Hepatic Hamartoma Research Articles

Arterial embolization in the treatment of multiple renal and hepatic hamartomas with spontaneous hemorrhage and 2-year follow-up: a case report

BackgroundHamartoma is a common benign tumor that usually occurs in the kidney, liver, lung, and pancreas. Large renal hamartomas may spontaneously rupture and hemorrhage, which is potentially life-threatening.Case presentationThis report describes a 46-year-old Han Chinese female patient with multiple renal and hepatic hamartomas with rupture and hemorrhage of giant hamartoma in the left kidney. She underwent arterial embolization three times successively, and her condition was stable during the 2-year follow-up. This report includes a review of the relevant literatureConclusionsthe findings in this report and previous literature suggest that arterial embolization can not only rapidly treat hamartoma hemorrhage in the acute phase but can also effectively control multiple lesions in the long term after repeated multisite arterial embolization.

Patient-Specific 3D Printed Soft Models for Liver Surgical Planning and Hands-On Training

Background: Pre-surgical simulation-based training with three-dimensional (3D) models has been intensively developed in complex surgeries in recent years. This is also the case in liver surgery, although with fewer reported examples. The simulation-based training with 3D models represents an alternative to current surgical simulation methods based on animal or ex vivo models or virtual reality (VR), showing reported advantages, which makes the development of realistic 3D-printed models an option. This work presents an innovative, low-cost approach for producing patient-specific 3D anatomical models for hands-on simulation and training. Methods: The article reports three paediatric cases presenting complex liver tumours that were transferred to a major paediatric referral centre for treatment: hepatoblastoma, hepatic hamartoma and biliary tract rhabdomyosarcoma. The complete process of the additively manufactured liver tumour simulators is described, and the different steps for the correct development of each case are explained: (1) medical image acquisition; (2) segmentation; (3) 3D printing; (4) quality control/validation; and (5) cost. A digital workflow for liver cancer surgical planning is proposed. Results: Three hepatic surgeries were planned, with 3D simulators built using 3D printing and silicone moulding techniques. The 3D physical models showed highly accurate replications of the actual condition. Additionally, they proved to be more cost-effective in comparison with other models. Conclusions: It is demonstrated that it is possible to manufacture accurate and cost-effective 3D-printed soft surgical planning simulators for treating liver cancer. The 3D models allowed for proper pre-surgical planning and simulation training in the three cases reported, making it a valuable aid for surgeons.

Cystic hepatic hamartoma causing pseudo-prune belly in a female infant in Tamale, Ghana

Cystic hepatic hamartoma causing pseudo-prune belly in a female infant in Tamale, Ghana

Clinical Presentation and Anatopathologic Finding of a Hepatic Vascular Hamartoma: a Case Report.

HVH (hepatic vascular hamartoma) is a tumor like malformation arising from the vascular tissue of the liver. HVH has been previously reported in animals and presents distintive features from the most frequent benign tumor like malformation of the liver, the hepatic mesenchymal hamartoma (HMH). Herein we report a case of HVH localized in hepatic segment 4b, involving the gastro hepatic ligament, successfully treated with total excision. We describe the anatomo-pathologic findings focusing on the clinical and radiological presentation, the intraoperative characteristics and the differential diagnosis.

Cystic intra-abdominal masses in children.

