Renal medullary carcinoma: Prolonged remission with chemotherapy, immunohistochemical characterisation and evidence of bcr/abl rearrangement

Authors

  • Jens Stahlschmidt MD,

    1. Department of Histopathology, St. James's University Hospital, Leeds, United Kingdom
    Search for more papers by this author
  • Catherine Cullinane MB, BAO, BCh,

    1. Department of Histopathology, St. James's University Hospital, Leeds, United Kingdom
    Search for more papers by this author
  • Paul Roberts BSc(HONS),

    1. Yorkshire Regional Cytogenetics Unit, St. James's University Hospital, Leeds, United Kingdom
    Search for more papers by this author
  • Susan V. Picton FRCPCH

    Corresponding author
    1. Department of Paediatric Oncology, St. James's University Hospital, Leeds, United Kingdom
    • Department of Paediatric Oncology, Children's Day Hospital, St. James's University Hospital, Beckett Street, Leeds LS9 7TF, United Kingdom
    Search for more papers by this author

Abstract

Background

Renal medullary carcinoma (RMC), an extremely rare tumour of the kidney, carries a dismal prognosis, with no reports to date of significant response to chemotherapy or radiotherapy. A case of this tumour in a male child, who showed a dramatic response to chemotherapy, is described.

Procedure

A detailed histological evaluation of the tumour and cytogenetic analysis using fluorescent in situ hybridisation (FISH) was carried out. The child was treated with multiagent chemotherapy, followed by abdominal radiotherapy.

Results

A detailed histopathological and immunohistochemical portrait of this tumour is described, and FISH studies confirmed the presence of a bcr/abl rearrangement. The child obtained complete radiological remission following chemotherapy, although he later relapsed and died of progressive disease despite further attempts at treatment with chemotherapy.

Conclusions

Although there are no previous reports of response of this tumour to chemotherapy, this case illustrates that treatment of this disease is justified. The responses of other cases to similar drug regimens would be of interest to confirm whether the encouraging response described for this case could be reproduced. Cytogenetic analysis of other cases of RMC may clarify whether the abnormalities seen in this case are typical. Med. Pediatr. Oncol. 33:551–557, 1999. © 1999 Wiley-Liss, Inc.

Ancillary