Edit concept Question Editor Create issue ticket

Mesenchymal Hamartoma of the Liver

Hepatic Mesenchymal Hamartoma


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]
  • It is potentially pre-malignant lesion presenting as a solid and/or cystic neoplasm.[ncbi.nlm.nih.gov]
  • This case is unusual as mesenchymal hamartoma has not been previously reported presenting with pyrexia and anaemia as well as hepatomegaly.[ncbi.nlm.nih.gov]
  • Four patients are presented and reviewed with 25 previously reported cases.[ncbi.nlm.nih.gov]
  • We present the case of a 1-year-old male patient with a huge tumour of the right lobe of the liver which showed rapid enlargement.[ncbi.nlm.nih.gov]
Increased Abdominal Girth
  • Abstract A 28-year-old black woman presented with increasing abdominal girth and gross hepatomegaly. Preoperative investigations demonstrated an enlarging vascular tumor involving the right lobe of the liver.[ncbi.nlm.nih.gov]
  • She presented to our hospital with increasing abdominal girth, abdominal pain, and vomiting.[wjgnet.com]
Epigastric Tenderness
  • In the time of admission, she was febrile ( 39 C ) ,BP 120/70 mmHg, PR 90/min and RR 18/min RUQ and epigastric tenderness ,guarding and fullness was detected and obstetric examinations were normal.[mirabolfathi.com]
Left Upper Quadrant Pain
  • In addition to diffuse abdominal pain, reported symptoms and signs include hepatomegaly, right hypochondriac pain and left upper quadrant pain. Radiographic appearance of MHL represents a continuum from predominantly cystic to mostly solid tumors.[mirabolfathi.com]
Hepatic Mass
  • In children under 2 years of age who present with partly solid and partly cystic hepatic masses, the possibility of MH of the liver should be considered. FNA has a role in the diagnosis of MH.[ncbi.nlm.nih.gov]
  • Each of the patients was a woman who had a solid or multicystic hepatic mass ranging from 5 to 24 cm in maximum dimension.[ncbi.nlm.nih.gov]
  • A septated, noncalcified, cystic hepatic mass in an infant with normal liver function studies and characteristic ultrasound or CT is likely a benign mesenchymal hamartoma that can be cured by total local excision.[ncbi.nlm.nih.gov]
  • An unusual presentation of a large hepatic mass in a newborn complicated by pulmonary hypertension and vascular " steal” with renal insufficiency is presented.[experts.umn.edu]
  • The differential diagnosis consists of other pediatric hepatic masses as well as select infectious cysts.[mirabolfathi.com]
Indecisiveness
  • Due to non-specific clinical symptoms and indecisive imaging findings, correct diagnosis may be difficult to establish.[scinapse.io]

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]
Karyotype Abnormal
  • Rakheja et al, recently reported a case of HMH in an month old boy that demonstrated a break point at 19q13.4, which was similar to the previous 4 reported cases that had a documented karyotypic abnormality.[mirabolfathi.com]
Multiple Liver Cysts
  • Usually it presents as multiple liver cyst formations without communication with the principal bile duct. They are frequent in children, being rare in adults.[unboundmedicine.com]
  • Computed tomography (CT) of her abdomen revealed multiple liver cysts, with the diameter of largest cyst being 16 cm 14 cm. The liver hilar structures were not clearly displayed. The adjacent organs were compressed and displaced.[ncbi.nlm.nih.gov]
  • In the present case, the initial abdominal enhanced CT scan revealed multiple liver cysts, which could easily have been misdiagnosed as a polycystic liver.[wjgnet.com]

Treatment

  • The treatment is surgical removal. The tumor is characterized by proliferation of collagenous connective tissue, immature mesenchyme, and multiple cysts or pseudocysts of varying sizes. The origin of the tumor has not yet been definitely determined.[ncbi.nlm.nih.gov]
  • 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]
  • Treatment of choice is radical excision. The authors report a case of solid stromal predominant MHL in a 12-month-old male infant who also had an elevated serum alpha-fetoprotein level.[ncbi.nlm.nih.gov]
  • These findings suggest that the conventional approach of completely resecting the tumor whenever possible is the best treatment.[ncbi.nlm.nih.gov]
  • From the literature, it is not clear whether marsupialization is an effective method of treatment.[journals.lww.com]

Prognosis

  • Prognosis after extirpation is very good.[ncbi.nlm.nih.gov]
  • With resection, the prognosis is excellent.[ncbi.nlm.nih.gov]
  • Although histologically benign, because this lesion frequently results in perinatal complications such as fetal hydrops, maternal toxemia, and preterm labor, early careful fetal ultrasonography should improve the prognosis of this lesion.[ncbi.nlm.nih.gov]
  • The prognosis of this tumor is good and recurrences after the tumor extirpated have not been Accepted Dec. 31, 1996 reported. Chinese Journal of Cancer Research Springer Journals[deepdyve.com]
  • Study of more cases will be needed to know whether there are any differences in the prognosis and behavior of AS arising de novo and AS arising in MH.[ijpmonline.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 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]
  • The etiology of MHL has been the subject of considerable discussion. The possibility of malignant transformation of MHL is inclusive.[link.springer.com]
  • […] 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 rotation. [1] MHL has diverse etiology[jcnonweb.com]

Epidemiology

  • 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]
  • 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]
  • 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]
  • […] novel tyrosine kinase inhibitor of angiogenesis, has shown some benefit in clinical trials and has been approved for HCC in adults by the US Food and Drug Administration (FDA). [28] The overall survival rate remains poor, with a recent Surveillance, Epidemiology[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • This immunohistochemical study was performed to gain insight into the pathophysiology of its development.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • 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]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!