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Year : 2010  |  Volume : 53  |  Issue : 4  |  Page : 833-834
Lipoblastomatosis: A rare lesion


1 Department of Pathology, Pt. B. D. Sharma P. G. I. M. S, Rohtak, India
2 Department of Paediatric Surgery, Pt. B. D. Sharma P. G. I. M. S, Rohtak, India

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Date of Web Publication27-Oct-2010
 

How to cite this article:
Chhabra S, Kalra R, Singh S, Rattan K N. Lipoblastomatosis: A rare lesion. Indian J Pathol Microbiol 2010;53:833-4

How to cite this URL:
Chhabra S, Kalra R, Singh S, Rattan K N. Lipoblastomatosis: A rare lesion. Indian J Pathol Microbiol [serial online] 2010 [cited 2020 Dec 3];53:833-4. Available from: https://www.ijpmonline.org/text.asp?2010/53/4/833/72071


Lipoblastomatosis is quite a rare neoplasm, deriving from embryonic white fatty tissues and seen in infancy and early childhood. Lipoblastoma and lipoblastomatosis refer to circumscribed and diffuse forms of the same tumor. The lesion differs from lipoma and lipomatosis by its cellular immaturity and close resemblance to the myxoid form of liposarcoma. [1]

A 5-year-old female child presented with a slow-growing painless lump in the right gluteal region. Computed tomography (CT) revealed a 10 cm x 8 cm, infiltrative soft tissue mass with the density of adipose tissue. Excision biopsy of the lump was performed and the specimen was submitted for histopathological examination.

Grossly, a poorly circumscribed tumor mass measuring 8 cm in diameter was seen. The cut-surface was lobulated, pale yellow and gelatinous. Multiple small cysts filled with mucoid material were identified.

Microscopically, the hematoxylin and eosin section showed small irregular lobules of immature fat cells separated by connective tissue septa of varying thickness along with mesenchymal areas with loose myxoid appearance [Figure 1]. The lobules were composed of lipoblasts in various stages of maturation, ranging from primitive stellate and spindle-shaped cells to multivacuolated and univacuolated signet ring cell adipocytes. Supporting extracellular stromal tissue was myxoid and showed prominent plexiform vascular pattern. Mitotic figures were not seen [Figure 2]. A histopathological diagnosis of lipoblastomatosis was made.
Figure 1: Photograph showing lobules of immature fat cells separated by connective tissue septa (H and E, ×100)

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Figure 2: Photograph showing lipoblasts in varying stages of maturation and supporting stromal tissue (H and E, ×400)

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Lipoblastomatosis is an uncommon benign mesenchymal tumor that usually occurs in infants and children. [2] It is found most commonly in the subcutis of the upper and lower extremities, other locations being head and neck, mediastinum, retroperitoneum, trunk, lung, mesentery, peritoneal cavity and intramuscular and inguinal canals. [3] They are poorly circumscribed, benign lesions that may grow very fast, attain large size and become painless masses compressing the adjacent structures.

Jaffe described it to be a developmental anomaly characterized by continued proliferation of lipoblasts in the postnatal period. [4]

Although CT shows the tumor to be of fatty origin, the preoperative differential diagnosis between lipoma, liposarcoma and myxoliposarcoma is quite difficult. [1]

Clinically, differentiation from liposarcoma can be suspected from age of the patient. Lipoblastomatosis occurs exclusively in infants and children while liposarcoma is a disease of adults and is rare in children. Histologically, distinction is based on clinical character of the tumor, its invasion of soft tissues microscopically and absence of atypical mitotic figures and hyperchromatic bizzare nuclei. [5]

Whenever a tumor of such morphology resembling myxoid liposarcoma superficially is seen in children, the possibility of lipoblastomatosis should always be considered before labelling it as a sarcomatous process.

Clinical course is benign. Recommended treatment is local excision. Recurrence is unusual and appears to be related to incomplete excision. Metastasis/malignant transformation have not been encountered. Prognosis is excellent. [1] However, recurrent lesions are best imaged by magnetic resonance imaging to assess the extent of tumor and to plan for reconstruction, if needed.

 
   References Top

1.Dogan RZ, Kara M, Firat P, Gedikoglu G. An unusual tumor of the neck and mediastinum: Lipoblastomatosis resulting in paraparesis. Eur J Cardiothorac Surg 2007;31:325-7.  Back to cited text no. 1
    
2.Chang PF, Teng RJ, Tsou Yau KI, Chen CL, Chen CC. Lipoblastomatosis in a newborn: Case report. Pediatr Surg Int 1990;12:71-2.  Back to cited text no. 2
    
3.Ang DC, Chekmareva MA, Ke Y. A 14 year-old girl with a retroperitoneal soft tissue mass. Arch Pathol Lab Med 2005;129:176-8.  Back to cited text no. 3
    
4.Jaffe RH. Recurrent lipomatous tumors of the groin: Liposarcoma and lipoma pseudomixomatodes. Arch Pathol 1926;1:381-7.  Back to cited text no. 4
    
5.Langloh JT, Reing CM, Chun BK, Grant E. Lipoblastomatosis: A case report. J Bone Joint Surg Am 1978;60:130-2.  Back to cited text no. 5
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Correspondence Address:
Sonia Chhabra
Department of Pathology, Pt. B. D. Sharma P. G. I. M. S, Rohtak
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.72071

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    Figures

  [Figure 1], [Figure 2]

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