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  • Perinatal/Neonatal Case Presentation
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Perinatal/Neonatal Case Presentation

Congenital high-grade sarcoma presenting as skin nodules and respiratory distress in a neonate

Journal of Perinatology volume 28, pages 160–162 (2008)Cite this article

Abstract

We report, to our knowledge, the first case of a congenital, widespread, aggressive high-grade sarcoma, presented as multiple skin nodules and respiratory distress in a neonate that had a t(9;22)(q22;q11–12) cytogenetic abnormality suggestive of a more indolent extraskeletal myxoid chondrosarcoma (EMC). EMC is generally thought of as a slow-growing tumor that presents between the fourth and sixth decades of life. Our patient was a 45,XY, t(13;14) newborn who presented at birth with subcutaneous nodules involving the face, scalp, back and extremities, as well as multiple intrathoracic, intraabdominal and intracranial masses. Diagnosis was made using electron microscopy and immunohistochemical and cytogenetic studies. Despite attempts to control rapid growth of lesions using high–dose steroids and cis-retinoic acid, patient's clinical status continued to deteriorate and life support was withdrawn at the 26 day of life.

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Acknowledgements

We thank thank Dr Brigitte Gomperts of the Division of Pediatric Hematology/Oncology at University of California Los Angeles for her expertise and help in management of the patient, and Drs Nagesh Rao and Sunita Bhuta of the Department of Pathology at University of California Los Angeles for cytogenetics and electron microscopy. MAT was supported by National Institutes of Health (NIH) Grants CA90571 and CA107300, by CMISE, a NASA URETI Institute (NCC 201364) and is a Scholar of the Leukemia and Lymphoma Society. VM is supported by NIH, National Institute of Child Health and Human Development (NICHD) Career Development Award (K12) K12HD034610-11.

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Authors and Affiliations

  1. Division of Neonatology and Developmental Biology, Department of Pediatrics, Institute for Stem Cell Biology and Medicine, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, USA

    J W Powers & V Milisavljevic

  2. Department of Pathology and Laboratory Medicine, Institute for Stem Cell Biology and Medicine, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, USA

    M Teitell

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  1. J W Powers
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Correspondence to V Milisavljevic.

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Powers, J., Teitell, M. & Milisavljevic, V. Congenital high-grade sarcoma presenting as skin nodules and respiratory distress in a neonate. J Perinatol 28, 160–162 (2008). https://doi.org/10.1038/sj.jp.7211890

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