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LETTER TO THE EDITOR
Year : 2014  |  Volume : 9  |  Issue : 3  |  Page : 300-301
 

Primary spinal extradural lipoblastoma: Rare occurrence


1 Department of Neurosurgery, LTMMMC, Sion, Mumbai, Maharashtra, India
2 Department of General Medicine, Zynova Hospital, Mumbai, Maharashtra, India

Date of Web Publication23-Dec-2014

Correspondence Address:
Sachin A Giri
c/o Mr. Jitendra Goswami, 702, Unique Tower, Bhaktidham Temple Marg, Sion Chunabhatti, Mumbai - 400 022, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1817-1745.147604

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How to cite this article:
Giri SA, Diyora B, Giri D, Sharma A. Primary spinal extradural lipoblastoma: Rare occurrence. J Pediatr Neurosci 2014;9:300-1

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Giri SA, Diyora B, Giri D, Sharma A. Primary spinal extradural lipoblastoma: Rare occurrence. J Pediatr Neurosci [serial online] 2014 [cited 2019 Oct 21];9:300-1. Available from: http://www.pediatricneurosciences.com/text.asp?2014/9/3/300/147604


Dear Sir,

Tumors arising from adipocytes comprise around 6% of all soft tissue tumors. Of these around 94% are lipomas, 4.7% are lipoblastomas and 1.7% are liposarcomas. [1] Lipomas and lipoblastomas are benign tumors of mesenchymal origin in contrast to liposarcoma that is a malignant tumor. Atypical lipomatous lesion arising from embryonal fat cells is termed as lipoblastoma. Lipoblastomas are classified into two varieties: Lipoblastoma, which is well-encapsulated, well-circumscribed lesion, and lipoblastomatosis that is nonencapsulated, multicentric infiltrative tumor. It is a tumor of pediatric age group with a higher incidence in males. Lipoblastomas most commonly occur in extremities followed by trunk, abdomen and head and neck. [2] Jung et al. found a higher incidence in head and neck. [3] Spine and spinal cord involvement in lipoblastoma in most of the cases is secondary, and that may be from neck lesion, mediastinal lesion or from the pelvis and may result in neurological deficit. Though there are various reports of lipoblastoma with spinal extension, we have not found any case of primary spinal lipoblastoma.

A 1-year-old male child presented to us with delayed milestones, inability to move lower limbs since 6 months and difficulty in defecation and urination since 6 months. On examination, there was wasting of lower limb muscles. Tone in upper limbs was normal while there was hypotonia in both lower limbs. Power in lower limbs was grade 0; there was areflexia in both lower limbs and plantar reflex was absent. Furthermore, there was absence of sensation below the waist.

Magnetic resonance imaging (MRI) study of dorsolumbar spine revealed 8 cm × 4 cm size extradural lesion, which was isointense on T1 weighted image (T1WI) [Figure 1] and hyperintense on T2 weighted image (T2WI) extending from D8 to L2 vertebra with displacement of cord towards right side. Lesion was infiltrating the L2 vertebra and extending through the neural foramen into paravertebral space. The lesion was heterogeneously enhancing on gadolinium administration
Figure 1: T1WI axial cuts showing dumbbell shaped iso-intense lesion extending into left paraspinal space

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Patient underwent D10 to L2 laminotomy in the prone position. Dura was intact. Extradural lesion was noticed which was yellowish, well encapsulated. It was soft to firm, minimally vascular and removed with microsurgical techniques. Part of lesion was eroding the pars interarticularis of L2 vertebra and extending into paraspinal space that was dissected out through same incision and excised. Dura was pulsatile at the end of surgery. Laminoplasty done.

Histopathological examination showed well circumscribed lesion composed of cells arranged in lobules separated by septae. It comprised of stellate mesenchymal cells and vacuolated lipoblast. The cells had abundant clear cytoplasm, and stroma was myxoid [Figure 2]. Patient was discharged on postoperative day 10 without any complication. However, there was no clinical improvement in lower limb power at 6 months follow-up.
Figure 2: HPR confirms lipoblastoma

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Term lipoblastoma was proposed by Jaffe in 1926. [4] It is a tumor of pediatric age group with 55% of cases diagnosed before 1 year of age and almost all reported cases diagnosed in the first decade of life. [5] Occurrence of lipoblastoma in adults is rare. Male:Female incidence is 3.8:1. [6] Lipoblastomas most commonly occur in extremities (60%) followed by trunk (15%), abdomen (14%) and head and neck (14%). [2] However, Jung et al. found a higher incidence in head and neck. [3] Lipoblastomas show rearrangement in chromosome 8q11-13. [7] Rapid growth of lipoblastoma can give rise to compressive symptoms.

