|Year : 2015 | Volume
| Issue : 4 | Page : 1025
An unusual case of cervical lipoblastoma with review of literature
K Premkumar1, Mheboob Aalam V Basle2, K Jassim3, TP Waseem Ahamed4
1 Department of Neurosurgery, Muslim Educational Society Medical College Hospital, Perinthalmanna, Kerala, India
2 Department of Paediatrics, Muslim Educational Society Medical College Hospital, Perinthalmanna, Kerala, India
3 Department of Radiodiagnosis, Muslim Educational Society Medical College Hospital, Perinthalmanna, Kerala, India
4 Department of Surgery, Muslim Educational Society Medical College Hospital, Perinthalmanna, Kerala, India
|Date of Web Publication||15-Feb-2016|
Mheboob Aalam V Basle
Department of Pediatrics, MuslimEducational Society Medical College Hospital, Perinthalmanna Kerala
Source of Support: None, Conflict of Interest: None
Lipoblastoma is a rare, benign, soft tissue neoplasm most commonly seen in children less than 3 years. It is usually seen on the trunk and on the limbs and rarely in the head and neck. In our case, a child of age 1 year and 3months presented with a swelling over the nape of the neck. The swelling had rapidly increased in size and was associated with difficulty in neck movements. Intraoperatively it was found to have very minimal adhesions to the underlying muscles of neck. Histopathological examination confirmed it to be a lipoblastoma. The swelling was removed by wide excision, and there was no postoperative complication.
Keywords: Head and neck region, lipoblastoma, magnetic resonance imaging, total excision
|How to cite this article:|
Premkumar K, Basle MV, Jassim K, Waseem Ahamed T P. An unusual case of cervical lipoblastoma with review of literature. J Can Res Ther 2015;11:1025
| > Introduction|| |
Lipoblastoma is a benign neoplasm of fetal white fat tissue, which occurs usually in infants and children less than 3 years. Less than 100 cases of lipoblastoma have been reported in literature till now, with involvement of head and neck being reported in very few cases. We are reporting a case of lipoblastoma arising from the nape of the neck, which is still rare, and such a huge and extensive tumor has not been reported yet.
| > Case Report|| |
Case report of lipoblastoma of posterior neck
A 1 year and 3-month-old male child was brought by his parents with complaints of a huge swelling over the back of the neck. They had noticed a small swelling at birth, but during the last 6 months there was a rapid increase in size. It was not associated with pain or fever. No history of trauma. The child was able to walk and play without any difficulty, but had restriction of neck movement. Over the last 4 months he developed a nonhealing ulcer over the surface of the swelling due to repeated friction. There was no significant medical or surgical history or any similar condition in the family.
The swelling was found to extend from the posterior hair line to the base of the neck. It was diffuse and trilobed [Figure 1] and [Figure 2] extending from posterior inferior occipital protuberance upto the base of the neck. Overlying skin showed dilated veins and an ulcer with well-defined borders. On palpation, the swelling was firm, non-fluctuant, nonreducible, mobile in the vertical plane; but immobile in the horizontal plane. Transillumination test was negative and the three swellings measured 15 × 10,5 × 7,5 × 8 cm in size. Flexion, extension, and the sideways movements of the neck were found to be restricted.
|Figure 2: Magnetic resonance imaging finding showing hypointense T1-weighted images|
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Magnetic resonance imaging (MRI) revealed that the lesion was predominantly fat-containing with significant septations and heterogeneity, which is an uncommon finding in a lipoma. The Magnetic resonance imaging findings of an uncomplicated lipoma usually show pure homogenous fat signal and thin septations (if present). Magnetic resonance imaging demonstrated that the lesion insinuated the planes of the posterior spinal muscles (particularly the trapezius, semispinalis, and splenius muscles). However, there was no evidence of intraspinal extension or spinal dysraphism [Figure 2].
In view of minimal adherence to the underlying muscles, we did a wide excision of the lesion with 2 cm clear margin. Peroperatively, the lesion was found to be well-encapsulated [Figure 3]. The postoperative period was uneventful. The histopathological examination showed well-encapsulated, mature and immature fat cells in lobules with thin fibrous stroma and myxoid cytoplasm. The child is under regular follow-up over the last 1 year, and has not showed any complications or recurrence.
| > Discussion|| |
Jaffe coined the term “lipoblastoma” in 1926 to describe an atypical lipomatous lesion that consisted of cells resembling embryonic white fat. This term was intended to differentiate these lesions from common lipomas, which contain no lipoblasts. Lipoblastomas are predominantly well-circumscribed; however, they can also be diffuse (a condition called lipoblastomatosis), and thus more difficult to remove completely. Lipoblastoma is a rare benign tumor arising from embryonic white fat which occurs in the early childhood. It usually arises on the extremities. To date, very few cases of cervical lipoblastomas have been described in the English literature. Chung and Enzinger described two forms–the term lipoblastoma for the localized circumscribed type and lipoblastomatosis for the diffuse multicentric variant. It occurs exclusively in infants and children, with 90% of the cases in children <3 years of age. It shows a male preponderance (3:1). Lipoblastomas commonly arise from the extremities (36-72%) and the trunk (20-50%), and less commonly in the head and neck (10-l5%). As mentioned above, very few cases of lipoblastomas in head and neck region have been reported in literature. Dilley et al., had reviewed 24 patients that presented to a single institution over a 15-year period. Of these 24 patients, only 4 had tumor in the head and neck region. The tumor may present as a focal or diffuse lesion with the former being more common. It has also been mentioned that focal lesions usually do not require further surgery following initial resection. Patients with lipoblastoma may present with various symptoms depending upon its site. Rasmussen et al., mentioned a case of cervical lipoblastoma causing intermittent airway obstruction. Farrugia et al. reported another case of lipoblastoma from the neck which had presented as a rapidly enlarging mass, mimicking cystic hygroma. In our case it was located over the nape of the neck. Recent cytogenetic analysis revealed specific chromosomal abnormalities in adipose tissue tumors, which may aid in accurate diagnosis. Cytogenetic breakpoint abnormalities occur consistently in the chromosome 8q11-13 in lipoblastomas affecting PLAG1. Lipoblastoma exhibits a tendency to invade locally. If not excised early, it may enlarge, and infiltrate the various surrounding structures as seen in this case, and various pressure symptoms may occur which can lead to life-threatening complications. It is one of the few cases of a lipoblastoma arising from the nape of neck, which has not been reported in the literature till now.
| > References|| |
Jaffe RH. Recurrent lipomatous tumors of the groin: Liposarcoma and lipoma pseudomyxomatodes. Arch Pathol 1926;1:381-7.
Chung EB, Enzinger FM. Benign lipoblastomatosis: An analysis of 35 cases. Cancer 1973;32:482-92.
Dilley AV, Patel DL, Hicks MJ, Brandt ML. Lipoblastoma: Pathophysiology and surgical management. J Pediatr Surg 2001;36:229-31.
Rasmussen IS, Kirkegaard J, Kaasbol M. Intermittent airway obstruction in a child caused by a cervical lipoblastoma. Acta Anaesthesiol Scand 1997;41:945-6.
Ohjimi Y, Iwasaki H, Kaneko Y, Ishiguro M, Ohgami A, Kikuchi M. A case of lipoblastoma with t (3;8)(q12;q11.2). Cancer Genet Cytogenet 1992;62:103-5.
[Figure 1], [Figure 2], [Figure 3]