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To conduct a rapid systematic review comparing the outcomes of primary tumor resection versus non-resection on patients with hepatic mesenchymal hamartoma. We 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. 62 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. Complete 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 I. • Hepatic mesenchymal hamartoma is a common benign liver mass in pediatric patients with varied surgical management described in the literature. • Complete tumor resection should remain the gold standard with a recommendation against watchful waiting, regardless of mass size. [ABSTRACT FROM AUTHOR]

Abstract Background Hepatic mesenchymal hamartoma (HMH) is the second most common benign hepatic tumor in the pediatric age group. The etiology of HMH is unclear. Theories include abnormal hepatic mesenchyme, a reactive change or a proliferative lesion. The condition can present prenatally in USG as a mass, high output cardiac failure, pulmonary hypertension, respiratory distress, and abdominal distension or mass in older children. We present this case to discuss an extremely rare presentation of a comparatively rare disease and to emphasise the need for a high index of suspicion even if the patient presents with symptoms common for that age. Case presentation A 7-day-old male baby with an uneventful antenatal period, born by vaginal delivery, with a birth weight of 3.1 kg and normal APGAR scores, presented with jaundice, poor feeding, and labored breathing noticed on the same day itself. Examination revealed a grossly distended abdomen with a mass of size 8 × 7 cm occupying the right side down to the right iliac fossa, crossing midline and dullness continuous with liver dullness. On investigation an abnormal liver function test with unconjugated hyperbilirubinemia was seen. USG and CECT abdomen were suggestive of HMH with close proximity to hepatic vessels. Exploratory laparotomy and near total excision with marsupialisation was done. Histopathology report was suggestive of HMH. A 2-year follow-up was normal. Conclusion This case of HMH presented as neonatal jaundice with unconjugated hyperbilirubinemia which is a rarity. We had to limit our surgery to near total excision and marsupialisation due to its proximity to vascular structures.

Background: Hepatic mesenchymal hamartoma (HMH) is the second most common benign hepatic tumor in the pediatric age group. The etiology of HMH is unclear. Theories include abnormal hepatic mesenchyme, a reactive change or a proliferative lesion. The condition can present prenatally in USG as a mass, high output cardiac failure, pulmonary hypertension, respiratory distress, and abdominal distension or mass in older children. We present this case to discuss an extremely rare presentation of a comparatively rare disease and to emphasise the need for a high index of suspicion even if the patient presents with symptoms common for that age. Case presentation: A 7-day-old male baby with an uneventful antenatal period, born by vaginal delivery, with a birth weight of 3.1 kg and normal APGAR scores, presented with jaundice, poor feeding, and labored breathing noticed on the same day itself. Examination revealed a grossly distended abdomen with a mass of size 8 × 7 cm occupying the right side down to the right iliac fossa, crossing midline and dullness continuous with liver dullness. On investigation an abnormal liver function test with unconjugated hyperbilirubinemia was seen. USG and CECT abdomen were suggestive of HMH with close proximity to hepatic vessels. Exploratory laparotomy and near total excision with marsupialisation was done. Histopathology report was suggestive of HMH. A 2-year follow-up was normal. Conclusion: This case of HMH presented as neonatal jaundice with unconjugated hyperbilirubinemia which is a rarity. We had to limit our surgery to near total excision and marsupialisation due to its proximity to vascular structures. [ABSTRACT FROM AUTHOR]

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.

