Imaging features of rare mesenychmal liver tumours: beyond haemangiomas

Thampy, Rajesh; Elsayes, Khaled M.; Menias, Christine O.; Pickhardt, Perry J.; Kang, Hyunseon C.; Deshmukh, Sa Ndee P. P; Ahmed, Kareem; Korivi, Brinda Rao

doi:10.1259/bjr.20170373

Cited by 26 publications

(18 citation statements)

References 67 publications

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“…Although the exact etiology is not clear, mesenchymal hamartomas seem to originate from a congenital, localized abnormality in ductal plate development [7]. It is typically composed of loose mesenchyme with variably sized cysts lacking an epithelial lining, accompanied by islands or cords of hepatocytes admixed with bile ducts and blood vessels [8].…”

Section: Discussionmentioning

confidence: 99%

Mesenchymal Hamartoma in Children: A Diagnostic Challenge

Khan

Binkovitz

Smyrk

et al. 2019

Case Reports in Pediatrics

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Mesenchymal hamartoma is a benign tumor of the liver with a poorly understood pathogenesis. It is uncommon in older children, especially after 2 years of age. The signs and symptoms may be nonspecific; therefore, a high index of suspicion is required for diagnosis and treatment. We report a 5-year-old previously healthy male who presented with acute abdominal pain, fatigue, and fever. He was diagnosed with pneumonia initially and treated with antibiotics. A computed tomography (CT) scan done for evaluation of his persistent abdominal pain demonstrated a hepatic mass. Follow-up magnetic resonance imaging (MRI) of the liver demonstrated multiple serpiginous tubular-type structures, read as possible Caroli syndrome. He had a normal abdominal examination and normal biochemistries including alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, alkaline phosphatase, and alpha-fetoprotein. He was referred to our institution for second opinion. On further review of his imaging studies, the lesion was thought to be a mesenchymal hamartoma. He subsequently underwent resection of the mass. Pathology confirmed the diagnosis of mesenchymal hamartoma.

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Section: Discussionmentioning

confidence: 99%

Mesenchymal Hamartoma in Children: A Diagnostic Challenge

Khan

Binkovitz

Smyrk

et al. 2019

Case Reports in Pediatrics

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“…MH is the second-most common hepatic benign tumor in children. Although there are reports of its spontaneous regression, it can potentially progress to an aggressive malignant undifferentiated embryonal sarcoma (UES) (8). The most common presentation is painless abdominal distension.…”

Section: Discussionmentioning

confidence: 99%

A giant primary hepatic low-grade malignant mesenchymal tumor of the liver in a teenager

Zhang

Jia

Yang

et al. 2018

Hepatobiliary Surg. Nutr.

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“…Most cases are sporadic. The diagnosis can be very difficult, even using modern imaging and liver biopsy (6,38,(64)(65)(66). Macroscopically, HHS presents as illdefined spongy haemorrhagic nodule(s) involving the whole liver.…”

Section: Hhsmentioning

confidence: 99%

“…Unlike hepatocellular and cholangiocellular cancers, diagnostic and therapeutic algorithms of vascular tissue neoplasms are far from being standardized (1,2). This uncertainty is explained by the protean clinical, morphological and histopathological presentation and also by the limited awareness of the medical community about this "orphan" disease (defined as a disease occurring in less than 6/pmp) (3)(4)(5)(6)(7)(8)(9). Not surprisingly, many debates concerning the optimal management are on-going.…”

Section: Introductionmentioning

confidence: 99%

Vascular tumours of the liver: a particular story

Lerut

Iesari

2018

Transl. Gastroenterol. Hepatol

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Vascular tumours of the liver represent an underrated chapter of medical and surgical hepatology. These tumours cover a wide spectrum ranging from the frequent and most benign hepatic haemangioma (HH), via the rare and intermediately aggressive hepatic epithelioid haemangioendothelioma (HEHE) to the rare and most malignant hepatic haemangiosarcoma (HHS). In contrast to the treatment algorithms for hepatocellular and cholangiocellular cancer, the diagnostic and therapeutic approaches to HEHE and HHS are not well developed. The related uncertainty is explained by their rare occurrence and their protean clinical, morphological (imaging) and histopathological presentation and behaviour. This article gives an update about these particular tumours based on the analysis of the recent literature and of the studies on vascular tumours published by the European Liver Intestine Transplantation Association (ELITA)-European Liver Transplant Registry (ELTR). It focuses also on the place of liver transplantation (LT) in the respective therapeutic algorithms. The differential diagnosis between these vascular and other tumour types may be very difficult. Correct diagnosis is of utmost importance and is based on a high index of clinical suspicion and on the integration of clinical, radiological, histological [including immunohistochemistry (IHC) and molecular biology findings]. Surgery, be it partial or total hepatectomy (LT), should be proposed whenever possible, because it is the therapeutic mainstay. In HEHE, LT provides excellent results, with long-term diseasefree survivals (DFS) reaching 75%. Good results can be obtained even in case of (frequent) extrahepatic spread. Based on the extensive ELITA-ELTR study a HEHE-LT prognostic score has been proposed in order to estimate the risk of recurrence after LT. In contrast, results of surgery and LT are extremely poor for HHS, for the almost invariably rapid recurrence (within 6 months) and related death within 2 years. LT remains a contraindication for HHS. Due to the still important recurrence rate after surgical resection (25% in HEHE and almost 100% in HHS), there is an urgent need to develop pharmacological treatments targeting angiogenic and non-VEGF angiogenic pathways. To date, some prospective pilot studies and case reports have shown some short-term stabilisation of the disease in small groups of patients. In order to make progress, combination of surgery, anti-angiogenic and immunotherapy seems worthwhile. To complete the panel of vascular liver tumours, infantile haemangioendothelioma, haemangiopericytoma, nodular regenerative hyperplasia (NRH) and hepatic small vessel neoplasms (HSVN) are also discussed.

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Imaging features of rare mesenychmal liver tumours: beyond haemangiomas

Cited by 26 publications

References 67 publications

Mesenchymal Hamartoma in Children: A Diagnostic Challenge

Mesenchymal Hamartoma in Children: A Diagnostic Challenge

A giant primary hepatic low-grade malignant mesenchymal tumor of the liver in a teenager

Vascular tumours of the liver: a particular story

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