Benign intra-abdominal cystic masses in infancy are fairly uncommon and their etiopathogenesis, histology and clinical presentation differ significantly. Our aim is to report our experience in their treatment in order to discuss the best diagnostic and treatment modality. The medical records of 5 children (2M, 3F) with cystic intraabdominal masses referred to our hospital between November 2012 and September 2016, were retrospectively reviewed. All patients underwent open surgery and subsequent histopathologic analysis. Different clinical presentations, localizations of the masses, diagnostic tools, surgical approaches, histological examinations and outcomes were reviewed. Patients mean age was 5.4 years (range: 8 months-9 years). Two patients presented recurrent abdominal pain and abdominal distension; 1 patient had a palpable mass discovered incidentally and 2 complained acute abdominal pain. Routine laboratory tests, tumor markers and abdominal ultrasound were immediately done in all patients. Three patients underwent MRI and 1 abdominal CT. At laparotomy 2 hepatic cysts, 2 mesenteric cyst and 1 retroperitoneal cyst were discovered. Histology reports described: 1 hepatobiliary cystadenoma, 1 benign hepatic hamartoma and 3 cystic lymphangiomas (1 retroperitoneal and 2 mesenteric). There were no major postoperative complications, deaths, or recurrences in our series (follow-up 3-24 months). Despite the rarity of these lesions, benign cystic abdominal masses in children are not so uncommon and should be considered as causes of acute abdominal pain. The differential diagnosis is not always possible preoperatively. In our series, radical excision of the lesions was possible in all cases, allowing reliable histological results and avoiding recurrences.

Mesenchymal hepatic hamartoma associated with elevated alpha fetoprotein mimicking a hepatoblastoma: A rare case and a literature review

Introduction: A mesenchymal hepatic hamartoma is a heterogeneous and a rare congenital benign neoplasm whose presentation may be cystic, solid or mixed and the mass may demonstrate varying degrees of vascularity. Treating mesenchymal hepatic hamartomas conservatively is not encouraged because these lesions have the potential to grow rapidly and may degenerate to a malignant undifferentiated embryonal sarcoma. The standard treatment of mesenchymal hepatic hamartoma is surgical resection. However, marsupialization is also indicated in surgically unresectable cases. Case Report: We report a case of one-year-old girl who presented with a one-month history of abdominal distention. The patient was born through a normal vaginal delivery at term. Apgar score 9/10-10/10, birth weight 2.8 kg. Head circumference 36 cm. The patient is retro-viral disease negative. She has normal developmental milestones and the immunizations are up to date. Ultrasound of the abdomen was performed and it showed right liver lobe large masses. Computed tomography (CT) scan was subsequently performed to further characterize the liver lesions. Ultrasound guided biopsy was performed and it confirmed the diagnosis of mesenchymal hepatic hamartoma. The alpha-fetoprotein was markedly elevated. Conclusion: It is of utmost importance to note that as much as the mesenchymal hepatic hamartoma typically shows a normal alpha-fetoprotein, a rare presentation of this benign tumor may be associated with markedly elevated alpha fetoprotein.

Hepatic angiomyolipoma: clinical, imaging and pathological features in 178 cases

The aim of the study was to review the main clinical, imaging and pathological features of hepatic angiomyolipoma (HAML). We retrospectively analyzed the imaging, pathological and clinical features of 178 patients who underwent surgical resection for HAML. Forty-three males and 135 females with a median age of 43.5 years (range: 17-76 years) were enrolled in the study. Routine blood tests, including those for α-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen determinant, were normal. Liver function was classified as Child-Pugh A for all patients. Twenty-one patients were positive for hepatitis B surface antigen (HBsAg), while two had liver cirrhosis. Seventy-five lesions were located in the right hepatic lobe, 42 in the left hepatic lobe, 12 in the hepatic caudal lobe, and 49 in both left and right lobe. All patients underwent examination by ultrasound sonography (US), and additional examinations by computed tomography (CT) and magnetic resonance (MR) were carried out for 115 and 81 patients, respectively. Various diseases including HAML, liver cancer, liver hemangioma, hepatic lipoma, hepatic hamartoma, focal nodular hyperplasia and liver adenoma were diagnosed before operation. CT and MRI are more conclusive than US (US vs CT: χ(2) = 17.72, P = 0.00; US vs MRI: χ(2) = 12.14, P = 0.00), but for the sensitivity of these three imaging modalities, MRI performs the best (US vs MRI: χ(2) = 5.938, P = 0.015; CT vs MRI: χ(2) = 2.544, P = 0.111). All the patients underwent liver resection (96 right hepatic lobectomy, 41 left hepatic lobectomy and nine caudal lobe hepatic lobectomy). Several sampled tissues were positive for HBsAg (21/168, 12.5 %), β-catenin (4/41, 9.8 %), CD-34 (119/165, 72.1 %), HMB-45 (162/169, 92.9 %), SMA (145/158, 91.8 %) and vimentin (20/27, 74.1 %). The diagnosis of HAML remains difficult and mainly relies on pathological findings. For the imaging modalities, we recommend MRI examination because of its better definition and sensitivity. Although HMB-45 is considered the main histopathological biomarker for HAML, SMA may be also helpful in the diagnostic process (HMB-45 vs SMA: χ(2) = 2.37, P = 0.123). When the diagnosis of HAML is suspicious or hypothesized, surgical resection remains the recommended strategy due to the difficulty in diagnosis preoperatively and the potentially invasive growth of the lesion.

