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Infantile lipofibromatosis of the upper limb

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Abstract

The imaging features of extensive lipofibromatosis presenting in a 1-day-old female infant are reported. This lesion involved her entire right upper limb, extending from the axilla to the palm of the hand. Radiographs showed marked deformity and thinning of all the right upper-limb bones due to pressure effect of soft-tissue enlargement, especially affecting the distal humerus and proximal forearm bones. Magnetic resonance imaging showed a huge soft-tissue mass infiltrating most of the muscles of the entire upper limb, with bony erosion. The mass was largely T1-isointense, moderately T2-hyperintense and showed marked enhancement. There were intra-lesional signal changes consistent with fatty elements. A lesion debulking procedure was performed and the histology was that of lipofibromatosis. The limb was found to be non-viable after the procedure and a subsequent above-elbow amputation was performed. Although the resection margins were not clear, she had no further recurrence over a subsequent 3-year follow-up period.

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References

  1. Fetsch JF, Miettinen M, Laskin WB, Michal M, Enzinger FM. A clinicopathologic study of 45 pediatric soft tissue tumors with an admixture of adipose tissue and fibroblastic elements, and a proposal for classification as lipofibromatosis. Am J Surg Pathol 2000; 24:1491–1500

    Article  PubMed  CAS  Google Scholar 

  2. Eich GF, Hoeffel JC, Tschappeler H, Gassner I, Willi UV. Fibrous tumors in children: imaging features of a heterogeneous group of disorders. Pediatr Radiol 1998; 28:500–509

    Article  PubMed  CAS  Google Scholar 

  3. Murphey MD, Carroll JF, Flemming DJ, Pope TL, Gannon FH, Kransdorf MJ. From the archives of the AFIP: benign musculoskeletal lipomatous lesions. Radiographics 2004; 24:1433–1466

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Diagnostic Imaging, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, 229899, Singapore, Singapore

    Harvey E. L. Teo & Wilfred C. G. Peh

  2. Department of Paediatric Medicine, KK Women’s and Children’s Hospital, Singapore, Singapore

    Mei-Yoke Chan

  3. Department of Pathology, Tan Tock Seng Hospital, Singapore, Singapore

    Norman Walford

Authors
  1. Harvey E. L. Teo
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  2. Wilfred C. G. Peh
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  3. Mei-Yoke Chan
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  4. Norman Walford
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Corresponding author

Correspondence to Wilfred C. G. Peh.

Additional information

This case (case number 16) was presented at the 31st Closed Meeting of the International Skeletal Society held in Malta, October 2004

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Teo, H.E.L., Peh, W.C.G., Chan, MY. et al. Infantile lipofibromatosis of the upper limb. Skeletal Radiol 34, 799–802 (2005). https://doi.org/10.1007/s00256-005-0937-4

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  • DOI: https://doi.org/10.1007/s00256-005-0937-4

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