Mesenchymal Hamartoma Of The Liver Research Articles

Outcomes of Primary Tumor Excision do not Differ from Non-Resection Methods in Pediatric Mesenchymal Hamartoma of the Liver: A Rapid Systematic Review

BackgroundTo conduct a rapid systematic review comparing the outcomes of primary tumor resection versus non-resection on patients with hepatic mesenchymal hamartoma. MethodsWe searched the Ovid MEDLINE, EMBASE, Scopus, PubMed, Web of Science, and Google Scholar databases from January 1, 2000 to March 31, 2022. Studies that described cases of hepatic mesenchymal hamartoma, including management and outcomes, were included. Results62 articles met inclusion criteria with 95 cases in total. Patients were assigned to the primary tumor resection (n = 85) or non-resection (n = 10) cohort based on the described management. Similar rates of morbidity (17% vs 20%) and mortality (6 vs 10%) were identified between cohorts. There were no differences in sex, liver lobe involvement, or tumor size among study groups. ConclusionsComplete tumor resection should remain the gold standard for hepatic mesenchymal hamartomas when an adequate liver remnant exists. Reports of non-resected tumors demonstrating spontaneous regression or stability with watchful waiting have poor long term follow-up and have inadequate evidence of a true mesenchymal hamartoma diagnosis. Level of EvidenceLevel I.

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.

Mesenchymal Hamartoma of the Liver Incidentally Discovered in a 4 Year Old Girl: Management and Treatment

Abstract Mesenchymal hamartomas of the liver is considered a benign tumour, although it provides significant challenges in the diagnosis and operative management. Clinical features, laboratory results and radiographic imaging are often non-specific and inconclusive. Thus, it is still difficult to differentiate from malignant lesion without pathological examination. While this tumour is usually diagnosed in the first 2 years of life, few cases in older children have been reported and most of them are case reports. In this article, we want to report our clinical evaluation, results of image diagnosis, surgical procedure and the follow-up simultaneously we discuss the possible differential diagnosis of such a case at this age.

Clinicopathological analysis of mesenchymal hamartoma of the liver

Clinicopathological analysis of mesenchymal hamartoma of the liver

Fetal mesenchymal hamartoma of the liver: A case report and literature review.

This paper reports the diagnosis and treatment process of a case of fetal mesenchymal hamartoma of the liver (MHL), and reviews the previous literature reports. At 38+2 weeks of gestation, prenatal ultrasound found a well bound mixed solid and cystic mass, which was located at the lower edge of the right lobe of the liver and in front of the right kidney of the fetus, but the source and nature of the mass were not clear by ultrasound. Due to the approaching due date, the fetus showed no other abnormal symptoms, and no special treatment was given with the consent of the family members. A female fetus was delivered weighing 3,520 g at 39 weeks. An exploratory laparotomy was performed on the eighth day after delivery. During the operation, it was found that the tumor originated from the fifth, sixth and seventh hepatic segment and the corresponding hepatic segments were removed. Recovery was uneventful and the infant was discharged on the 6th day after surgery. Follow-up at 2 years showed a thriving young girl, and there was no tumor recurrence.

Mesenchymal Hamartoma of the Liver: About A Case

Mesenchymal hamartoma of the liver is a rare benign tumor, diagnosed mainly during the first two years of life. Its pathogenesis is poorly understood. Imaging plays an essential role in diagnosis and post-therapeutic follow-up. The diagnosis of certainty remains histological and the treatment is essentially surgical. We report a case of hepatic mesenchymal hamartoma diagnosed in a one-year-old infant with isolated abdominal distension. The diagnosis was evoked on imaging and confirmed after histological examination.

