1. Exuberant liver adenomatosis presenting with iron deficiency anemia
- Author
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Válter R. Fonseca, Marisa Teixeira Silva, Rui M. M. Victorino, Ana Margarida Carvalho, and Catarina Mota
- Subjects
medicine.medical_specialty ,Case Report ,Disease ,Case Reports ,Gastroenterology ,030218 nuclear medicine & medical imaging ,Malignant transformation ,Pathogenesis ,03 medical and health sciences ,0302 clinical medicine ,Germline mutation ,Internal medicine ,Diabetes mellitus ,medicine ,Glycogen storage disease ,iron deficiency anemia ,business.industry ,intratumoral hemorrhage ,General Medicine ,Hepatocellular adenoma ,medicine.disease ,Endocrinology ,Iron-deficiency anemia ,030211 gastroenterology & hepatology ,business ,liver adenomatosis - Abstract
Hepatocellular adenoma is an uncommon benign hepatic tumor, with an increased incidence in women of reproductive age with a history of oral contraceptives 1, 2, men taking anabolic steroids 3, or patients with glycogen storage disease or iron‐overload disorders 4, 5. The majority of hepatic adenomas are solitary, although the occasional occurrence of two or three adenomas is well recognized in the literature 6, 7. Liver adenomatosis, defined by the presence of multiple adenomas (arbitrarily, defined by more than 10) in a normal hepatic parenchyma, was described in 1985 by Flejou et al. as a distinct entity 8. Subsequently, case reports and small series have documented multiple adenomas in an otherwise normal liver, both in men and women, in the absence of glycogen storage disease or association with steroid medication 9, 10, 11, 12. Nevertheless, the role of estrogen intake in the outcome and progression of liver adenomatosis is still controversial, in contrast to solitary adenomas where the association with estrogens is well established 13. The pathogenesis and natural history of liver adenomatosis are still unclear. Some reports show that hepatic adenomatosis occurs more often in patients who have coexistent vascular tumors, portal vein absence, or occlusion or portohepatic venous shunts, leading to a speculative association between this entity and congenital or acquired abnormalities of the hepatic vasculature 14, 15, 16. Germline mutation of hepatocyte nuclear factor‐1 alpha, which is associated with maturity‐onset diabetes of the young type 3, has been also recently implicated in some reported cases 17, 18. Although considered a benign entity, liver adenomatosis has been associated with an increased risk of malignant transformation and hemorrhage, both potentially fatal 8, 13. Complications of adenomatous lesions, such as intraperitoneal bleeding, intratumoral hemorrhage, or necrosis‐producing acute pain, often reveal a frequently silent disease 8, 9, 19, 20, 21, 22. We describe a rare case of massive and multifocal liver adenomatosis, who presented clinically as a chronic iron deficiency anemia due to chronic intratumoral bleeding that illustrates an unreported presentation of this condition.
- Published
- 2017