Mesenchymal Hamartoma Of Liver Research Articles

Mesenchymal Hamartoma of the Liver Associated with Discontinuity of the Vena Cava

Primary liver tumors in children are infrequent, accounting for 0.5-2 % of all pediatric neoplasms. About two-thirds are malignant. The most common pediatric malignant liver tumours are, in order of frequency, hepatoblastoma, hepatocellular carcinoma, and embryonic sarcoma of the liver. Among benign tumors, the most frequent are hemangioendothelioma and mesenchymal hamartoma of the liver, and more rarely focal nodular hyperplasia and hepatocellular adenoma [1,2]. We report the case of an infant who was entrusted to us for the management of a large liver tumor. Radiology (CT scan and MRI), in addition to the elements in favor of a mesenchymal hamartoma of the right liver revealed a congenital anomaly associated, in this case, with the absence of the inferior vena cava in its intraabdominal portion. A right hepatectomy enlarged to segment IV was performed. The right renal vein drained into the right suprahepatic vein was preserved. Note that this tumor represents 50% of the total weight of the patient, which explains the difficulties and constraints that hindered the development and quality of life of the young patient.

Case Report of Undifferentiated Hepatic Embryonal Sarcoma with Mesenchymal Hamartoma: A Rare Entity

AbstractUndifferentiated embryonal sarcoma (UES) is a highly malignant hepatic neoplasm, which occurs mostly in pediatric population. There is a link between embryonal sarcoma and mesenchymal hamartoma as evidenced by clinicopathological overlap and similar genetic abnormality. Here, we report a case of UES in a young female in a background of mesenchymal hamartoma of liver.

Mesenchymal Hamartoma Liver Showing Malignant Transformation to Undifferentiated Embryonal Sarcoma- A Case Report

Mesenchymal hamartoma of liver is the third most common hepatic tumor in pediatric age group. Typically, it presents as a large multicystic mass in children. Gradually they get increases in size and some can undergo incomplete spontaneous regression and very rarely, few have shown malignant transformation to undifferentiated(embryonal) sarcoma. Here, we report a 6 year old female child with no relevant personal and family history, who presented with abdominal distension and vomiting. Radiology revealed a multiloculated large cystic lesion in the right lobe of liver with a possibility of hemangioendothelioma on ultrasonography and CT abdomen was suggestive of mesenchymal hamartoma of liver. Following worsening of symptoms, partial excision and marsupialisation of the cyst was performed. Histologically, it was diagnosed as mesenchynal hamartoma with undifferentiated embryonal sarcomatous component and immunohistochemical confirmation done.

Adult mesenchymal hamartoma of liver: A case report and discussion of associated molecular abnormalities

Adult mesenchymal hamartoma of liver: A case report and discussion of associated molecular abnormalities

Mesenchymal Hamartoma of Liver in a Neonate

Introduction: Mesenchymal hamartoma of liver (MHL) is the second most common benign liver tumor in children. This tumor typically presents as a large benign cystic mass, solid or mix in children less than three years. Prenatally, it may grow quickly to an abnormal size that may lead to death or hydrops. Prenatal suspicion arises by increasing maternal serum α-FP or HCG and polyhydroamnious and can usually identify the case in the last trimester of pregnancy by ultrasound. Case presentation: The patient was a sick 19 day old baby boy. The patient was admitted to the hospital due to the poor feeding, restlessness and nausea. On physical examination, a mass was found on the left side of the abdomen. In the MRI of the abdomen, the heterogeneous multi-lobulated mass was reported in the upper quadrant of the abdomen. Incision and drainage was done and purulent material was drained from a large abscess located in the left lobe of the liver. Finally, the pathology report showed that the infected mesengymal hamartoma of the liver. Conclusion: In neonatal period MHL can lead to emergency surgery due to fluid or puss accumulation in the cysts. Complete surgical removal is the preferred treatment and histopathological diagnosis helps.

Long-term follow up of mesenchymal hamartoma of liver- Single center study

Background/Aim:Mesenchymal hamartoma of liver (MHL) is a rare liver tumor of childhood. About 200 cases have been reported till now. Most of the work on MHL is limited to case reports and there are not many long term follow-up studies. We present our 20 years of experience with this uncommon entity. This study aims to highlight clinical features, diagnosis and treatment of MHL.Materials and Methods:All patients with a diagnosis of MHL in last 20 years were included in this retrospective study. The patients were evaluated clinically, radiologically and pathologically.ResultsThe total number of patients with a diagnosis of MHL was nine. Mean age of the patients was 19.89 ± 2.75 months. Right lobe was involved in eight patients. The prominent clinical features were distension of abdomen and anorexia. Surgical options used were hepatic lobectomy, wedge resection and enucleation. Histopathology of the specimens showed cysts of variable size with normal hepatocytes, bile ducts and connective tissue stroma. Overall mortality was one (11.11%).Conclusion:MHL is a benign tumor that can present with various clinical features. It should be differentiated carefully from other liver masses especially malignant ones. The diagnosis can be made with the help of radiology and histopathology. Adequate resection is curative in most of the cases and long-term follow up is satisfactory.

