Post-transplant lymphoproliferative disorder resembling Wilms tumor. Diagnostic dilemma

Renal biopsy or nephrectomy?

Edaire Cheng, Nicholas Fustino, Laura Klesse, Srinath Chinnakotla, Rinarani Sanghavi

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Post-transplant lymphoproliferative disorder is a life-threatening neoplasm that can occur after orthotopic liver transplant. We report a 14-month-old female status-post OLT with an atypical presentation of PTLD as a solitary renal mass. At eight-wk post-transplant, she presented with elevated transaminases, CMV counts (73 000 copies/mL), and EBV counts (35 000 copies/mL). CT scan revealed a solid heterogeneously enhancing right renal mass measuring 2.6 × 2.4 × 3.3 cm. The radiological diagnosis was Wilms tumor, although PTLD could not be excluded. Complete resection of a Wilms tumor is potentially curative. A needle biopsy would upstage the malignancy and result in radiochemotherapy that is deleterious to a liver graft. The mass was not amenable to partial nephrectomy. A total nephrectomy, given life-long nephrotoxic immunosuppressants, was an unfavorable option. Thus, needle biopsy was performed. Histology confirmed monoclonal, EBV-associated PTLD and diffuse large B-cell lymphoma. Her therapy included immunosuppression reduction, cyclophosphamide, steroids, and anti-CD20 monoclonal antibody. Concomitantly, she received Cytogam and gancyclovir. Complete remission was achieved three months after chemotherapy. This case illustrates that young age, CMV infection, and EBV infection are strong risk factors for PTLD. With such risk factors present, any mass or lesion in a solid organ transplant patient should be considered PTLD until proven otherwise.

Original languageEnglish (US)
JournalPediatric Transplantation
Volume15
Issue number8
DOIs
StatePublished - Dec 2011

Fingerprint

Wilms Tumor
Lymphoproliferative Disorders
Nephrectomy
Transplants
Kidney
Biopsy
Needle Biopsy
Human Herpesvirus 4
Ganciclovir
Epstein-Barr Virus Infections
Lymphoma, Large B-Cell, Diffuse
Liver
Chemoradiotherapy
Immunosuppressive Agents
Transaminases
Cyclophosphamide
Immunosuppression
Neoplasms
Histology
Steroids

Keywords

  • cytomegalovirus
  • Epstein-Barr virus
  • liver transplant
  • post-transplant lymphoproliferative disorder
  • Wilms tumor

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

Cite this

Post-transplant lymphoproliferative disorder resembling Wilms tumor. Diagnostic dilemma : Renal biopsy or nephrectomy? / Cheng, Edaire; Fustino, Nicholas; Klesse, Laura; Chinnakotla, Srinath; Sanghavi, Rinarani.

In: Pediatric Transplantation, Vol. 15, No. 8, 12.2011.

Research output: Contribution to journalArticle

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