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Citation: Thamri F, Sahli S, Zouaoui A, Jouini R. Hepatic Mesenchymal Hamartoma.Jr.med.res.2021;4(2): 14-16. Thamri et al © All rights are

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Case Report

Thamri Fatma

1,2

, Sahli Sondes

1,2,*

, Zouaoui Arije

1,2

, Jouini Riadh

1,2

Hepatic Mesenchymal Hamartoma.

1: Department of pediatric surgery A,

Children’s Hospital, Tunis , Tunisia

2: College of medicine, Tunis, Tunisia

* Corresponding author

Correspondence to:

sondessahli@yahoo.fr

Publication Data:

Submitted: July 21,2021

Accepted: September 22,2021

Online: November 30,2021

This article was subject to full

peer-review.

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Hepatic mesenchymal hamartoma is a rare benign tumor arising from the mesenchyma of

the portal tract. Hamartoma usually presents before the third year of life. Remarkable

abdominal swelling is the most frequent clinical feature.

We herein present a new case with a comprehensive literature review. The aim was to

highlight he clinical , radiological and histopathological characteristics of this entity.

Keywords:

Mesenchyma; Hamartoma; Liver tumor; Children.

Introduction

Hepatic mesenchymal hamartoma (HMH) is an uncommon tumor arising from the

mesenchyma of the periportal tract. It accounts for 8% of all pediatric hepatic tumors.

However, its ethiopathogeny is still unclear and the management remains controversial

[1]. Abdominal enlargement and respiratory distress are the most common clinical

presentations [2]. HMHs are benign lesions and must be considered in the differential

of liver malignancies.

Observation

A 17-month-old boy was referred to us for progressive diffuse abdominal enlargement

started 2 months ago. His medical history was unremarkable. The physical examination

showed a large abdominal mass palpable on the right upper abdominal quadrant. All

the laboratory tests performed including alpha-fetoprotein (AFP) were within normal

range. The abdominal radiograph showed a large non calcified intra-abdominal soft

tissue density in the right upper quadrant with a displacing mass effect on the gastro-

intestinal tract (figure 1). Chest radiograph showed an elevated right hemidiaphragm.

Abdominal ultrasound examination identified multiloculated cystic mass occupying the

right hepatic lobe with multiple thin echogenic septae (figure2).

CT scan of the abdomen and pelvis was performed for further assessment. It revealed

large 13x7x7cm cystic tumor with heterogeneous enhancement of its solid component.

The cystic portion contained multiple enhancing septae (figures 3).

The patient underwent a laparotomy. A huge, encapsulated tumor developed between

the hepatic segments IV and V was noted. Tumorectomy was performed. Frozen

sections confirmed its benign character. The postoperative courses were uneventful.

The patient was discharged on the fifteenth postoperative day.

Microscopically, the tumor contained hepatocytes, abnormal bile ducts and immature

mesenchyma in variable portions. Most of the cysts were areas of degeneration with

fluid accumulation separated by thin strands of connective tissue (figure 4). The

histopathology concluded to mesenchymal Hamartoma of the liver. On the six months

follow up, the child was asymptomatic with normal physical examination.

Abstract

Hepatic Mesenchymal Hamartoma in Children

Citation: Thamri F, Sahli S, Zouaoui A, Jouini R. Hepatic Mesenchymal Hamartoma.Jr.med.res.2021;4(2): 14-16. Thamri et al © All rights are

reserved. Submit your manuscript: www.jmedicalresearch.com

Figure 1: Displacing mass effect on the gastro-intestinal tract

Figure3: histopathological features

Figure 2: CT features of liver mass with heterogeneous enhancement of its solid component. The cystic portion contains multiple septae.

Hepatic Mesenchymal Hamartoma in Children

reserved. Submit your manuscript: www.jmedicalresearch.com

Discussion

Described since 1903, Hepatic mesenchymal hamartoma (HMH) is one of the most common benign liver tumors in

children. The lesion is usually diagnosed within the first 2 years of life [3]. However, prenatal cases diagnosis has been

described [4]. The most frequent clinical presentation is upper abdominal palpable mass [5]. The tumor is in the right

lobe in more than 90% of cases. The liver function remains usually normal. Mild elevation of the alpha-fetoprotein is

occasionally observed and has no malignancy significance [6]. Histologically, HMH is characterized by a lobular growth of

myxomatous connective tissue containing scattered bland stellate-shaped mesenchymal cells [7]. The etiopathogenesis

involves mutations and genetic alterations in both sporadic and syndromic HMHs [8]. Imaging features are typically of

complex cystic mass with multiple solid enhanced septae [9]. Complete surgical excision is the only curative option.

Malignant transformation on the residue of incomplete excision has been described [10]. Ultrasound guided aspiration

and monitoring are proposed for unresectable tumor cases.

Conflict of Interest: None

References

[1] Martins-Filho SN, Putra J. Hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of the liver: a pathologic review. Hepat Oncol. 2020;7:HEP19.

[2] Le LT, Phan HT, Nguyen TS, Truong KD, Nguyen DT, Nguyen TB, et al. Infantile mesenchymal hamartoma of the liver with elevated alpha fetoprotein. BJR Case Rep. 2021;

7:20200196.

[3] Rosado E, Cabral P, Campo M, Tavares A. Mesenchymal hamartoma of the liver--a case report and literature review. J Radiol Case Rep. 2013;7:35-43.

[4] Habibzadeh P, Ansari Asl M, Foroutan HR, Bahador A, Anbardar MH. Clinicopathological study of hepatic mesenchymal hamartoma and undifferentiated embryonal sarcoma of

the liver: a single center study from Iran. Diagn Pathol. 2021;16:55.

[5] Gupta R, Parelkar SV, Sanghvi B. Mesenchymal hamartoma of the liver. Indian J Med Paediatr Oncol. 2009;30:141-3.

[6] Iacob ER, Popoiu CM, Nyiredi A, Mogoantă L, Badea O, Boia ES. Mesenchymal hamartoma of the left liver lobe in an 18-month-old female patient. Rom J Morphol Embryol.

2016;57:841-47.

[7] Bahador A, Geramizadeh B, Rezazadehkermani M, Moslemi S. Mesenchymal hamartoma mimicking hepatoblastoma. Int J Organ Transplant Med. 2014;5:78-80.

[8] Chandramouleeswari K, Anita S, Shivali B. Mesenchymal hamartoma of the liver: a case report. J Clin Diagn Res. 2012;6:1552-4.

[9] Aineseder M, Mestas Núñez MA, López Grove R, Padilla ML, et al. Mesenchymal hamartoma and undifferentiated embryonal sarcoma: diagnostic keys that radiologists need to

know. Radiologia (Engl Ed). 2021;63:42-49.

[10] Koganti SB, Thumma VM, Nagari B. Mesenchymal hamartoma of the liver: Complete excision always necessary. Case Rep Surg. 2017;2017:8314102.