Prenatal Diagnosis of Congenital Mesenchymal Hamartoma of Liver: A Case Report

Hepatic mesenchymal hamartoma is a rare benign tumor. We present an unusual case of a fetal abdominal cyst, later diagnosed histopathologically to be mesenchymal hamartoma of liver. The organ of origin was indeterminate on both prenatal and postnatal ultrasounds. As there are no specific sonological findings, whenever a large multicystic fetal abdominal cyst is seen, mesenchymal hamartoma should be considered as a possibility.

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.

Mesenchymal hamartoma of liver: Magnetic resonance imaging and histopathologic correlation

To describe the imaging features of hepatic mesenchymal hamartoma with emphasis on magnetic resonance imaging (MRI) compared to histopathologic results. Spin-echo sequence(SE),fast spin-echo sequence(FSE) were performed in 12 children (7 males,5 females) with mesenchymal hamartoma of the liver (MHL), aged 1.2 months to 12 years;( mean age, 6.3 years) axial, saggital, coronary plain imaging with an Elscint 2.0T MR equipment. Their main symptoms were abdominal mass (5 cases), enlarged liver (8 cases), abdominal pain (1 case) and anemia (2 cases), and negative alphafetoprotein. Dynamic enhancement examination was added in 2 cases. Six cases had single mass type of MHL, in which 3 cases had solid masses showing slight low-signal-intensity in T1WI, and irregular high-signal-intensity in T2WI, 1 case had a cystic-solid mixed mass showing several border-clear cysts in a solid mass, 2 cases had cystic masses with multi-septa. Five cases had diffuse and multifocal lesions type of MHL with its signal intensity being similar to that of the solid mass. One case had a combined diffuse and single cystic mass. In the early dynamic enhancement examination, the lesions were slightly circum-enhanced , and the center was enhanced in the later scan images. Inner hepatic vessels were compressed in 5 cases, vena cava and abdominal aortae were compressed in 3 cases. Pathological findings included fiber hyperplasia, hyaline degeneration, biliary duct hyperplasia, lobule-like array. MR imaging is a better way to differentiate and diagnose MHL. MHL may be recognized by its characteristic occurrence in infancy and MR imaging features.

Chromosomal translocation in mesenchymal hamartoma of liver: what is its significance?

Mesenchymal hamartoma of the liver is generally considered to be benign in nature, and surgical excision has been the treatment of choice. There have been 3 reports on the association of cytogenetic abnormalities with this tumor, and the possibility of malignant potential has been raised. The authors report a fourth case with a previously unreported complex chromosomal translocation between chromosomes 11, 17, and 19 at bands q12, p11, and q13.3, respectively in all cells examined and discuss the significance of this finding.

Mesenchymal hamartoma of liver in a man: comparison with cases in infants.

A 53 year old man with a large mesenchymal hamartoma is reported. Only a few bile ducts could be found in the periphery of the lesion and no hepatocytes were identified within the lesion. As far as is known, this is the only adult male patient reported to date. On the basis of the reported findings of mesenchymal hamartoma in other adults, it is suggested that there could be changes in the morphology of this lesion with age: progressive loss of hepatocytes; degeneration of bile duct epithelium; and cystic change of the mesenchymal component. The haematopoietic element is considered to be part of the fetal hepatic haematopoiesis that occurs in the hamartoma.

Mesenchymal Hamartoma of Liver

To evaluate histologic and gross features of mesenchymal hamartoma of liver and similar lesions in relation to determining likely pathogenesis. Case series of patients presenting to a tertiary care hospital over 16 years. Three children with mesenchymal hamartoma ranging from newborn to 11 months of age and one 12-year-old girl with torsion of an accessory lobe of liver. Similarity of gross vascular and segmented anomalies as well as apparent consequent histologic features of mesenchymal hamartoma to those of torsion of an accessory lobe of liver indicate that they are pathogenetically related. Dissecting microscopic examination revealed a single vascular supply in one case and remote thrombi in two cases of mesenchymal hamartoma evaluated. The histologic features of mesenchymal hamartoma (hypocellular central zone and hypercellular periphery) were duplicated in the lobe of liver with torsion. Mesenchymal hamartoma represents a lesion with an anomalous solitary vascular supply that may evolve into its specific pattern with stromal cysts as a result of early ischemic changes.