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Imaging findings in children with proliferative disorders following multivisceral transplantation

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Abstract

Background

Multivisceral transplantation represents an important treatment option for children with intestinal failure. The attendant immunosuppression can lead to a spectrum of cellular proliferations including benign and malignant smooth muscle tumors and lymphoproliferative disorders, many related to cellular dysregulation from Epstein-Barr virus infection.

Objective

The purpose of this study is to investigate the rates of post-transplantation proliferative disorders among children with multivisceral transplantation and to characterize the imaging and pathological features of these disorders.

Materials and methods

We identified all consecutive children who underwent multivisceral transplant from August 2004 to October 2011 with at least 27 months of clinical and imaging follow-up. We reviewed medical records to determine the underlying causes of the multivisceral transplant, age at transplantation, onset of neoplasm development, and outcome. Two pediatric radiologists reviewed all imaging studies independently and diagnosis of disease was made by consensus interpretation. Pathological specimens were reviewed for histopathological findings of post-transplantation neoplasm in this pediatric patient population.

Results

The study population consisted of 14 consecutive pediatric patients (7 boys and 7 girls; mean age 26 months, range 4–113 months). Of these 14 children, 4 (29%) developed histologically confirmed post-transplant neoplasms at a mean time of 2.4 years after multivisceral transplantation. Types of neoplasms included post-transplant lymphoproliferative disorder (PTLD) in three (21%) and Epstein-Barr-virus-associated smooth muscle tumor in two (14%). (One child developed both neoplasms following transplantation). Both children with smooth muscle tumor associated with Epstein-Barr virus presented with characteristic hypointense solid masses with peripheral rim enhancement on cross-sectional imaging studies. The mortality rate of children who developed post-transplant neoplasms was higher than that of those who did not develop post-transplant neoplasm (50% vs. 10%, P = 0.17), suggesting a possible risk factor for death.

Conclusion

Post-transplant neoplasm in children with multivisceral transplantation occurs with high frequency, often presents as Epstein-Barr-virus-associated smooth muscle tumor showing characteristic peripheral rim enhancement on cross-sectional imaging studies.

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Authors and Affiliations

  1. Department of Radiology, Tufts Medical Center, Boston, MA, USA

    Anastasia L. Hryhorczuk

  2. Department of Surgery, Boston Children’s Hospital, Boston, MA, USA

    Heung Bae Kim

  3. Department of Pathology, Boston Children’s Hospital, Boston, MA, USA

    Marian H. Harris & Sara O. Vargas

  4. Department of Biostatistics, Boston Children’s Hospital, Boston, MA, USA

    David Zurakowski

  5. Departments of Radiology and Medicine, Boston Children’s Hospital and Harvard Medical School, 300 Longwood Ave., Boston, MA, 02115, USA

    Edward Y. Lee

Authors
  1. Anastasia L. Hryhorczuk
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  2. Heung Bae Kim
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  3. Marian H. Harris
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  4. Sara O. Vargas
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  5. David Zurakowski
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  6. Edward Y. Lee
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Correspondence to Edward Y. Lee.

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Hryhorczuk, A.L., Kim, H.B., Harris, M.H. et al. Imaging findings in children with proliferative disorders following multivisceral transplantation. Pediatr Radiol 45, 1138–1145 (2015). https://doi.org/10.1007/s00247-015-3303-2

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  • DOI: https://doi.org/10.1007/s00247-015-3303-2

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