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Table of Contents   
CASE REPORT
Year : 2023  |  Volume : 28  |  Issue : 5  |  Page : 431-432
 

Labial lipoblastoma in an infant: An uncommon tumor at an uncommon site


1 Department of Pathology, IPGME and R, Kolkata, West Bengal, India
2 Park Clinic, Kolkata, West Bengal, India

Date of Submission01-Apr-2023
Date of Decision31-May-2023
Date of Acceptance01-Jun-2023
Date of Web Publication05-Sep-2023

Correspondence Address:
Uttara Chatterjee
Department of Pathology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_70_23

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   Abstract 


Lipoblastoma and lipoblastomatosis are benign fatty tumors of soft tissues that are unique to childhood. Occurrence of lipoblastoma in the labial region is infrequent and can cause diagnostic dilemma. Awareness about this entity is important to avoid hazardous treatment in young children. Here, we present a case of labial lipoblastoma in a 2-year-old child along with its differential diagnosis.


Keywords: Labial tumors, lipoblastoma, pediatric age group


How to cite this article:
Mukherjee R, Bhattacharyya S, Chatterjee U. Labial lipoblastoma in an infant: An uncommon tumor at an uncommon site. J Indian Assoc Pediatr Surg 2023;28:431-2

How to cite this URL:
Mukherjee R, Bhattacharyya S, Chatterjee U. Labial lipoblastoma in an infant: An uncommon tumor at an uncommon site. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Oct 2];28:431-2. Available from: https://www.jiaps.com/text.asp?2023/28/5/431/385149





   Introduction Top


Lipoblastoma and lipoblastomatosis are benign adipocytic tumors seen in children <3 years of age.[1] Histological diagnosis of lipoblastoma is sometimes difficult because of its close resemblance with myxoid, round cell, and well-differentiated liposarcomas. Labial lipoblastoma is uncommon and to the best of our knowledge, only two cases have been reported so far.[2],[3] Here, we present a case of labial lipoblastoma in a 2-year-old child.


   Case Report Top


A 2-year-old girl was referred to the pediatric surgery department for labial swelling noted by the parents for the past 1 month. Her birth history was unremarkable. The physical examination revealed a 3.5 cm × 2 cm mobile, nontender, ovoid mass in the left labia majora. Left labial ultrasound revealed a circumscribed, solid mass with thin echogenic internal septations, and no visualized sinus tract. A decision for surgical resection was made and the mass was excised. During surgery, the mass was difficult to shell out from the deeper fascial plane and could only be removed piecemeal. The gross measurement of the excised mass was approximately 3.8 cm × 3.5 cm × 2.0 cm. It was bosselated, yellow-pink, mass. On microscopic examination, it showed an adipocytic tumor with lobular architecture. The lobules were separated by thick fibrovascular septa. Prominent myxoid areas were noted adjacent to the septae. There were adipocytes in various stages of maturation from stellate mesenchymal cells, lipoblasts to univacuolated adipocytic cells. The periphery of the lobules also showed plexiform vascular pattern. The fat cells showed a distinctive zonation with more immature myxoid cells at the periphery and adjacent to fibrous septa and mature adipocytes in the center of the lobule. There was no evidence of mitosis, necrosis, or pleomorphism [Figure 1]. Based on these typical features, a diagnosis of lipoblastoma was made. Six monthly follow-up was advised for the patient. After 6 months, no recurrence was noted at the tumor site.
Figure 1: (a) Gross photograph of the mass in left labia, (b) Gross photograph of the specimen, showing irregular lobules of fatty tissue, (c) Photomicrograph showing low power view of the tumor composed of lobules of adipocytes with prominent myxoid areas at the periphery (H and E, ×100); inset shows high power view of the same, (d) Postoperative photograph

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   Discussion Top


Adipocytic tumors account for only 6% of all soft-tissue tumors in children.[2] These mainly comprise lipoblastoma, liposarcoma, hibernoma, lipoma, and spinal lipomatous malformations. Lipoblastomas occur predominantly in infancy and early childhood, with 75%–90% of cases occurring before the age of 3 years.[1],[4] Lipoblastoma is a circumscribed lesion, and lipoblastomatosis is the diffuse form of the same.[1]

