Undifferentiated embryonal sarcoma of liver with macroaneurysms and arteriovenous shunt

Undifferentiated embryonal sarcoma (UES) is an uncommon primary hepatic tumour of childhood. The mass usually shows paradoxical features of being cystic on CT and solid on ultrasound. These lesions are usually hypovascular. Very rarely they may present as hypervascular liver masses with macroaneurysms and arteriovenous (AV) shunt, with only less than six cases reported in literature. We report a case of an 11-year-old child who presented with progressive abdominal distention, and CT revealed a large exophytic hypervascular mass of liver with multiple macroaneurysms, pooling of contrast and a high-flow AV shunt. Histopathology, along with immunohistochemistry, revealed the mass to be UES. The child underwent neoadjuvant chemotherapy followed by successful surgery. The prognosis of this tumour depends on prompt diagnosis and early intervention. We present this case to highlight the atypical presentation of UES, which will encourage radiologists to keep this differential in relevant clinical settings.

Undifferentiated embryonal sarcoma masquerading as liver abscess: A case report with typical imaging features

Undifferentiated embryonal sarcoma (UES) is an uncommon paediatric hepatic tumour that clinically simulates a liver abscess when present with fever. This report describes a case of UES in a 12-year-old boy, who presented with abdominal pain, swelling and fever, all simulating a liver abscess. The possibility of UES was considered at imaging, based on the solid appearance on ultrasound and cystic appearance with serpiginous peripheral vessels on computed tomography/magnetic resonance imaging. The diagnosis was confirmed at histopathology.

Successful multidisciplinary treatment of Doege-Potter syndrome: hypoglycaemia caused by paraneoplastic IGF-2 production by a metastatic haemangiopericytoma

Hypoglycaemia due to insulin-like growth factor (IGF)-2 secretion is a paraneoplastic complication of malignancy with significant morbidity that can often go unrecognised due to its uncommon presentation. We report on a case of a 51-year-old man with metastatic haemangiopericytoma presenting with refractory hypoglycaemia, requiring continuous dextrose 10% infusion while in hospital. IGF-2 levels were significantly elevated, in keeping with a rare entity associated with solitary fibrous tumours, known as Doege-Potter syndrome. The patient was managed using uncooked cornstarch in conjunction with debulking of the hepatic tumour burden with bland IR-guided transarterial embolisation, and eventual surgical resection to treat his non-islet cell tumour hypoglycaemia (NICTH). The case highlights this rare paraneoplastic phenomenon that should be included in the differential for hypoglycaemia, especially if a history of a solitary fibrous tumour is elicited. Our case is the first to document a successful approach to treating the hypoglycaemia using preoperative transarterial bland embolisation.

Imaging after percutaneous thermal and non-thermal ablation of hepatic tumour: normal appearances, progression and complications

The increasing number of liver tumours treated by percutaneous ablation leads all radiologists to be confronted with the difficult interpretation of post-ablation imaging. Radiofrequency and microwave techniques are most commonly used. Recently, irreversible electroporation treatments that do not induce coagulation necrosis but cellular apoptose and respect the collagen architecture of bile ducts and vessels have been introduced and lead to specific post-ablation features and evolution. Ablations cause ‘normal’ changes in ablation and periablation zones. It is necessary to know these post-ablation features to avoid the misinterpretation of recurrence or complication that would lead to unnecessary treatments. Another challenge for the radiologist is to detect as early as possible the residual unablated tumour or the disease progression (local progression and tumour seeding) that will require a new treatment. Finally, the complications, frequent or rarer, should be recognised to be managed adequately. The purpose of this article is therefore to describe the large spectrum of normal and pathological aspects related to the treatment of hepatic tumour by percutaneous thermal ablation and irreversible electroporation ablation.

Infantile hepatic haemangioendothelioma resection in a newborn: A case report and literature review

Infantile hepatic haemangioendothelioma (IHH) is the most common benign hepatic tumour in infants. However, experience of managing IHH is lacking and treatments for symptomatic IHH are controversial. Here we report the case of a patient with IHH treated by liver resection. A liver mass was found in a newborn by prenatal ultrasonography. The patient presented with abdominal distention with a tangible mass. Further imaging diagnosis and biopsy were carried out and complete surgical resection of the mass was performed. Histological examination confirmed IHH. The patient recovered uneventfully after surgery, with no additional therapy after discharge and no recurrence during follow-up. We also summarise previously published resected cases of IHH and review the surgical outcomes. Surgical resection and liver transplantation appear to be effective treatments for symptomatic IHH.