A preterm infant with anaemia and left leg mild hemihypertrophy (Discussion and Diagnosis)

The Case Presentation can be found on page 1192. DISCUSSION The clinical course suggested placental mesenchymal dysplasia (PMD) that was confirmed by pathological examination of the placenta. Genetic test confirmed Beckwith– Wiedemann syndrome (BWS, paternal uniparental disomy). First described in 1991 by Moscoso et al. (1), PMD is characterized by placentomegaly with multicystic ‘grapelike vesicles’, similar to a partial mole at ultrasound scan and macroscopic examinations. There is a diffuse hydropic degeneration of chorionic villi and a absence of trophoblastic proliferation (1,2). In contrast to a hydatidiform mole, PMD may coexist with a viable foetus. Final diagnosis of PMD is made by histologic examination of the placenta. Most cases of PMD in early pregnancy are diagnosed by prenatal ultrasonography done for routine prenatal checkup, although it has been associated with pre-eclampsia (3,4). The incidence of PMD is unknown. A Japanese study estimated an incidence of 0.02% of placentas examined (3,5). Recently, a Canadian review (6) with more than 95 000 placentas examined, found two cases, 0.002 ⁄%.There is a marked female predominance in PMD (3), but this is much less apparent in cases of BWS which has a ratio of 1.6 female to male compared to PMD without BWS 4.33:1. Foetal morbidity and mortality are more frequent (3), as IUGR, intrauterine foetal demise, polyhydramnios, prematurity, BWS (20–30% of cases) and anaemia–thrombocytopenia (7) secondary to placental haemolysis. It has also been associated with 13 trisomy, other mesenchymal tumours, as hepatic, vascular or pulmonary hamartomas (8), serous cystadenoma of pancreas and congenital adrenal hyperplasia, suggesting a common defect in placental and embryonic mesoderm. Pham et al. (3). stated that among all cases without BWS, 50% had IUGR and 43% had intrauterine foetal demise or neonatal death. IUGR (9) and intrauterine foetal demise are explained by hypoxia secondary to vascular thrombosis and reduced gas exchange area. Foetuses with BWS had normal weight percentiles. Aetiology of PMD remains unclear. Hypoxia and hypoperfusion may give rise to the phenotypic findings in the placenta with PMD as during hypoxia, fibroblasts are stimulated to produce increased connective tissue fibres with subsequent increased production of vascular endothelial growth factor by villous macrophages leading to angiogenesis (10). Genetically, most PMD are diploid, while 70–80% of partial moles are triploid. Some cases of PMD have been associated to androgenetic ⁄biparental mosaicism (11,12), with the hypothesis that biparent cell line gives rise to normal structures (foetus, amnion and trophoblast) and androgenetic cell line would result in the chorion and mesenchymal villi with the characteristic changes of the DMP. Such mosaicism arose as a result of failure of the maternal genome to duplicate before the first cleavage with normal duplication and segregation of the paternal genome, resulting in two daughter cells, one with normal biparental genes and another with only paternal genes that became ‘diploid’ through endoreduplication. The association with BWS could be explained because androgenetic cells express a biallelic paternal IGF2 gene from 11p15.5. Studies have shown a female predominance in cases of PMD, and it is speculated that there is a relationship with the endothelial growth factor gene deletions in the X-chromosome (5,13,14). Acta Paediatrica ISSN 0803–5253