Lipoblastoma is a benign tumor thought to arise from embryonal white fat.[1] Lipoblastoma was first described by Jaffe in 1926 as a tumor of immature white fat.[5] It is found primarily in soft tissues of extremities and trunk; commonly upper extremities such as the axilla, shoulder, upper arm, elbow, and hand. Less common sites include the face, head-and-neck area, trunk, buttock, mesentery, mediastinum, labia, and retroperitoneum.[1],[2],[4] There is a slightly higher incidence in males.[1],[4] Usually, these are asymptomatic and detected as incidental findings. However, when deep-seated, these lipoblastomas can present with a wide range of symptoms. We observed in our previous series, lipoblastoma in the mediastinum presenting with features of respiratory distress and lipoblastoma in the mesentery can cause volvulus leading to acute abdomen.[6] The differential diagnosis of labial lipoblastoma in the pediatric age group includes hamartoma, hemangioma, lymphangioma, embryonal duct remnant, fibroepithelial polyp, granular cell tumor, and angiomyxoma.

Histology of this entity is quite distinctive and is usually not a problem to diagnose. Hence, awareness regarding the clinicopathological features of lipoblastoma is essential. On histological examination, lipoblastoma is a well-circumscribed lesion with a lobular pattern. It shows a spectrum of maturation from monovacuolated adipocytes to multivacuolated lipoblasts and a pattern of zonation with mature adipocytes toward the center.[1] Spindle- and stellate-shaped mesenchymal cells are seen in the background of the myxoid matrix. Lipoblastoma is known to mature over a period of time, as we have documented earlier.[7] The recurrence rate of lipoblastoma is unusually high, from 13% to 50%, probably due to incomplete excision.[1],[4],[6] It is more important in cases of lipoblastomatosis as there is an invasion into the surrounding tissue which leads to incomplete resection.[1]

Lipoblastomas typically exhibit a structural alteration of 8q11-q13 leading to the rearrangement of PLAG1, a developmentally regulated zinc finger transcription factor gene.[1],[5],[6] PLAG1 expression distinguishes lipoblastoma from other adipose tissue malformations, especially in resolving the dilemma between gradually maturing lipoblastoma and lipoma.

Treatment of lipoblastoma usually is surgical excision and regular follow-up owing to its propensity to recur.


   Conclusion Top


Labial lipoblastoma is an uncommon lesion and benign in nature. Therefore, awareness about this entity is important to avoid mutilating surgery and chemotherapy in young children.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Black JO, Bridge JA, Pedutor F. Lipoblastoma and lipoblastomatosis. In: Lokuhetty D, White VA, Cree IA, editors. WHO Classification of Soft Tissue and Bone Tumours. 5th ed. Lyon: IARC Press; 2020. p. 20-2.  Back to cited text no. 1
    
2.
Childress KJ, Hicks J, Wu H, Brandt ML, Adeyemi-Fowode OA. Lipoblastoma of the labia: A case report. J Pediatr Adolesc Gynecol 2016;29:e97-9.  Back to cited text no. 2
    
3.
Kirkham YA, Yarbrough CM, Pippi Salle JL, Allen LM. A rare case of inguinolabial lipoblastoma in a 13-month-old female. J Pediatr Urol 2013;9:e64-7  Back to cited text no. 3
    
4.
.Coffin CM, Lowichik A, Putnam A. Lipoblastoma (LPB): A clinicopathologic and immunohistochemical analysis of 59 cases. Am J Surg Pathol 2009;33:1705-12.  Back to cited text no. 4
    
5.
Jaffe R. Recurrent lipomatous tumour of the groin: Liposarcoma and lipoma pseudomyxomatodes. Arch Pathol 1926;11:381-7.  Back to cited text no. 5
    
6.
Ghosh P, Das RN, Ghosh R, Chatterjee U, Datta C, Mishra PK. Lipoblastoma and lipoblastomatosis: A clinicopathological study of six cases. J Cancer Res Ther 2015;11:1040.  Back to cited text no. 6
    
7.
Mitra J, Chatterjee U, Chatterjee SK. Lipoblastomatosis – A case report. Indian J Pathol Microbiol 2003;46:222-3.  Back to cited text no. 7
    


    Figures

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    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
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