Abstract Background Although infantile hepatic hemangioma and hepatic mesenchymal hamartoma are relatively common in benign pediatric liver tumors, coexistence of the two tumors is rare. Placental mesenchymal dysplasia is also a rare disorder. We report the case of a baby girl born after a pregnancy complicated by placental mesenchymal dysplasia, who developed both infantile hepatic hemangioma and hepatic mesenchymal hamartoma. Case presentation The patient was born at 32 weeks and 5 days of gestation for impending placental abruption, weighing 1450 g. Liver tumors, composed of both hypervascular solid and large cystic lesions, were detected after birth and markedly increased to create abdominal distention within 9 months. Diagnostic imaging suspected the coexistence of infantile hepatic hemangioma and cystic hepatic mesenchymal hamartoma. Following propranolol therapy for infantile hepatic hemangioma and needle puncture of a large cyst, the cystic lesions and adjacent hypervascular lesions were partially resected via laparotomy. Pathological findings confirmed the coexistence of hepatic mesenchymal hamartoma and infantile hepatic hemangioma, which had no association with androgenetic/biparental mosaicism. The postoperative course was uneventful, and the tumor had not regrown after 3 years. Conclusions Although the coexistence of infantile hepatic hemangioma and hepatic mesenchymal hamartoma associated with placental mesenchymal dysplasia is extremely rare, the pathological and pathogenetic similarities between these disorders suggest that they could have derived from similar embryologic origins rather than being a mere coincidence. Further follow-up is required, with careful attention to the potential for malignant hepatic mesenchymal hamartoma transformation.

Liver tumors can be divided into benign and malignant types. Benign liver tumors are relatively common, which are mainly treated with follow-up observation and interventional therapy, and surgical resection should be performed for few cases. Liver transplantation can effectively treat end-stage liver diseases, whereas it is seldom applied in the treatment of benign liver tumors. Liver transplantation is only considered for benign liver tumors with huge space-occupying compression, liver failure, the risk of tumor rupture and bleeding or malignant transformation. Compared with malignant tumors and chronic liver failure, liver transplantation yields an equivalent risk for benign liver tumors, favorable clinical prognosis and long-term survival. However, comprehensive evaluation should be delivered before liver transplantation for benign liver tumors, and extensive attention should be paid to the difficulty of donor liver matching in the new situation. In this article, research progress on liver transplantation for benign liver tumors including hepatic hemangioma, polycystic liver, hepatocellular adenoma, hepatic mesenchymal hamartoma was reviewed, aiming to provide reference for liver transplantation in the treatment of benign liver tumors. [ABSTRACT FROM AUTHOR]

Background: Although infantile hepatic hemangioma and hepatic mesenchymal hamartoma are relatively common in benign pediatric liver tumors, coexistence of the two tumors is rare. Placental mesenchymal dysplasia is also a rare disorder. We report the case of a baby girl born after a pregnancy complicated by placental mesenchymal dysplasia, who developed both infantile hepatic hemangioma and hepatic mesenchymal hamartoma. Case presentation: The patient was born at 32 weeks and 5 days of gestation for impending placental abruption, weighing 1450 g. Liver tumors, composed of both hypervascular solid and large cystic lesions, were detected after birth and markedly increased to create abdominal distention within 9 months. Diagnostic imaging suspected the coexistence of infantile hepatic hemangioma and cystic hepatic mesenchymal hamartoma. Following propranolol therapy for infantile hepatic hemangioma and needle puncture of a large cyst, the cystic lesions and adjacent hypervascular lesions were partially resected via laparotomy. Pathological findings confirmed the coexistence of hepatic mesenchymal hamartoma and infantile hepatic hemangioma, which had no association with androgenetic/biparental mosaicism. The postoperative course was uneventful, and the tumor had not regrown after 3 years. Conclusions: Although the coexistence of infantile hepatic hemangioma and hepatic mesenchymal hamartoma associated with placental mesenchymal dysplasia is extremely rare, the pathological and pathogenetic similarities between these disorders suggest that they could have derived from similar embryologic origins rather than being a mere coincidence. Further follow-up is required, with careful attention to the potential for malignant hepatic mesenchymal hamartoma transformation. [ABSTRACT FROM AUTHOR]

