Presentation
Mesenchymal hamartoma of the liver is a rare benign hepatic tumor that typically presents in the first 2 years of life. Approximately 85% of affected children present before the age of 3 years and less than 5% present after the age of 5 years. [ncbi.nlm.nih.gov]
We present the case of an elder 87 year old male who presented a 20 cm mesenchymal hamartoma liver cyst. [unboundmedicine.com]
Entire Body System
- Fever
It has a fast-growing rate; the most frequent symptoms are pain and abdominal mass, also jaundice, weight loss, fever, emesis and hepatomegaly. [go.gale.com]
We report a case of a six month–old male infant, who presented with abdominal distension for two month and fever and vomiting for two weeks. The management and etiology of this case is discussed. KEY WORDS: Mesenchymal hamartoma, Liver Tumor. [pjms.com.pk]
Most of the cases remain asymptomatic while the others are detected incidentally when they present with right upper quadrant mass, respiratory distress, fever and raised right hemidiaphragm(2,4). [indianpediatrics.net]
A two-years-old girl presented with an abdominal mass with loss of appetite, vomiting and low-grade fever for five days. [ijri.org]
Except the abdominal mass, there was no sign of fever, respiratory distress or vomiting. On the admission day, his weight was 3160 g. [omicsonline.org]
- Inflammation
The adjacent hepatic parenchyma shows congestion and mild portal fibrosis, portal inflammation and proliferation of bile ductules consistent with adjacent mass effect. [pathologyoutlines.com]
- Fatigue
Chronic fatigue secondary to anemia thrombocytopenia, and leukocytosis and lack of appetite are often reported. Jaundice and biochemical derangement are signs of advanced neoplastic change. [intechopen.com]
Gastrointestinal
- Diarrhea
[…] cystic mass with internal septations on ultrasound Complex cystic mass with septal and solid stromal enhancement on CT or MRI Radiology images Images hosted on other servers: CT scans Case reports 5 month old girl with Beckwith-Wiedemann syndrome with diarrhea [pathologyoutlines.com]
[…] and ear anomalies. 1-5 Children with BWS have an increased risk for embryonic tumors, mainly Wilms tumor and hepatoblastoma. 1,6,7 Case Report A 5-month-old female infant with known Beckwith-Wiedemann syndrome (BWS) was admitted following episodes of diarrhea [acgcasereports.gi.org]
Other uncommon associated symptoms are vomiting, fever, constipation, diarrhea, and weight loss.[1] Laboratory investigations usually reveal normal liver function with elevated alpha-fetoprotein, which is believed to be secreted by the proliferating hepatocytes [mirabolfathi.com]
Cardiovascular
- Hypotension
• No fatal events • Adverse events in 29 investigations (0.0086%) – Itching, nausea, sense of warmth – Dizziness – Headache – Mild hypotension • Four adverse events required treatment – Two serious (dysponea, hypotension) – Two non-serious • Lower adverse [slideshare.net]
Liver, Gall & Pancreas
- Liver Mass
Embolization of liver mass Mesenchymal hamartoma Neonatal liver masses Pulmonary hypertension Cite this Apa Standard Harvard Vancouver BIBTEX RIS Mulrooney, D. A., Carpenter, B., Georgieff, M. K., Angel, C., Hunter, D., Foker, J. E., & Steiner, M. [experts.umn.edu]
Markedly increased serum alpha-fetoprotein (AFP) levels (1 060 000 mg/L), highly suspicious for hepatoblastoma, were detected, and resection of the liver mass was performed. [scholars.northwestern.edu]
Mesenchymal hamartoma of the liver is a cystic benign liver mass occurring in children. Diagnostic confusion with hepatoblastoma may arise when alpha-feto-protein (AFP) level is elevated. [ncbi.nlm.nih.gov]
On the other hand, adult hepatobiliary surgeons faced with a fetus or infant with a liver mass are ill equipped to care for the patient even if they have done a high volume of adult liver surgery and are expert in the field. [benthamsciencepublishers.wordpress.com]
Skin
- Flushing
A total of 2.8 mL of ethylene-vinyl alcohol copolymer (Onyx 18, ev3, France) was injected downstream through a 2.4-F microcatheter (Rebar 18, ev3), previously flushed with 1.5 mL dimethyl sulfoxide solution. [pediatrics.aappublications.org]
Urogenital
- Amenorrhea
Imaging workup after birth (34 weeks of amenorrhea) had revealed multiple cystic lesions in the right liver, suggesting mesenchymal hamartoma. At admission, the patient was asymptomatic, fed normally, and had regular bowel habits. [pediatrics.aappublications.org]
Neurologic
- Confusion
Mesenchymal hamartoma of the liver is a rare benign tumor of childhood which has been confused with various other benign liver lesions, particularly vascular hamartomas which are mesodermal but not mesenchymal. [ncbi.nlm.nih.gov]
The imaging findings are often confused for a liver abscess. The lesion is typically located in the right lobe, and there may be extrahepatic involvement at diagnosis. [giornalechirurgia.it]
PNST is another rare haemangiopericytoma-like soft tissue tumours that may be confused with SFT. [diagnosticpathology.biomedcentral.com]
It occurs most commonly in the first 2 years of life. [2] A hepatoblastoma (HB) is the most common primary hepatic tumor in children, with 68% of cases manifesting in the first 2 years of life. [3] On fine-needle aspiration (FNA) cytology, MH can be confused [jcytol.org]
Workup
The serum tumor marker, AFP, is obtained during the workup; more than 90% of patients with HB have elevated AFP levels. [emedicine.medscape.com]
