CEN Case Reports

, Volume 7, Issue 1, pp 121–126 | Cite as

Renal medullary carcinoma: a nearly fatal malignancy specifically affecting patients with a so-called benign condition

  • Yamila Goenaga-Vázquez
  • Gloria Colón
  • Nilka Barrios
  • María Correa
Case Report
  • 39 Downloads

Abstract

Renal medullary carcinoma (RMC) is a highly aggressive and rare malignancy found almost exclusively in young patients with sickle cell trait (SCT). Metastatic disease is commonly present at diagnosis. There is very limited experience treating disseminated disease and the prognosis is dismal. We report the case of a young 9-year-old boy with SCT, who presented with 4 months’ progression of abdominal pain, nausea and vomiting associated with cough spells, dysphagia, and weight loss. Upon evaluation, he was underweight, pale, and in mild respiratory distress. Cervical lymphadenopathy was evident and abdomen was diffusely tender. A whole-body CT scan showed a left kidney lesion with associated cervical, mediastinal, and retroperitoneal lymphadenopathy. Biopsy of a cervical lymph node revealed metastatic RMC. Patient was started on combination chemotherapy with paclitaxel, carboplatin, and gemcitabine followed by left adrenalectomy. In spite of having advanced disease, our patient achieved an excellent response with a progression-free survival of 17 months. Although SCT is thought to be a “benign” condition, RMC is one devastating complication associated with it. Considering its rarity, the near uniform associated fatality should prompt the question of whether clinical practice should change regarding proper counseling of these patients and raise awareness in the medical community.

Keywords

Renal medullary carcinoma Sickle cell disease Sickle cell trait Sickling Hematuria Kidney medulla Hemoglobinopathies 

Notes

Acknowledgements

We declare no funding source for our work.

Author contributions

YGV did the literature review, obtained the radiologic images, and wrote the article. GC and NB contributed to the literature review and to the correction of the first draft. MC provided the pathology images and helped to correct the first draft. All authors were directly involved in the care of the patient.

Compliance with ethical standards

Conflict of interest

All the authors have declared no competing interest.

Human and animal rights

This article does not contain any studies with human participants or animals performed by any of the authors.

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Copyright information

© Japanese Society of Nephrology 2018

Authors and Affiliations

  • Yamila Goenaga-Vázquez
    • 1
  • Gloria Colón
    • 2
  • Nilka Barrios
    • 2
  • María Correa
    • 3
  1. 1.Department of Pediatrics, Medicine-Pediatrics Residency ProgramUniversity of Puerto Rico Medical Sciences CampusSan JuanUSA
  2. 2.Division of Pediatric Hematology Oncology, Department of Pediatrics, University Pediatric HospitalUniversity of Puerto Rico, Medical Sciences CampusSan JuanUSA
  3. 3.Department of Pathology, School of MedicineUniversity of Puerto Rico, Medical Sciences CampusSan JuanUSA

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