Hepatic mesenchymal hamartomas (HMH) are benign liver lesions that present as cystic, slow growing masses within the first few years of life. Few cases demonstrate placental abnormalities and abnormal levels of alpha-fetoprotein (AFP). We report an unusual case of a rapidly growing HMH with associated placental anomalies and elevated AFP. A 2-month-old female born prematurely due to concerning placental findings presented with a prenatally-identified liver mass. Magnetic resonance imaging (MRI) showed a marked increase in the size of the mass over a three-month period. Prior to surgery, the AFP was elevated and decreased after operative resection. Histopathology confirmed the diagnosis HMH. Rapid growth of HMH is not commonly reported and raises concerns for malignancy. Abnormal placental findings may lead to prematurity, which can affect surgical management. We identified 27 cases of prenatally diagnosed HMH since 1983. The mean gestational age for identification was 29 weeks, with the majority managed by delayed resection. The timing for surgical intervention lacks consensus, but malnutrition and perioperative complications should be considered. High AFP levels with an infantile hepatic mass may indicate a more malignant process but does not exclude HMH.

Abstract Background Undifferentiated embryonal sarcoma of liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare entities which mainly affect the pediatric population. The aim of this investigation was to provide a comprehensive overview of the clinicopathologic characteristics of the patients diagnosed with these two conditions in a tertiary referral center in Iran. Methods In this retrospective study patients diagnosed with UESL or HMH between 2012 and 2020 were studied. A comprehensive histopathologic evaluation of the cases along with immunohistochemistry evaluation using a panel of antibodies was conducted. Furthermore, clinical, paraclinical, and treatment data and follow up information was collected. Results A total of 16 patients (8 UESL, 8 HMH) were studied in this investigation. Patients with UESL had a significantly (p = 0.002) higher age at diagnosis compared with those with HMH. Histologically, UESL cases were characterized by anaplastic cells with eosinophilic cytoplasm and bizarre nuclei and frequent atypical mitosis and spindling in a myxoid stroma while disordered arrangement of hepatic parenchyma, bile ducts, and primitive mesenchyme was seen in HMH. Furthermore, small round cells and extramedullary hematopoiesis were seen in 2 UESL and 3 HMH cases, respectively. Concurrent HMH was also seen in two UESL cases. Immunohistochemistry panel showed positive staining for Vimentin, Glypican-3, Desmin, CD56, CD10, and BCL2 in UESL cases and immunoreactivity for Vimentin, HepPar 1, Glypican-3, SMA, CD56, BCL2, and CD34 in various components of HMH. Conclusions In this study, the clinicopathologic features of UESL and HMH cases are presented. We also evaluated the utility of an immunohistochemistry panel in the diagnosis of these two rare entities and suggested novel markers. Our study corroborated the findings of previous investigations and expanded the clinicopathologic features of these two rare entities with diagnostic and potential therapeutic implications.

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Hepatic mesenchymal hamartoma is an uncommon tumor composed of myxoid mesenchymal tissue with fluid accumulation. Here, we report a case of hepatic mesenchymal hamartoma in a 3-year-old patient who was referred to the hospital with abdominal pain and a slight fever. An abdominal ultrasound suggested a liver abscess; however, computed tomography revealed a mass with both cystic and solid components. Liver biopsy confirmed hepatic mesenchymal hamartoma, and this patient underwent the complete removal of this tumor.

