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ANALYTICAL REPORT
Year : 2012  |  Volume : 8  |  Issue : 1  |  Page : 157-158

Giant lipoblastoma of the thigh: A rare soft tissue tumor in an infant


1 Department of Pediatric Surgery, Vani Vilas Hospital, Bangalore, India
2 Department of Pathology, Bangalore Medical College and Research Institute, Bangalore, India

Date of Web Publication19-Apr-2012

Correspondence Address:
Tanveer Akhtar
Department of Pediatric Surgery, Vani Vilas Hospital, BMC and RI, Bangalore - 560 002
India
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DOI: 10.4103/0973-1482.95202

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 > Abstract 

Lipoblastoma is a rare lipomatous tumor encountered almost exclusively in infants and young children. It arises from embryonic white fat. The common site of involvement is the extremities. In spite of their potential for local invasion, they are benign tumors. We report a case of a lipoblastoma in an infant and review the literature pertaining to clinical management of these tumors.

Keywords: Benign tumor, children, lipoblastoma


How to cite this article:
Akhtar T, Alladi A, Ahmed SM, Siddappa OS. Giant lipoblastoma of the thigh: A rare soft tissue tumor in an infant. J Can Res Ther 2012;8:157-8

How to cite this URL:
Akhtar T, Alladi A, Ahmed SM, Siddappa OS. Giant lipoblastoma of the thigh: A rare soft tissue tumor in an infant. J Can Res Ther [serial online] 2012 [cited 2014 Oct 25];8:157-8. Available from: http://www.cancerjournal.net/text.asp?2012/8/1/157/95202


 > Introduction Top


Lipoblastoma is a benign soft tissue tumor originating from white embryonic fat. It typically occurs before three years of age and usually develops in the extremities. Fewer than 10% of all the pediatric soft tissue tumors are adipose tumors, of which lipoblastoma accounts for only 5 to 30%. Surgical excision is the treatment of choice for lipoblastoma. Follow-up period of two to five years is recommended as recurrence has been reported in up to 14-25% of cases.


 > Case Report Top


One-year-old male child presented with an asymptomatic swelling over the right thigh that has been present since two months of age, which progressed rapidly. On examination, the swelling was mobile, non-tender, well-circumscribed and without any skin changes. It was occupying the whole of anterior, medial, and lateral aspect of the thigh [Figure 1]. Ultrasonography revealed a subcutaneous oval shaped soft tissue mass in the anterior, medial and lateral aspect of the thigh. Fine needle aspiration cytology revealed it to be a lipoma. On surgical exploration, the tumor was lobulated, well encapsulated [Figure 2] except at the undersurface where it was extending into the intermuscular planes. The tumor was completely excised. The specimen measured 18 × 12 × 8 cms and weighed 600 grams. Histopathological examination revealed it to be a mature lipoblastoma [Figure 3] with tumor containing predominantly mature adipose tissue and lipoblasts, with prominent paucicellular fibrous septa. The patient is on a regular follow-up since two years with no recurrence.
Figure 1: Tumor occupying the whole of anterior, medial, and lateral aspect of the thigh

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Figure 2: On surgical exploration, lobulated and encapsulated tumor

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Figure 3: Histopathology showing predominantly mature adipose tissue and lipoblasts, with prominent paucicellular fi brous septa

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 > Discussion Top


The term "lipoblastoma" was coined by Jaffe in 1926 to describe an atypical lipomatous lesion that consisted of cells resembling embryonic white fat. This term was intended to differentiate the lesion from the common lipoma which contains no lipoblasts [1] It is classified into two forms: a) lipoblastoma, which is a well localized, circumscribed and capsulated tumor, b) lipoblastomatosis is a deeply embedded, non-capsulating, multicentric, and infiltrative tumor [2] Hicks et al. in their study found that the majority of patients presented with a painless mass, which gradually increased in size [3] In our case, though the swelling was painless it progressed rapidly. Lipoblastoma can exert a mass effect on the surrounding structures causing abdominal pain, vomiting, respiratory insufficiency, neurodeficits depending on its location [3],[4] Arda et al. reviewed 84 cases from the literature and found that 61% of lipoblastomas occurred in the extremities, followed by 15% in the trunk, 14% in the abdomen, and 11% in the head and neck. [5] In contrast, several smaller studies have demonstrated a preponderance of tumor sites in the trunk (44-48%), [3],[6] and a study by Jung et al. found 31% of lipoblastomas in the head and neck region. [7] Lipoblastomas are generally found in pediatric patients with an estimated 55% of cases diagnosed in patients under one year of age. Ninety percent are identified before the age of three years, and virtually all reported cases occur within the first decade of life. Very few cases have been reported in adults. There is a male preponderance with male to female ratio of 3.8 to 1. [8]