Undifferentiated embryonal sarcoma of liver arising within mesenchymal hamartoma in a child "case report"

BackgroundPrimary pediatric liver tumors are scarce. Undifferentiated embryonal sarcoma of the liver (UESL) embraces nine to fifteen percent of the pediatric hepatic neoplasms. Mesenchymal hamartoma of the liver (MHL) is the second most prevalent benign pediatric hepatic neoplasm following infantile hemangioendothelioma.Case presentationA 6-year-old female child presented with vague diffuse abdominal pain and palpable right lumbar mass by clinical examination. The US showed a well-circumscribed large right hepatic complex multilocular cystic lesion with multiple internal septations and some turbid loculi. The constellation of CT and dynamic MRI findings raised the presence of atypical suspicious complex cystic hepatic mass lesion with hemorrhagic, calcific entities and internal soft tissue enhancement. The histopathological correlation proved UESL on the background of MHL.ConclusionsA case of a pediatric hepatic complex cystic lesion, which had typical ultrasound features of MHL, however, CT and MRI had atypical suspicious criteria. Finally, our case is presented as UESL in the background of MHL based upon pathological diagnosis. The radiologists must be familiar with overlapped entities between MHL, UESL, and other differential diagnosis entities regarding pediatric hepatic neoplasms.

18F-FDG PET/CT findings of mesenchymal hamartoma of the liver in an adolescent.

18F-FDG PET/CT findings of mesenchymal hamartoma of the liver in an adolescent.

Mesenchymal Hamartoma of the Liver in Two Older Children and a Review of Pediatric Cases in Japan

Mesenchymal Hamartoma of the Liver in Two Older Children and a Review of Pediatric Cases in Japan

Giant biliary mucinous cystadenoma mimicking mesenchymal hamartoma of the liver in a child: A case report.

IntroductionBiliary cystadenomas (BCAs) are rare benign tumors with malignant potential. They are most commonly found in middle-aged women and are quite infrequently reported in children. Even with advanced imaging, diagnosing and distinguishing BCA from other cystic liver lesions remain challenging.Case presentationA 5-year-old boy was brought in by his parents to our hospital with abdominal swelling that had been persistent for a year, along with loss of appetite and weight loss. On examination, the abdomen was distended and dull on percussion. We considered mesenchymal hamartoma of the liver (MHL) as the top differential after an abdominal CT scan with contrast showed a multi-loculated cystic tumour. For both definitive diagnosis and therapy, the patient underwent exploratory laparotomy with excision of the cystic mass. Surprisingly, histopathology examination of the resected specimen revealed biliary mucinous cystadenoma (BCA).ConclusionSince conservative methods are associated with high recurrence rates, biliary mucinous cystic neoplasms require a high index of suspicion and should be handled with total surgical resection. In the post-operative phase, periodic surveillance imaging is recommended due to the risk of recurrence and malignant transformation.

The Cystic Hepatic Mesenchymal Hamartoma in an Adult Mistaken for Hydatid Disease: Case Report

: Hydatid cyst is one of the predominantly cystic liver lesions and is also a zoonotic disease that is highly endemic in the Mediterranean region. This condition has extensive differential diagnoses, such as hepatic mesenchymal hamartoma, a rare benign hepatic tumor, which commonly occurs in infants and children, and is extremely rare in adults. Its imaging findings vary from a cystic tumor to a solid mass occupying one or both lobes of the liver. We report an unusual case of a purely cystic mesenchymal hamartoma of the liver in a 60-year-old female patient. The patient had been treated with albendazole for several years; however, the final histopathology revealed mesenchymal hamartoma.

Mesenchymal hamartoma of the liver

A case report of a 7-year-old girl who presented with a 4-year history of painless progressive abdominal swelling that became painful 2 weeks before presentation. Examination revealed a girl in painful distress with distended abdomen and a tender palpable firm right upper quadrant abdominal mass measuring 28 cm × 22 cm with well-defined borders. Abdominal ultrasound scan showed a cystic liver mass filling the abdomen which was multilocular. Liver function tests show mildly elevated liver enzymes and mild prolongation of the prothrombin time. She had repeated aspiration of the cyst content in various peripheral hospitals with transient relief of symptoms. She thereafter had abdominal exploration through a right upper transverse incision and was found to have a cystic mass involving segments V, VI, and VII of the right lobe of the liver measuring 30 cm × 26 cm. Marsupialization of the cyst was done and an incisional biopsy of the cyst wall was taken. Histology of the cyst wall showed a mesenchymal hamartoma of the liver (MHL). The aim of this study is to highlight the clinical features, diagnosis, treatment, and differential diagnosis of MHL