Background: Undifferentiated embryonal sarcoma of liver (UESL) and hepatic mesenchymal hamartoma (HMH) are two rare entities which mainly affect the pediatric population. The aim of this investigation was to provide a comprehensive overview of the clinicopathologic characteristics of the patients diagnosed with these two conditions in a tertiary referral center in Iran. Methods: In this retrospective study patients diagnosed with UESL or HMH between 2012 and 2020 were studied. A comprehensive histopathologic evaluation of the cases along with immunohistochemistry evaluation using a panel of antibodies was conducted. Furthermore, clinical, paraclinical, and treatment data and follow up information was collected. Results: A total of 16 patients (8 UESL, 8 HMH) were studied in this investigation. Patients with UESL had a significantly (p = 0.002) higher age at diagnosis compared with those with HMH. Histologically, UESL cases were characterized by anaplastic cells with eosinophilic cytoplasm and bizarre nuclei and frequent atypical mitosis and spindling in a myxoid stroma while disordered arrangement of hepatic parenchyma, bile ducts, and primitive mesenchyme was seen in HMH. Furthermore, small round cells and extramedullary hematopoiesis were seen in 2 UESL and 3 HMH cases, respectively. Concurrent HMH was also seen in two UESL cases. Immunohistochemistry panel showed positive staining for Vimentin, Glypican-3, Desmin, CD56, CD10, and BCL2 in UESL cases and immunoreactivity for Vimentin, HepPar 1, Glypican-3, SMA, CD56, BCL2, and CD34 in various components of HMH. Conclusions: In this study, the clinicopathologic features of UESL and HMH cases are presented. We also evaluated the utility of an immunohistochemistry panel in the diagnosis of these two rare entities and suggested novel markers. Our study corroborated the findings of previous investigations and expanded the clinicopathologic features of these two rare entities with diagnostic and potential therapeutic implications. [ABSTRACT FROM AUTHOR]

Hepatic mesenchymal hamartoma is a rare hepatic tumour seen in the paediatric population. Although this entity has a variable imaging appearance, it has a favourable prognosis if diagnosed and managed correctly. This case presents the ultrasound and computed tomography findings of an 11-month-old patient who presented with a history of progressive abdominal distension and an elevated alfa fetoprotein level on biochemistry. The case describes how a confident perioperative diagnosis could be made on the basis of characteristic imaging features.

This review highlights two rare entities that are predominantly seen in children: hepatic mesenchymal hamartoma (HMH) and undifferentiated embryonal sarcoma of the liver (UESL). HMH is a benign lesion predominantly seen in the first 2 years of life, while UESL is malignant and usually identified in patients between 6 and 10 years of age. UESL may arise in the background of HMH, and the association has been supported by similar chromosomal aberrations (19q13.4). The diagnosis of both lesions is primarily based on histologic evaluation, as the clinical and radiological features are not always typical. The clinicopathologic characteristics, pathogenesis, differential diagnoses and treatment for both lesions are discussed.

Hepatic mesenchymal hamartoma is a rare hepatic tumour seen in the paediatric population. Although this entity has a variable imaging appearance, it has a favourable prognosis if diagnosed and managed correctly. This case presents the ultrasound and computed tomography findings of an 11-month-old patient who presented with a history of progressive abdominal distension and an elevated alfa fetoprotein level on biochemistry. The case describes how a confident perioperative diagnosis could be made on the basis of characteristic imaging features. [ABSTRACT FROM AUTHOR]

Hepatic mesenchymal hamartomas (HMH) are benign liver lesions that present as cystic, slow growing masses within the first few years of life. Few cases demonstrate placental abnormalities and abnormal levels of alpha-fetoprotein (AFP). We report an unusual case of a rapidly growing HMH with associated placental anomalies and elevated AFP. A 2-month-old female born prematurely due to concerning placental findings presented with a prenatally-identified liver mass. Magnetic resonance imaging (MRI) showed a marked increase in the size of the mass over a three-month period. Prior to surgery, the AFP was elevated and decreased after operative resection. Histopathology confirmed the diagnosis HMH. Rapid growth of HMH is not commonly reported and raises concerns for malignancy. Abnormal placental findings may lead to prematurity, which can affect surgical management. We identified 27 cases of prenatally diagnosed HMH since 1983. The mean gestational age for identification was 29 weeks, with the majority managed by delayed resection. The timing for surgical intervention lacks consensus, but malnutrition and perioperative complications should be considered. High AFP levels with an infantile hepatic mass may indicate a more malignant process but does not exclude HMH. • Consider hepatic mesenchymal hamartomas in a rapidly growing pediatric liver tumor. • Prenatally-identified cases of HMH may be associated with placental abnormalities. • Elevated alpha-fetoprotein levels should not preclude benign liver tumors like HMH. [ABSTRACT FROM AUTHOR]

