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Lipoblastoma in children - Case Report

MS. Anilkumar¹, K .Jagadish Kumar², K. Raheja¹, R. Sunila³
¹Pediatric Surgery Department

²Pediatrics Department

³Pathology Department

JSS Medical College, JSS University, Mysore, India

 

Correspondence

Dr. K .Jagadish Kumar, MD
Professor of pediatrics,
JSS Medical College, JSS University,
Mysore, Karnataka, India
Mobile: 09844281859
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Abstract

Lipoblastomas and lipoblastomatosis are very rare benign tumors arising from embryonal fat cells. They mainly occurs in children less than 3 years of age with the male preponderance. These tumors usually arise in the extremities and present as painless soft mass. Even though there are no metastasis reported, recurrences are known after excision. Therefore a follow up period of at least 3 years is recommended.

Key words: lipoblastoma, children, benign tumor

 

Introduction

Lipoblastoma is a rare benign lipomatous tumor, most commonly occurring in the first 3 years of life [1]. Lipoblastoma is lobulated, superficial and encapsulated whereas lipoblastomatosis is diffuse, deeper and infiltrative to adjacent tissues .It is more common in males with M: F ratio of 3:1 [1,2,3,4].

Extremities are the common site of involvement and usually presents as painless mass [1,5,6]. Lipoblastoma is a tumor with cells resembling fetal fat, which has admixture of multivacuolated lipoblasts and mature adipocytes with plexiform vascular pattern and abundant myxoid stroma.We report two cases of lipoblastoma with the follow up period of 4 years.

Case Report

Case 1

A 18 month old girl presented to us with right gluteal region mass since 4 months, gradually increasing in size without any pain. On examination a soft mobile non tender subcutaneous 10x5 cm mass was palpable with normal overlying skin .Otherwise baby was normal. Fine Needle Aspiration Cytology (FNAC) was not conclusive. The mass was soft and lobulated, excised en bloc .There was no invasion of the underlying tissues. On gross examination: single gray globular mass was seen and cut section showed fatty tissue. On microscopy: tissue showed adipose cells in various stages of maturation divided into lobules by fibrous septae. Proliferating blood vessels were seen admixed with adipose cells under the background of myxoid stroma.

Case 2

A 12 month old girl presented to us with mass in the left gluteal region since 6 months. On gross examination 3x3 cm ulcer was present at the apex of mass with 9x7x5cm in size (fig. 1). Ultrasound of the mass showed increased vascularity.

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Figure 1: Photograph showing ulcerated Lipoblastoma of gluteal region

FNAC smears showed mature adipose tissue with vacuolated adipose cells, few capillaries under the myxoid background. The mass was excised with wide margin. On gross examination the lobular gray mass was seen with ulcerated skin. On microscopy smear showed adipocytes at various stages of maturation with lobules separated by septae, the background showed large amount of myxoid matrix with fine capillaries (fig. 2). Both the cases were followed up for 4 years. The second case came back with recurrence of mass at the same site after 2 years but refused to undergo surgery.

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Figure 2: Histopathology photograph showing adipocytes and plexiform vascular pattern

Discussion

Lipoblastoma and lipoblastomatosis are two different presentations of the same rare benign soft tissue mesenchymal tumour arising from fetal white fat and occurring almost exclusively in young children under 3 years [7]. Out of 149 adipose tumors recorded by Miller et al., 7 (4.7%) were lipoblastomas and 2 (1.3%) were liposarcomas [8]. In a study by Wallas et al. out of 27 lipomatous tumors only one was lipoblastoma [9]. Typically they present in children below 3years of age and extremities are the common sites of involvement [3,5,7]. Stringel et al. reported that 70% lipoblastomas occurred in the extremities with a M: F ratio of 3:1 and 55% of them were under 1 year of age [3]. Out of 25 cases reported from Collin et al. 11 of them involved the extremities [6]. However both our cases were females and were less than 2 years of age.

In a series by Jung et al., neck was the common site with male preponderance [2]. Usually they present as painless, well circumscribed mass and are located subcutaneously. The differential diagnosis of a fatty soft tissue lesion in a young child includes lipoma, liposarcoma and teratoma.

Lipomas are rare in the first two decades of life and are slow growing when compared to lipoblastoma.The extent of vacuolized adipocytes is much greater in lipoblastoma when compared to lipoma [10]. It is very difficult to differentiate lipoblastoma from liposarcoma. Helpful clues include increased nuclear atypical pattern , lack of lobulation and age of onset in liposarcoma [1,9].

Liposarcoma in children less than 10 years of age is very rare [1, 2, 10]. Cytogenetical studies is helpful to differentiate between the two [4,7,8,11]. It is difficult to diagnose lipoblastoma because it does not have any characteristic clinical signs and the diagnosis is established on histopathological examination of the specimen [11].

The preoperative duration at the time of presentation ranges from 3 years to several years [1]. However our cases presented with the history of 6 months duration. The size ranges from 1 cm to 21 cm in greatest diameter [6].

They are suggested as developmental anomaly with continued proliferation of lipoblasts in the postnatal life Lipoblastomas are well capsulated, lobulated, soft and are yellow-white in color.

They are benign and no metastasis have been reported [3,4,7].Lipoblastoma is referred to tumor with cells resembling fetal fat, which has admixture of multivacuolated lipoblasts and mature adipocytes with plexiform vascular pattern and abundant myxoid stroma [2]. Complete excision of the tumor is the treatment of choice [6,11].

Recurrence rate in lipoblastoma is between 14% to 25% in various studies [1,5]. Both our cases were followed up for 4 years and one had recurrence. In view of the tendency for diffuse lesion to recur locally complete excision with margin of normal tissue is advisable [1]. At least a follow up period of 3 years is recommended [2]. To conclude although lipoblastoma and lipoblastomatosis are rare benign tumors of embryonal fat in children, we should keep them as differential diagnosis of a soft subcutaneous mass. In view of their tendency for recurrence, complete excision with a wide margin of normal tissue is advisable. A follow up of atleast 3 years is recommended with this condition.

 

 

 

References

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