# A Rare Case of Giant Mesenteric Lipoblastoma in a 6-Year-Old Child and Review of the Literature

Authors: Maria Enrica Miscia; Gabriele Lisi; Giuseppe Lauriti; Angela Riccio; Dacia Di Renzo; Valentina Cascini; Pierluigi Lelli Chiesa
Journal: Case Reports in Surgery
 (2020)

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## Abstract

Giant mesenteric lipoblastoma is a rare benign tumor arising from the adipocytes. It can mimic malignant tumors, and its diagnosis is difficult before surgery. Imaging studies could lead the diagnosis but not confirm it. Those tumors arising in the abdomen are usually larger and can cause symptoms of compression. Surgical excision is the treatment of choice, and a long-term follow-up is necessary to detect local recurrences. Only a few cases of lipoblastomas arising from the mesentery are reported in literature. We present a case of a rare giant lipoblastoma arising from the mesentery of a 6-year-old girl, with a history of postprandial abdominal pain.


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## Body

## 1. Introduction

Adipose tumors are rare in childhood and represent 6% of all the neoplasms of the soft tissue.They can be classified into malignant (liposarcoma) and benign (lipoma, lipoblastoma, lipoblastomatosis) [1, 2].Lipoblastoma is a rare, encapsulated tumor arising from the embryonal fat tissue [3].It is more frequent in children younger than 3 years old, with a male to female ratio of 3 : 1 [1, 3].Common localizations are extremities and trunk. Intra-abdominal lipoblastoma is extremely rare (<7%) and mesenteric localization is exceptional [2–4].We present a case of giant mesenteric lipoblastoma in a girl, reviewing and discussing pertinent literature.


## 2. Case Report

A 6-year-old girl came to our attention for a 2-month-lasting postprandial abdominal pain.Her medical history was suggestive of constipation for 2 years. A painless, mildly distended, not tender abdomen was palpable on physical examination.An abdominal ultrasound showed a hypoechoic abdominal mass of9×4 cm in size, not vascularized at the color-Doppler study and well separated from the adjacent organs. In the suspicion of a lipoma, the girl underwent an abdominal magnetic resonance imaging (MRI), which confirmed the presence of an encapsulated fatty-dense mass (20×4×18 cm), occupying the entire abdominal cavity (Figures 1(a) and 1(b)).Figure 1
(a, b) MRI ((a) T1 weighted, (b) Thrive sequences) showing the mass occupying the entire abdominal cavity, compressing and displacing the bowel, the inferior vena cava, and common iliac veins.
(a)(b)Blood exams (complete blood count, C-reactive protein, and liver function tests) and tumoral markers (alfa-fetoprotein, beta-human chorionic gonadotropin, lactic dehydrogenase, carcinoembryonic antigen, and neuron-specific enolase) were within ranges.An explorative laparoscopy was then performed. It showed a huge fatty mass arising from the ileal mesentery and stretching the ileal loops. The mass was completely excised through a minilaparotomy, even if a resection of a tract of intestine involved was necessary. The lesion was21×19×7 cm and weighted 1,236 g (Figures 2(a) and 2(b)). Bowel continuity was restored through a primary end-to-end anastomosis.Figure 2
(a, b) Intraoperative findings: the mass comes from the mesentery, stretching the involved ileum (a), which was resected together with the mass (b).
(a)(b)Histopathological analysis showed adipocytes at different stages of maturation with focal myxoid areas and confirmed the diagnosis of giant abdominal lipoblastoma.Postoperative period was uneventful with excellent esthetic results. No recurrence was noticed at a 1-year ultrasonographic follow-up (Figure3).Figure 3
Esthetic results at follow-up.


