Lymphoproliferative disorder of fetal origin presenting as oligohydramnios

M. Fritsch, E. S. Jaffe, C. Griffin, J. Camacho, M. Raffeld, Douglas W. Kingma*

*Corresponding author for this work

Research output: Contribution to journalArticle

Abstract

Lymphoma involving the placenta or fetus remains a very rare event. All cases reported to date have shown the lymphoma cells to be of maternal origin in that the tumor cells have preferentially involved the intervillous spaces with sparing of the villi and fetal circulation. We report a novel case of a monoclonal primary placental Epstein-Barr virus (EBV)-associated B-cell lymphoma of fetal origin. The placenta of a 20-week stillborn fetus born to a 19-year-old gravida 1 para 0 woman, presenting with oligohydramnios, showed a large cell infiltrate confined within villi and sparing the intervillous spaces, indicative of preferential involvement of the fetal circulation. Necropsy did not show any other site of involvement by malignant lymphoma or other abnormalities. Immunophenotypic studies showed the tumor cells to be of B-cell phenotype with a relatively high proliferation rate. EBV EBER1 RNA was identified in more than 95% of tumor cells, and polymerase chain reaction studies showed EBV EBNA1 strain type A and wildtype EBV LMP1. Analysis of the immunoglobulin heavy chain by polymerase chain reaction showed a monoclonal B-cell population. In situ hybridization studies using a commercially available probe directed at repeated sequences on the human Y chromosome showed a single intense signal within trophoblastic epithelium and lymphoma cells, indicative of male origin. The mother remains in good health 11 months after delivery.

Original languageEnglish (US)
Pages (from-to)595-601
Number of pages7
JournalAmerican Journal of Surgical Pathology
Volume23
Issue number5
DOIs
StatePublished - May 1 1999

Keywords

  • Epstein-Barr Virus
  • In situ hybridization
  • Lymphoproliferative disease
  • Placenta

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

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    Fritsch, M., Jaffe, E. S., Griffin, C., Camacho, J., Raffeld, M., & Kingma, D. W. (1999). Lymphoproliferative disorder of fetal origin presenting as oligohydramnios. American Journal of Surgical Pathology, 23(5), 595-601. https://doi.org/10.1097/00000478-199905000-00015