On radiological examination, lipoblastoma appears as nonspecific soft tissue mass without bone erosion. [7] MRI is an investigation of choice that classically shows high signal intensity on T1WI and T2WI identical to fat. [1] However, increased cellularity secondary to myxoid and mesenchymal component may lead to lower T1 weighted signals. [8]

Grossly size of lipoblastoma varies significantly. It is pale yellow lobulated lesion which is soft in consistency with moderate vascularity. Differential diagnosis includes lipoma, hibernoma, liposarcoma. [9] Lipoma is the closest differential of lipoblastoma and can be differentiated by presence of abundant vacuolation in cytoplasm. [10] In lipoblastoma, there is varying degree of cellular differentiation. Hibernoma is completely formed of brown fat with a central nucleus. [9] There is abundant finely granular cytoplasm. Apart from cellular atypia and hyperchromasia, liposarcoma can be differentiated by prominence of plexiform capillaries. [9] There is the absence of lobulation in liposarcoma.

Complete surgical excision is the treatment of choice in lipoblastoma. Incomplete resection is significantly associated with recurrence. Lipoblastoma arising from back and neck have a significant risk of recurrence.

In our case, there is primary involvement of the spine with spread to the para spinal region through eroded pars interarticularis giving rise to dumbbell configuration. The erosion of pars interarticularis secondary to mass effect and spread to para spinal region is uncommon in lipoblastoma. T1 weighted MRI showed decreased signal intensity which is unusual for tumor arising from adipocytes. This decreased signal intensity can be explained by occasional increased cellularity, that is, myxoid and mesenchymal components of lipoblastoma.

Lipoblastoma is rare benign tumor of adipocytes. Preoperative diagnosis may be difficult due to varying picture on imaging. Though benign lesion, it can cause dysfunction of the organ by its mass effect. It can erode the bone and can spread to adjacent planes. Complete excision of the tumor not only clinches the diagnosis but also prevent recurrence.

 
   References Top

1.
Gardikis S, Kambouri K, Tsalkidis A, Chatzimicael A, Vaos G. Lipoblastoma on the posterior side of the neck. Turk J Pediatr 2009;51:287-9.  Back to cited text no. 1
    
2.
Arda IS, Senocak ME, Gögüs S, Büyükpamukçu 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. 2
    
3.
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. 3
    
4.
Jaffe RH. Recurrent lipomatous tumors of the groin: Liposarcoma and lipomapseudomyxomatodes. Arch Pathol 1926;1:381-7.  Back to cited text no. 4
    
5.
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. 5
    
6.
Collins MH, Chatten J. Lipoblastoma/lipoblastomatosis: Aclinicopathologic study of 25 tumors. Am J Surg Pathol 1997;21:1131-7.  Back to cited text no. 6
    
7.
Rasalkar DD, Chu WC . Lipoblastomatous tumours, a rare entity: Report of two cases in children Hong Kong J Radiol 2010;13:209-12.  Back to cited text no. 7
    
8.
Wang SF, Chang CY, Wu HD. Lipoblastomatosis of the shoulder: Unusual MR appearance. Br J Radiol 1998;71:884-5.  Back to cited text no. 8
    
9.
Brodsky JR, Kim DY, Jiang Z. Cervical lipoblastoma: Case report, review of literature, and genetic analysis. Head Neck 2007;29:1055-60.  Back to cited text no. 9
    
10.
López-Ferrer P, Jiménez-Heffernan JA, Yébenes L, Vicandi B, Viguer JM. Fine-needle aspiration cytology of lipoblastoma: A report of two cases. Diagn Cytopathol 2005;32:32-4.  Back to cited text no. 10
    


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