Imaging workup after birth (34 weeks of amenorrhea) had revealed multiple cystic lesions in the right liver, suggesting mesenchymal hamartoma. At admission, the patient was asymptomatic, fed normally, and had regular bowel habits. [pediatrics.aappublications.org]
The initial workup for hepatic masses includes radiographic assessment using ultrasonography. [intechopen.com]
Treatment
Treatment is partial or total excision of the lesion. Mortality (7-17%) is related to intraoperative or postoperative complications. Recurrence or malignant transformation has not been noted. [ncbi.nlm.nih.gov]
‘Watchful Waiting’ May Be an Appropriate Treatment for ELBW Infant with Hepatic Mesenchymal Hamartoma, Iran J Pediatr. 2013 ; 23(3):366-368. Abstract © 0, Author(s). [ijp.tums.pub]
Only small series have been published regarding treatment. [northshore.org]
Prognosis
Prognosis after extirpation is very good. [ncbi.nlm.nih.gov]
These categories will also aid in follow-up and prognosis. COG protocols have introduced a risk-based determination of treatment with very low-, low-, intermediate-, and high-risk categories. These categories have aided in follow-up and prognosis. [emedicine.medscape.com]
Prognosis When there is complete resection of the tumor, the prognosis is excellent. When aspiration or watching and waiting are the primary means of treatment, then the prognosis is not as clear cut and is evaluated on a case by case basis. [atlasgeneticsoncology.org]
Etiology
DIFFERENTIAL DIAGNOSIS: Pediatric hepatic masses include both neoplastic and nonneoplastic proliferations as well as infectious etiologies. [mirabolfathi.com]
Hence the etiology remains unknown. Review of literature revealed only one case of AS arising in an adult MH. [5] The authors have reported a case of a 33-year-old Chinese woman who was diagnosed as hepatic hamartoma eight years ago on FNAC. [ijpmonline.org]
The etiology of MHL has been the subject of considerable discussion. The possibility of malignant transformation of MHL is inclusive. [link.springer.com]
The management and etiology of this case is discussed. KEY WORDS: Mesenchymal hamartoma, Liver Tumor. Pak J Med Sci October - December 2008 (Part-II) Vol. 24 No. 6 876-878 How to cite this article: Majidpour HS, Yousefinejad V, Salehi MG. [pjms.com.pk]
Embryonic origin Mesoderm Etiology Unknown Epidemiology Second most common liver tumor following hepatic hemangiomas. Pathology Unknown. There are several possibilities with regards to the pathophysiology of MHL. [atlasgeneticsoncology.org]
Epidemiology
Epidemiology & Infection 143 (7), 1552-1555, 2015 2 2015 Gastrointestinal tract carcinoma in pediatric and adolescent age: The Italian TREP project experience A Indini, G Bisogno, G Cecchetto, M Vitellaro, S Signoroni, M Massimino, ... [scholar.google.com]
Ecographic epidemiology of non-parasitic hepatic cysts. J Clin Ultrasound 1993; 21: 115-118. 7. O'Sullivan MJ, Swanson PE, Knoll J, et al. [ispub.com]
Embryonic origin Mesoderm Etiology Unknown Epidemiology Second most common liver tumor following hepatic hemangiomas. Pathology Unknown. There are several possibilities with regards to the pathophysiology of MHL. [atlasgeneticsoncology.org]
[…] of chromosome 19 microRNA cluster (C19MC) Terminology Hepatic or giant lymphangiomas, bile cell fibroadenomas, cavernous lympangiomatoid tumors, hepatic cystic hamartoma (SA J Radiol 2020;24:1891) ICD coding ICD-10: D13.4 - benign neoplasm of liver Epidemiology [pathologyoutlines.com]
Pathophysiology
This immunohistochemical study was performed to gain insight into the pathophysiology of its development. [ncbi.nlm.nih.gov]
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. 1. Stringer, MD, Alizai, NK. [journals.sagepub.com]
There are several possibilities with regards to the pathophysiology of MHL. One should always consider the possibility that the recurring translocation has little or nothing to do with tumor formation, but is rather found as a secondary phenomenon. [atlasgeneticsoncology.org]
Ann R Surg Engl 85: 324–330 CrossRef Google Scholar Davit-Spraul A, Gonzales E, Baussan C, Jacquemin E (2010).The spectrum of liver diseases related to ABCB4 gene mutations: pathophysiology and clinical aspects.Semin Liver Dis 30(2): 134–46 PubMed CrossRef [doi.org]
Prevention
The second issue is to prevent any embolization of nontarget portal branches, which could be achieved with the use of an embolic agent, such as ethylene vinyl alcohol copolymer (Onyx; ev3). [pediatrics.aappublications.org]
Amir-Mohammad Armanian 1 *, Masoud Nazem 2, Nima Salehimehr 3 and Babak Nekooie 4 1 Division of Neonatology, Department of Pediatrics, Child Growth and Development Research Center, Research Institute for Primary Prevention of Non-Communicable Disease, [omicsonline.org]
Tumors may be very large, sometimes reaching 20-30 cm in diameter and poses surgical challenge for complete excision. [1] Spontaneous incomplete regression and malignant transformation are known to occur. [1] Malrotation was probably due to large cyst preventing [jcnonweb.com]
Chang MH, Chen TH, Hsu HM, et al.: Prevention of hepatocellular carcinoma by universal vaccination against hepatitis B virus: the effect and problems. Clin Cancer Res 11 (21): 7953-7, 2005. [northshore.org]
Since cholangitis is the principle cause of morbidity and mortality, OLT should be advocated earlier during the natural history of the ‘diffuse type’ of Caroli’s disease with recurrent biliary infections and an aggressive management to prevent and treat [karger.com]