Prenatal imaging and postnatal pathologic work‐up in a case of fetal hepatic hamartoma and placental mesenchymal dysplasia

Prenatal imaging and postnatal pathologic work‐up in a case of fetal hepatic hamartoma and placental mesenchymal dysplasia

Hepatic nodular hamartoma containing liver cysts, ductal plate malformations and peribiliary glands

An 83-year-old man with hepatocellular carcinoma was found to have a low-echoic and low-density tumor measuring 7.2 cm × 5.6 cm. Caroli's disease was absent. Clinical diagnosis was intrahepatic cholangiocarcinoma. Three cores of liver biopsy were obtained from the tumor. Histologically, it consisted of liver cysts, ductal plate malformations, peribiliary glands, hepatocytes, portal tracts and mesenchymal tissue. Apparent features of cirrhosis were not found. The liver cysts were lined by a layer of cuboidal cells with multiple papillary protrusions. The ductal plate malformations resembled fetal ductal plates. The peribiliary glands were seromucous glands. Immunohistochemically, these abnormal ductal structures showed positive reaction to biliary type cytokeratins, namely, cytokeratin (CK)7, CK8, CK18 and CK19. Mucin gene expression showed that these biliary structures are positive for fetal antigen MUC1. MUC6 is also positive in them. Aberrant expression of CD10 was observed in these biliary structures. MUC2, MUC5AC and CDX2 were negative. The author thinks that this lesion is hepatic hamartoma with ductal plate malformations, liver cysts and peribiliary glands. To the best of the author's knowledge, this type of liver nodule has not been reported in the published work.

Surgical removal of a localised vascular hepatic hamartoma in a dog

A 2-year-old male Labrador Retriever was presented for sudden abdominal distension. Abdominal ultrasonography revealed abundant abdominal fluid and a hepatic mass, which was removed by total lobectomy. Histologic evaluation of the mass supported a vascular hepatic hamartoma. Vascular hamartomas are rare malformations in animals and only two cases have been reported in the dog. This is the first recorded case of a canine vascular hepatic hamartoma treated surgically and having a successful long-term outcome.

In this issue – September 2009

In this issue – September 2009

Torsed pedunculated hepatic hamartoma

We report a 9-year-old boy with a 6-h history of acute abdominal pain due to torsion of a pedunculated hepatic mesenchymal hamartoma. The lesion was seen, on US and CT, to connect to the liver through a pedicle. Mesenchymal hepatic hamartomas are unusual tumours that may be pedunculated, and this is a unique case complicated by torsion. The radiological and pathological findings, differential diagnosis, and clinical course are discussed.

Hepatic angiosarcoma arising in an adult mesenchymal hamartoma

The histogenesis of the hepatic sarcoma and its association with hamartoma is not well understood. We hereby present a Chinese patient with hepatic angiosarcoma arising from an adult mesenchymal hamartoma of liver. A 33-yr-old woman was diagnosed hepatic hamartoma eight years ago and presented with epigastric distention recently. Now she was admitted to our hospital with some unusual features: (a) this patient was diagnosed in mid-twenties, (b) the tumor occupied the whole liver and most importantly (c) the hepatic angiosarcoma appeared 8 years after the diagnosis of hamartoma. Based on this case and some reports, hepatic hamartoma may develop to hepatic angiosarcoma.