Mesenchymal Hamartoma of the Liver and Spleen-A Rare Pseudotumour in Adults

Hepatic mesenchymal hamartoma is the third most common liver neoplasm of the pediatric age group, rarely diagnosed in adults, with under 50 cases having been reported so far. Mesenchymal hamartoma of the spleen is a very rare vascular lesion, documented in less than 200 cases since 1861 when it was first described by Karl von Rokitansky. 1.1. Aim: The aim of this paper is to discuss the differential diagnosis of these tumour-like lesions and the association between them, especially considering their rarity. 1.2. Methods: We present a case of multifocal mesenchymal hamartoma of the liver and spleen in a 51-year-old female patient. The hepatic tumour appears as a macro nodular lesion involving the interface between segments V and VIII [30x28mm], showing central scarring upon MRI imaging and suggesting the diagnosis of focal nodular hyperplasia. Moreover, a sub capsular splenic cystic mass was also revealed, involving the sub diaphragmatic surface of the organ [15x10mm]. 1.3. Results: The histological examination of the hepatic tumour revealed a myxoid stromal proliferation, composed of stellate and fibroblastoid cells, bile ducts with inconspicuous lumina, small vascular structures and chronic non-specific inflammatory infiltrate. On microscopic examination, the splenic lesion exhibited nodular collagenous fibrosis, radial septa and large hyalinized areas. 1.4. Conclusion: A key aspect in the effective treatment of patients diagnosed with mesenchymal hamartomas is surgery with negative margins. The peculiarity of this case consisted of the incidental finding of multiple liver tumours, the incidental finding of the splenic mass, and the rarity of this particular association in adults.

Pediatric fine-needle aspiration cytology: An audit of 266 cases of pediatric tumors with cytologic-histologic correlation.

Objectives:Fine-needle aspiration cytology (FNAC), a well-accepted minimally invasive diagnostic technique utilized in adults, is gradually gaining ground for pediatric patients as well. However, there are very few comprehensive reports in the literature on utility of FNA in pre-operative diagnosis of pediatric tumors.Material and Methods:An observational study was conducted at a cancer research center and a pediatric tertiary care hospital over a 5-year period. A cytologic-histologic correlation was performed for FNACs performed in pediatric patients for a clinical diagnosis of neoplastic lesions at both the centers. Relevant clinical details and histopathology, wherever available, were retrieved. Sensitivity, specificity, and accuracy of FNAC in diagnosis of malignant lesion were calculated from the cases with available histologic correlation.Results:Of the 266 cases included, there was a slight male predominance with lymphadenopathy being the most common presentation and non-Hodgkin’s lymphoma as the most frequent diagnosis in cases clinically suspected to have a neoplasm. Histologic correlation was available in 112 cases with 100% concordance in liver and kidney tumors. Few rare cytologic diagnoses such as papillary renal cell carcinoma, mesenchymal hamartoma of the liver, and thymolipoma could be accurately rendered on FNAC smears in conjunction with the clinic-radiologic features. The sensitivity, specificity, and accuracy of FNA in diagnosing malignant pediatric tumors were found to be 100%, 92.6%, and 97.7%, respectively.Conclusion:The present study underscores the high sensitivity and accuracy of FNAC in diagnosis of pediatric tumors, both in superficial and deep-seated locations. Awareness of the cytomorphologic features and clinic-radiologic correlation may assist the cytopathologists in rendering a precise diagnosis of rare pediatric tumors as well.

Giant solid mesenchymal hamartoma of the liver in a neonate: case report

BackgroundMesenchymal hamartoma is the second most common benign liver tumor in children, with 20% of the cases diagnosed during the neonatal period. The exact etiology in still unclear, and most investigators believe that it is a developmental anomaly rather than a true neoplasm. The presentation of these tumors is highly variable depending on the lesion’s size, ranging from small asymptomatic lesions to very large tumors with life threatening complications. Radical surgical excision, whenever possible, is the gold standard for treating these lesions to avoid the problems of local recurrence and possible malignant transformation. We present the rare occurrence of an entirely solid, giant hepatic mesenchymal hamartoma in a 3-week-old male newborn and discuss the mode of presentation, as well as the diagnostic and therapeutic approach.Case presentationA 3-week-old male newborn was referred to our institution with huge abdominal distension and respiratory distress. Imaging studies confirmed the presence of a very large solid intraabdominal mass occupying the majority of the abdominal cavity and abutting the inferior aspect of the right lobe of the liver, but did not reveal the diagnosis. At laparotomy, a huge solid mass was found attached to the right lobe of the liver. Complete excision was done, and histopathological examination confirmed the diagnosis of mesenchymal hamartoma.ConclusionAlthough rare, mesenchymal hamartoma of the liver can present as a neonatal surgical emergency. Emergency intervention is required in symptomatic patients. Radical surgical intervention is possible and is the treatment of choice to relieve the patient’s symptoms and avoid future complications.

