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

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.

• Authors: J Stahlschmidt, C Cullinane, P Roberts, S V Picton
• Journal: Medical and pediatric oncology (Med Pediatr Oncol) Vol. 33 Issue 6 Pg. 551-7 (Dec 1999) ISSN: 0098-1532 [Print] United States
• PMID: 10573578 (Publication Type: Case Reports, Journal Article, Review)
• Copyright: Copyright 1999 Wiley-Liss, Inc.

Chemical References

• Fusion Proteins, bcr-abl

Topics

• Adolescent
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Carcinoma, Medullary (diagnostic imaging, drug therapy, genetics, metabolism, pathology)
• Fatal Outcome
• Fusion Proteins, bcr-abl (genetics)
• Humans
• Immunohistochemistry
• In Situ Hybridization, Fluorescence
• Karyotyping
• Kidney Neoplasms (diagnostic imaging, drug therapy, genetics, metabolism, pathology)
• Male
• Remission Induction
• Tomography, X-Ray Computed
• Translocation, Genetic (genetics)