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Published on: October 31, 2012
Deforming lipoblastomatosis of the lower extremity
Claude Pierre-Jerome1, Deborah D Brahee, Norman W Kettner
1Department of Radiology, MRI Center, Ulleval University Hospital, Oslo, Norway.
Insights
Lipoblastomatosis, a rare pediatric tumor, requires surgical removal. While outcomes are typically good, recurrence is possible. This case highlights its presentation in a newborn.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Medical Imaging
Background:
- Lipoblastomatosis is an uncommon benign lipomatous tumor primarily affecting infants and children.
- It is characterized by extensive proliferation of mature and immature fat cells.
- This condition is virtually nonexistent in adults.
Purpose of the Study:
- To present a case of lipoblastomatosis in a newborn with extensive pelvic and lower extremity involvement.
- To discuss the clinical and radiological characteristics of this rare pediatric disorder.
- To emphasize the role of magnetic resonance imaging (MRI) in assessing the disease.
Main Methods:
- Case report of a 2-month-old male infant with a progressively enlarging pelvic and lower extremity deformity since birth.
- Clinical examination and radiological assessment, including MRI, were performed.
- No family history or other congenital abnormalities were noted.
Main Results:
- Surgical excision of the lipomatous neoplastic tissue is the primary treatment modality.
- Post-surgical outcomes are generally satisfactory, with complete removal of the tumor.
- Recurrence of lipoblastomatosis after surgical intervention has been documented in literature.
Conclusions:
- Lipoblastomatosis is a rare but significant diagnosis in pediatric patients presenting with soft tissue masses.
- Comprehensive clinical and radiological evaluation, particularly MRI, is crucial for diagnosis and management planning.
- While surgical excision offers a favorable prognosis, vigilant follow-up is necessary due to the potential for recurrence.
Objective:
Lipoblastomatosis is a rare disorder in infants and children and nonexistent in adults. We discuss a case of a newborn child with lipoblastomatosis extensively involving the pelvis and lower extremities. The clinical and radiological characteristics of the disorder are discussed.
Clinical Features:
A 2-month-old male had an enlarging deformity of the pelvis and lower extremities with progression of the condition from birth. There was no family history related to the disease. No other clinical abnormalities were present.
Intervention And Outcome:
Lipoblastomatosis is initially treated with surgical excision of the lipomatous neoplastic tissue. The postsurgical outcome is satisfactory; however, the likelihood of recurrence has been reported.
Conclusion:
We present the features of lipoblastomatosis, an uncommon disorder affecting infants and children. The clinical and radiologic manifestations of the disease are assessed with emphasis on magnetic resonance imaging.
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