Background: Benign liver tumors are common. They do not spread to other areas of the body, and they usually do not pose a serious health risk. In fact, in most cases, benign liver tumors are not diagnosed because patients are asymptomatic. When they are detected, it's usually because the person has had medical imaging tests, such as an ultrasound (US), computed tomography (CT) scan, or magnetic resonance imaging (MRI), for another condition. Materials and methods: A search of the literature was made using cancer literature and the PubMed, Scopus, and Web of Science (WOS) database for the following keywords: 'hepatic benign tumors', 'hepatic cystic tumors', 'polycystic liver disease', 'liver macroregenerative nodules', 'hepatic mesenchymal hamartoma', 'hepatic angiomyolipoma', 'biliary cystadenoma', and 'nodular regenerative hyperplasia'. Discussion and conclusion: Hepatocellular carcinoma (HCC) is one of the most common malignant tumors in some areas of the world; there is an increasing incidence worldwide. Approximately 750,000 new cases are reported per year. More than 75 % of cases occur in the Asia-Pacific region, largely in association with chronic hepatitis B virus (HBV) infection. The incidence of HCC is increasing in the USA and Europe because of the increased incidence of hepatitis C virus (HCV) infection. Unlike the liver HCC, benign tumors are less frequent. However, they represent a chapter always more interesting of liver disease. In fact, a careful differential diagnosis with the forms of malignant tumor is often required in such a way so as to direct the patient to the correct therapy. In conclusion, many of these tumors present with typical features in various imaging studies. On occasions, biopsies are required, and/or surgical removal is needed. In the majority of cases of benign hepatic tumors, no treatment is indicated. The main indication for treatment is the presence of significant clinical symptoms or suspicion of malignancy or fear of malignant transformation. [ABSTRACT FROM AUTHOR]

Hepatic mesenchymal hamartoma is a rare benign tumor in children, and infantile hepatic hemangioendothelioma is also a rare liver neoplasm. We report a female newborn with an abdominal mass noted by the regular maternal ultrasound at 32 weeks of gestation. After birth, a liver mass was detected by computed tomography and magnetic resonance cholangiopancreatography. Frequent postprandial vomiting and progressive abdominal distension occurred 4 months later. Three tumor masses were detected this time, and the serum alpha-fetoprotein (AFP) was 6700 ng/mL. Segmental resection was performed initially and complete resection of these tumors and left lobectomy were performed 21 days later. Pathologic examination of these liver masses revealed mesenchymal hamartoma combined with infantile hepatic hemangioendothelioma. After half a year of regular follow-up, the AFP level decreased gradually to 79.5 ng/mL, without evidence of tumor recurrence. [J Formos Med Assoc 2007;106(3 Suppl):S1-S4]

The incidence of hepatic mesenchymal hamartoma (HMH) is increased in patients with placental mesenchymal dysplasia (PMD), which appears to be caused by androgenetic-biparental mosaicism (ABM). We hypothesized that occult ABM might underlie cases of HMH with no known history of PMD. Formalin-fixed, paraffin-embedded HMH specimens from 10 such patients and liver specimens from 6 non-HMH controls were identified retrospectively from the surgical pathology records of a pediatric hospital. The relative abundance of maternal and paternal alleles was assessed by quantitative polymerase chain reaction amplification of polymorphic short tandem repeats and single nucleotide polymorphisms located on 15 different chromosomes. Androgenetic-biparental mosaicism was diagnosed in one patient based on global allelic imbalances at all informative loci. In that patient, the greatest imbalances were observed in stroma-rich portions of the hamartoma, with no significant imbalance in histologically normal liver or epithelium-rich portions of the hamartoma. A retrospective, unbiased review of the histology and clinical records from all 10 patients revealed no morphologic or clinical correlates to distinguish the affected patient, except that she had multiple cutaneous hemangiomas, which like HMH, appear to be more common in patients with PMD. Our findings suggest that other patients with apparently sporadic HMH, hemangioma, or other lesions seen more frequently with PMD may harbor occult ABM. Recognition of ABM may be important because its long-term consequences are unknown but may be significant. [ABSTRACT FROM AUTHOR]

