Hepatocellular Adenomas and Carcinoma in Asymptomatic, Non- Cirrhotic Type III Glycogen Storage Disease
| Autor: | Leendert H. Oterdoom, Evelyne K. Verweij, Katharina Biermann, Mirjam Langeveld, Henk R. van Buuren |
|---|---|
| Přispěvatelé: | Gastroenterology & Hepatology, Pathology, Internal Medicine |
| Rok vydání: | 2015 |
| Předmět: |
Male
Reoperation Oncology congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Carcinoma Hepatocellular Cirrhosis Adenoma Biopsy medicine.medical_treatment Liver transplantation Glycogen storage disease type III Gastroenterology Adenoma Liver Cell Glycogen Storage Disease Type III Internal medicine medicine Hepatectomy Humans Glycogen storage disease business.industry Liver Neoplasms nutritional and metabolic diseases Middle Aged Hepatocellular adenoma medicine.disease digestive system diseases Liver Transplantation Hepatocellular carcinoma Catheter Ablation Neoplasm Recurrence Local business |
| Zdroj: | Journal of Gastrointestinal and Liver Diseases, 24(4), 515-518. Romanian Society of Gastroenterology |
| ISSN: | 1842-1121 1841-8724 |
| DOI: | 10.15403/jgld.2014.1121.244.had |
| Popis: | Glycogen storage diseases (GSDs) are a group of inherited metabolic disorders characterized by accumulation of abnormal glycogen in muscle or liver or both. Specific hepatic complications include liver adenomas and hepatocellular carcinoma (HCC). Hepatocellular carcinomas described in GSD type I are often due to the degeneration of liver adenomas. Hepatocellular carcinoma in GSD type III, however, is rare and is thought to be associated with underlying cirrhosis.We present the case of a 63-year old male who was admitted for assessment of suitability for liver transplantation because of development of recurrent HCC in the presence of multiple liver adenomas. A diagnosis of GSD type III was made in this patient without underlying cirrhosis or metabolic disturbances resembling GSD. This case report is the first documentation of HCC development in an asymptomatic, non-cirrhotic patient with GSD type III. This raises the possibility that in GSD type III, the adenoma – carcinoma sequence can occur as it is also seen in GSD type I. Physicians taking care of GSD patients should be aware of this and some form of surveillance for cirrhosis and HCC should be considered. Also male patients with adenomas should have a thorough workup to reveal any underlying disease such as GSD. Abbreviations: GSD: glycogen storage disease; HCC: hepatocellular carcinoma; RFA: radiofrequency ablation. |
| Databáze: | OpenAIRE |
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