Central segments liver resection for pediatric tumors

Aim. To analyze the short-term outcomes of сentral segments liver resection in children, according to the initial experience.Мethods. The data of patients with different centrally located liver neoplasms were analyzed. This retrospective study included three patients with hepatoblastoma, one observation of mesenchymal hamartoma of the liver and one patient with hepatocellular carcinoma. Different types of central liver resections were carried out in these patients between March and October 2018. The technical aspects and the short-term results are described.Results. Median age of patients was 78 months (9 months – 12 years). Patients underwent: segmentectomy 4b, bisegmentectomy 4b, 5, threesegmentectomy 4, 5, 6, mesohepatectomy (anatomical segmentectomy 4, 5, 8) with biliary reconstruction for posterior liver segments, mesohepatectomy with anatomical segmentectomy 6 and atypical S2, 3 resection. Dindo–Clavien grade IIIa postoperative complication developed in one patient. Vascular complications and posthepatectomy liver failure were absent. The hospital stay ranged between 9 to 14 days. The median follow-up period came to 8 months (7–14 months). The overall and event free survival at the time of writing is 100%.Conclusion. Resections of central liver segments in children are feasible and should be carried out in experiences in liver surgery centers.

A Case of Infantile Mesenchymal Hamartoma of the Liver Excised After a Series of Sclerotherapy With OK-432

A Case of Infantile Mesenchymal Hamartoma of the Liver Excised After a Series of Sclerotherapy With OK-432

Mesenchymal hamartoma of the liver extending through a localized chest wall defect

Abstract Mesenchymal hamartoma of the liver (MHL), a common benign hepatic tumor in children, is not commonly associated with other congenital malformations. We describe a novel case of MHL extending through a localized chest wall defect. A 15-day-old Han female presented with a red, staghorn-shaped skin mass overlying the left chest wall. Ultrasound, CT and MRI showed masses in the left chest wall and liver that appeared connected. Laparoscopy identified a mass on the left lateral liver lobe that extended through an intercostal space to connect with the mass outside the left chest wall. The masses were surgically resected. Pathology indicated MHL with a localized chest wall defect. There were no complications or recurrence.

Mesenchymal Hamartoma of the Liver in Older Children

Mesenchymal hamartoma of the liver (MHL) is an uncommon benign hepatic tumor typically affecting children under 2 years of age. Only 5% of MHL occur after 5 years and are very rarely observed in adults. According to age, MHL may differ in their morphologic features. We report a case of an 11-year-old boy with MHL, resembling a malignant lesion from a clinical point of view, characterized by unusual histologic features: a prominent myxoid stroma, with a minimal ductular component, and absent cystic spaces. The present case and others reported in older children or adults demonstrate that these lesions may represent a potential diagnostic pitfall when occurring outside their classic clinical context especially because of their peculiar histologic findings. Moreover, it may be hypothesized that variation in morphology might be related to different evolutive stages of the cell of origin. To support this hypothesis, we therefore studied the presence of components of the Notch pathway inside and outside the lesion. Their absence inside the tumor and, in contrast, the expression of Notch2 and HES1 evident in overrepresented bile ducts present at the periphery might explain not only the lack of bile ducts, but also indicate a more adult phenotype compared with classic pediatric MHL, which show more bile ducts and liver trabeculae embedded in the mesenchymal matrix.

Mesenchymal Hamartoma of the Liver: Case Report and Review of the Literature

Mesenchymal Hamartoma of the Liver: Case Report and Review of the Literature