Complete resection is the mainstay of treatment which has an excellent prognosis. Recurrence has been reported in 14% to 25% of cases, usually due to incomplete resection or diffuse disease. [4],[6] Higher recurrence rates are associated with lipoblastomatosis as it invades into the surrounding tissues, which contributes for an incomplete resection. [4],[8] Cases involving the head and neck too have a higher recurrence because of close proximity to vital structures leading to an incomplete excision. [7] When adjacent vital structures are involved, the tumor should be excised conservatively to reduce the mass effect. Radical mutilating surgery is not advocated for these tumors considering their benign nature. Because recurrence has been reported to occur as long as 84 months postoperatively, a follow-up period of at least 3 to 5 years is recommended. Recurrent lipoblastomas have been reported to exhibit greater cellular maturation than the original tumors. On rare occasions, recurrent lesions will exhibit reverse maturation, whereby the myxoid stroma and lipoblast content increases, while cellular differentiation decreases. [7] Recurrent lesions are best imaged with magnetic resonance imaging to assess extent and plan reconstruction if necessary. Re-excision is the treatment.

A lobulated architecture, the presence of adipose tissue, thin septa, peripheral lobules of more immature and less specific tissue and a peripheral pseudo capsule clinches the diagnosis by histopathology. Abnormal mitosis, which is characteristic of liposarcoma is not found in lipoblastoma. [8] In cases of lipoblastoma, tissue culture for cytogenetic study showed a breakpoint in the long arm of chromosome 8. A review of the literature showed seven case reports of lipoblastoma karyotype, six of which had a similar breakpoint in chromosome 8. This distinguishes it from the histologically alike myxoid liposarcoma, the karyotype of which typically contains the clonal anomaly t(12;16)(q13:p11). The tumor karyotype will, in most cases, aid in differentiating lipoblastoma from myxoid liposarcoma. [2]

 
 > References Top

1.Jaffe RH. Recurrent lipomatous tumors of the groin: Liposarcoma and lipoma pseudomyxomatodes. Arch Pathol 1926;1:381-7.  Back to cited text no. 1
    
2.Miller GG, Yanchar NL, Magee JF, Blair GK. Tumor karyotype differentiates lipoblastoma from liposarcoma. J Pediatr Surg 1997;32:1771-2.  Back to cited text no. 2
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3.Hicks J, Dilley A, Patel D, Barrish J, Zhu SH, Brandt M. Lipoblastoma and lipoblastomatosis in infancy and childhood: Histopathologic, ultrastructural, and cytogenetic features. Ultrastruct Pathol 2001;25:321-33.  Back to cited text no. 3
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4.Lorenzen JC, Godballe C, Kerndrup GB. Lipoblastoma of the neck: A rare cause of respiratory problems in children. Auris Nasus Larynx 2005;32:169-73.  Back to cited text no. 4
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5.Arda IS, Senocak ME, Gogus S, Buyukpamukcu N. A case of benign intrascrotal lipoblastoma clinically mimicking testicular torsion and review of the literature. J Pediatr Surg 1993;28:259-61.  Back to cited text no. 5
    
6.Mentzel T, Calonje E, Fletcher CD. Lipoblastoma and lipoblastomatosis: A clinicopathological study of 14 cases. Histopathology 1993;23:527-33.  Back to cited text no. 6
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7.Jung SM, Chang PY, Luo CC, Huang CS, Lai JY, Hsueh C. Lipoblastoma/lipoblastomatosis: A clinicopathologic study of 16 cases in Taiwan. Pediatr Surg Int 2005;21:809-12.  Back to cited text no. 7
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8.Coffin CM. Adipose and myxoid tumors. In: Coffin CM, Dehner LP, O'Shea PA, editors. Pediatric Soft Tissue Tumors: A Clinical, Pathological, and Therapeutic Approach. New Salt Lake City, Utah: JA Majors Company; 1997. p. 254-76.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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