This report describes a 31-week stillborn female infant with placental mesen-chymal dysplasia (PMD) in association with hepatic mesenchymal hamartoma (HMH) and pulmonary hamartoma. Placental mesenchymal dysplasia was initially misdiagnosed as a partial mole. However, histologically, no trophoblastic proliferation or inclusions were observed. Differential diagnosis of the hepatic mass with similar tumors is discussed. To our knowledge, this is the first case of lung hamartoma reported in a fetus and the first case related to PMD and HMH. A common anomalous development of the mesoderm, a reparative post-injury process and a genetic mechanism, have been proposed to explain their pathogenesis. [ABSTRACT FROM AUTHOR]

Placental mesenchymal dysplasia ( PMD) is a rare condition characterised by placental enlargement, oedematous villi and multiple anechoic cysts. Hepatic mesenchymal hamartoma ( HMH) is a benign proliferation of mesenchymal tissue, commonly seen in infants below the age of 2. We report the case of a 28 years old female who was noted to have a fetus with a well-circumscribed cyst on the liver, suggestive of HMH and a large, thickened placenta, with multiple anechoic cysts, consistent with PMD during the third trimester. There were no other structural abnormalities and at 38 weeks she underwent an induction of labour with normal vaginal delivery of a live female infant. While the aetiology is poorly understood, the increased incidence of HMH with PMD and the morphological similarities of the changes seen in both the placenta and liver, suggests a possible common developmental mechanism. There are only 12 other cases of this concurrent pathology in the literature and only one of these had resulted in a term delivery, and ours is the second one to date. [ABSTRACT FROM AUTHOR]

We here present a 6-month-old girl with cystic hepatic mesenchymal hamartoma and elevated a-fetoprotein (aFP). Following hepatectomy of the left lobe and partial right lobectomy, decline of the serum aFP was observed. The child has been well for 20 years and it is one among a few cases with such a long observational period. [ABSTRACT FROM AUTHOR]

Hepatic mesenchymal hamartoma (HMH) is a rare benign liver tumor generally occurring in young children. Abnormalities of chromosome 19q13.4 and chromosomal aneuploidy detected by flow cytometry have been reported in HMH. We recently analyzed a HMH in a 14-month-old girl and identified additional novel chromosome abnormalities, including hypodiploidy with deletions of several chromosomes, without rearrangement of 19q13.4 on both routine chromosome analysis and fluorescent in-situ hybridization analysis using subtelomere probes for chromosome 19q. This case underscores the need for chromosome analysis of all HMH and the need to report these changes to better understand the pathophysiology and the entire spectrum of cytogenetics of this uncommon tumor. [ABSTRACT FROM AUTHOR]

This review highlights two rare entities that are predominantly seen in children: hepatic mesenchymal hamartoma (HMH) and undifferentiated embryonal sarcoma of the liver (UESL). HMH is a benign lesion predominantly seen in the first 2 years of life, while UESL is malignant and usually identified in patients between 6 and 10 years of age. UESL may arise in the background of HMH, and the association has been supported by similar chromosomal aberrations (19q13.4). The diagnosis of both lesions is primarily based on histologic evaluation, as the clinical and radiological features are not always typical. The clinicopathologic characteristics, pathogenesis, differential diagnoses and treatment for both lesions are discussed., Competing Interests: Financial & competing interests disclosure The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. No writing assistance was utilized in the production of this manuscript., (© 2020 Juan Putra.)

A case of massive mesenchymal hamartoma of the liver is described in a 13-month-old infant. A complex multicystic mass was demonstrated by ultrasound and computed tomography (CT). The appearances suggested the preoperative diagnosis. [ABSTRACT FROM AUTHOR]