## 3. Review of the Literature

A review of the English literature was performed using a defined search strategy. Scientific databases (PubMed, Medline, Cochrane Collaboration, and Scopus) were screened looking for studies reporting on mesenteric lipoblastoma in children. MeSH headings and terms used were “lipoblastoma AND children”. Reference lists were examined to identify relevant cross-references. Of 408 titles and abstracts, 22 case reports were included, reporting data on 22 cases from 1973 to date (Table1) [4–24]. We found a male to female ratio of 1.3 : 1 (12 M, 9 F; 1 not available). Mean age at diagnosis was 29±21 months, and preferred localization, when reported, was the ileal mesentery (6/11).Table 1
Review of the English literature.
AuthorYearSexAgeSymptomsSize (cm)PositionSurgeryFollow-upRecurrenceChung and Enzinger [9]1973F2 yrsn.a.n.a.n.a.Excision mass+23 cm small boweln.a.NoFriedman et al. [10]1981F13 moPalpable massn.a.n.a.n.a.n.a.NoStringel et al. [11]1982M13 moAbdominal distension, palpable mass12×10.8×17IleumExcision mass+26 cm small bowel1 yrNoJimenez [12]1986M11 moAbdominal distension, palpable mass12×10×0.8JejunumExcision mass+part small bowel3 yrsNoZanetti [13]1988F4 yrsPain, vomiting18n.a.Excision mass+part intestine2 yrsNoDenath [14]1988F2 yrsAbdominal distensionn.a.n.a.n.a.n.a.NoPrando et al. [15]1990M2 yrsAbdominal distension, palpable mass23×19×9n.a.Excision massn.a.n.a.Schulman et al. [16]1992M2 yrsAbdominal distension, palpable massn.a.n.a.Excision massn.a.n.a.Posey et al. [17]1998M10 moAbdominal distension, palpable mass10×6×13IleumExcision mass2 yrsNoO’Donnell et al. [18]2000F5 moAbdominal distension, diarrhea14.5×11×10.5Transverse colonExcision mass6 moNoMo et al. [19]2003F16 moAbdominal distension, palpable mass10×9×13IleumExcision mass+26 cm small boweln.a.n.a.Al-Salem and Al-Nazer [8]2003M2 yrsObstruction, midgut volvulus8×6×5IleumExcision massn.a.n.a.Jung et al. [20]2005M17 mon.a.9.7×7×5.5n.a.n.a.23 moNoCudnik et al. [5]2008M3 yrsAbdominal distention, fullness, intermittent pain15×4.5×10JejunumExcision mass+part small bowel1 yrNoYu et al. [7]2009F7 yrsObstruction, midgut volvulus10×8×6JejunumExcision mass+part small bowel1 moNoTang et al. [21]2009M4 yrsIntermittent abdominal pain18×15×10IleocecalExcision mass4 moNoJia and Zhang [22]2009M4 yrsn.a.n.a.n.a.n.a.n.a.NoGentimi et al. [4]2011M18 moAbdominal distension, palpable mass14×11×8IleumExcision mass+part small bowel30 moNoCapasso et al. [23]2014M3 yrsAbdominal pain and vomitingn.a.n.a.Excision massn.a.n.a.Ghosh et al. [24]2015F14 moPalpable abdominal mass, intestinal obstructionn.a.n.a.Excision massn.a.n.a.Yang et al. [6]2016n.a.n.a.n.a.n.a.n.a.n.a.n.a.NoPresent case2019F6 yrsPostprandial abdominal pain21×19×7IleumExcision mass+part small bowel1 yrNon.a.: not available.Three out of 18 patients had an acute onset, with volvulus [7–9]. None of the patients underwent a laparoscopic excision of the mass. A resection of small bowel has been reported in 9/17 cases.Mean time of follow-up was16±12 months. No recurrences have been reported, when mentioned.


## 4. Discussion

Lipoblastoma is a rare benign, encapsulated tumor of adipocytes, accounting for 5-30% of all the adipose tumors [3]. When diffuse and infiltrative, it is called lipoblastomatosis. It was firstly described by Jaffe in 1926 [4].It arises from the abnormal proliferation of embryonic fat cells but its etiopathogenesis is not completely understood [1, 3, 25].It is more frequent in the first decade of life, with a peak of incidence in males younger than 3 years (about 90% of all cases) [1, 3].These tumors are usually asymptomatic; however, they can cause a mass effect when reaching considerable dimensions [3, 25].Lipoblastoma is commonly located at the level of the trunk and extremities. Intra-abdominal localizations are rare; nonetheless, intraperitoneal tumors usually reach superior dimensions [2, 3].Typical symptoms of abdominal lipoblastomas are abdominal pain, constipation, and vomiting, secondary to organ compression. Acute manifestations, secondary to volvulus or intussusception, are also reported, although rare [7–9].Imaging studies, especially MRI, are useful to define the adipose nature, the extent, and tissue involvement of neoplasia. However, this study is unable to differentiate among lipoma, lipoblastoma, and liposarcoma [3, 26, 27]. In fact, the proportion of the myxoid stroma determines the imaging appearance of the lesion: a well-defined predominantly fatty lesion is likely to be a lipoblastoma, while an infiltrative lesion with a less-represented fatty component could be a lipoblastomatosis [27]. The first imaging study performed is the ultrasound; however, an MRI or a computed tomography scan is usually required to better define the adipose nature and the relationship with the surrounding organs. Definitive diagnosis is secondary to histopathological examination, and surgical excision is the treatment of choice.Microscopic features consist of lobular architecture and myxoid areas with spindle cells and lipoblasts at various stages of differentiation [28, 29].Rearrangements of chromosome 8q11 codifying for the oncogene PLAG1 are found in more than 70% of cases [2–4, 28–30]. The different molecular alteration found in adipocytic tumors could help in differentiating between lipoma, lipoblastoma, and liposarcoma [31]. This information could be useful to get before surgery, especially if the resection of the tumor requires a long length of intestinal resection.Recurrence rate is reported to range between 9 and 46%, and it is usually secondary to an incomplete surgical excision [3, 6, 30].A problem related to mesenteric lipoblastomas is the massive size of the mass and its close contiguity to the mesenteric vessels and intestinal loops, which makes a purely laparoscopic excision virtually impossible to perform. In children, laparotomy incision should be as small as possible, to limit the extent of the scar, as we did in our case.


## 5. Conclusions

Mesenteric lipoblastoma is a rare benign tumor.It can be completely asymptomatic or can cause symptoms related to a mass effect. Imaging studies are unable to reach the definitive diagnosis; therefore, surgical excision is the treatment of choice and leads to a definitive diagnosis through the histopathological examination of the specimen.We stress the importance of a surgery that should be as mini-invasive as possible, considering the localization and the size of the mass. A long-term ultrasonographic follow-up is necessary to detect local recurrences.



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