Liver hamartomas in tuberous sclerosis

Three patients with tuberous sclerosis (TS) and hepatic hamartomas are presented. Liver lesions are uncommonly documented in TS, compared to renal angiomyolipomas. The imaging characteristics of liver hamartoma in TS may resemble those of renal angiomyolipoma, hepatic cavernous haemangiomas, metastasis or abscess.

Incidence of hepatic hamartomas in tuberous sclerosis.

Hepatic hamartomas were thought to be a rare finding in patients with tuberous sclerosis. The purpose of this study was to assess their incidence in children with tuberous sclerosis and to review the literature. During 1984-90 we examined 51 children by ultrasonography; there were 25 boys and 26 girls. Their age ranged from 3 months to 18 years. Liver hamartomas were seen in 12 (23.5%) of the children, more often in girls than boys (5:1). Their incidence increased with age reaching 45% in children over the age of 10. They did not produce any symptoms of hepatic dysfunction. Our study and review of reported cases prove that hepatic hamartomas are a common finding in patients with tuberous sclerosis and may be very helpful in providing a more accurate diagnosis and consequently help in genetic counselling.

AN UNUSUAL CASE OF SOLID HAMARTOMA IN THE LIVER

An unusual case of solid hepatic hamartoma, which could not be correctly categorized under the WHO classification, is reported. The well defined, solid nodular lesion, arising in the anterior edge of the left hepatic lobe, was found in a 15-month-old female infant. The lesion was composed of irregularly shaped yellow islands of parenchyma and white-gray nodules of fibrous stroma of various sizes, which were intermingled revealing a mosaic pattern. Prominent bile ductal proliferation was observed histologically in the fibrous stroma, associated in parts with dense or loose periductal bundles and vascular proliferation. The stromal proliferation seemed to result in erosion of the islands of differentiated parenchymal cells with no regeneration. Alpha-fetoprotein was demonstrated immunohistologically in the parenchymal cells of the lesion. From these findings, the essential component of this solid hamartoma was thought to be markedly proliferating bile ducts associated with periductal fibrosis, resulting in a fibroadenomatous pattern.

Case report: The computed tomographic appearances of benign hepatic hamartoma

The case of a 32-year-old female with asymptomatic hepatomegaly due to a large cystic liver mass is described. Pathological examination after total excision revealed a benign hepatic hamartoma. Benign hepatic hamartoma is a rare cause of a cystic liver mass, usually occurring below the age of 5 years. The computed tomography findings included 'daughter cysts' and it is emphasised that these must not be regarded as pathognomonic of hydatid disease.

The thymus in congenital heart disease.

Some cases of thymus developmental defects (DiGeorge's syndrome) are associated with the development of defects of the heart and great vessels. To see if anomalies of the heart are also associated with anomalies of the thymus, a material of thymus tissue removed during operation for congenital heart disease was compared with thymus tissue from a forensic autopsy material representing the same age group, but without cardiac lesions. Among 27 cases of congenital heart disease, occurrence in the thymus of solid epithelial cords were seen in 6 and cyst- or duct-like structures were found in 7. Among 47 forensic cases, 1 showed cords and another showed duct-like structures. Of these, one had congenital anomalies in the form of hepatic hamartomas and nesidioblastosis, whereas the other had died from morbilli, raising the possibility of congenital immune deficiency. Anyhow, a striking occurrence of minor morphological deviations in the structure of the thymus was found in children with congenital heart disease. Such material therefore may be less suitable for studying the morphology and biology of the normal thymus gland.

Tumorigenic aspects.

This report presents a review of tumors, except those of pituitary, that have been reported to occur in women taking combined oral contraceptive preparations. Pathologic features, both gross and microscopic, and differential diagnosis are emphasized. Particular attention is given to tumors of the liver: focal nodular hyperplasia (hepatic hamartoma) and liver cell adenoma (benign hepatoma). The characteristic features of these usually distinctive lesions are illustrated, and an attempt is made to evaluate the significance of each with respect to oral contraceptives. Tumorigenic aspects relating to the uterus and the breast are briefly discussed.