin M

Case reports in genetics 20201007

In this report, the case of a 24-year-old female with Beckwith-Wiedemann Syndrome (BWS) who was diagnosed with well-differentiated hepatocellular carcinoma (HCC) is described. While BWS has been associated with childhood embryonal tumors, most commonly Wilms tumors and hepatoblastomas, this is the first case report to describe HCC in an adult with BWS. Although HCC typically occurs in elderly adults or those with underlying liver disease, in this case, we show that HCC can occur in a young adult ...[more]

PMID: 33083068

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Project description:The International Headache Society (IHS) defines ophthalmoplegic migraine (OM) as recurrent attacks of headache with migrainous characteristics, associated with paresis of one or more ocular cranial nerves (commonly the third cranial nerve), and in the absence of any demonstrable intracranial lesion other than MRI changes within the affected nerve. According to the IHS criteria, it is diagnosed when at least two attacks with migraine-like headaches are accompanied with, or followed within 4 days of onset by, paresis of one or more of the third, fourth or sixth cranial nerves. Parasellar, orbital fissure and posterior fossa lesions should be ruled out by appropriate investigations. It is unlikely that OM is a variant of migraine, since the headache often lasts for a week or more and there is a latent period of up to 4 days from the onset of headache to the onset of ophthalmoplegia. Furthermore, in some cases MRI shows gadolinium uptake in the cisternal part of the affected cranial nerve and this suggests that the condition may be a recurrent demyelinating neuropathy. In general, patients demonstrated a: (1) prolonged time for symptom resolution to occur (median time 3 weeks); (2) tendency for recurrent episodes to have more severe and persistent nerve involvement; (3) evidence of permanent neurological sequelae with recurrent episodes (30% of patients); (4) rapid improvement and shortened duration with corticosteroid therapy and; (5) transient, reversible MRI contrast enhancement of the affected cranial nerve (86% of patients). Different pathogenetic mechanisms, which include compressive, ischemic and inflammatory, have been suggested for OM. Here, a 15-year-old Ethiopian with recurrent attacks of headache and third nerve palsy is presented. The subsequent discussion focuses on current evidences with regard to the clinical characteristics, possible pathogenetic mechanisms and treatment. Finally, a brief discussion of the situation in Africa will be presented.

Dataset Information

Hepatocellular Carcinoma in a 24-Year-Old Female with Beckwith-Wiedemann Syndrome: A Case Report and Review of the Literature.

Publications

Hepatocellular Carcinoma in a 24-Year-Old Female with Beckwith-Wiedemann Syndrome: A Case Report and Review of the Literature.

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