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Renal Medullary Carcinoma

  • Reference work entry
  • First Online:
Uropathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 9 Accesses

Definition

A highly aggressive carcinoma centered on the renal medulla associated with sickle cell and related hemoglobinopathies (Davis et al. 1995).

Clinical Features

  • Incidence

    Renal medullary carcinoma is a very uncommon tumor.

  • Age

    There is a predilection for young adults.

  • Sex

    There is a remarkable male predominance.

  • Site

    By definition, it is centered on the renal medulla.

  • Treatment

    Radical nephrectomy is the standard treatment.

  • Outcome

    Renal medullary carcinoma is an aggressive tumor. All patients are symptomatic at diagnosis with flank or abdominal pain and gross hematuria, with metastatic disease at the time of diagnosis.

Macroscopy

These tumors are poorly circumscribed medulla-centered and variable in size.

Microscopy

Architecturally, the most common patterns are reticular and cribriform glands; however many other patterns have been seen. The stroma present in the neoplasia is fibrotic/desmoplastic with intratumoral neutrophil predominant inflammatory infiltrates....

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References and Further Reading

  • Amin, M. B., Smith, S. C., Agaimy, A., et al. (2014). Collecting duct carcinoma versus renal medullary carcinoma: An appeal for nosologic and biological clarity. The American Journal of Surgical Pathology, 38, 871–874.

    Article  PubMed  Google Scholar 

  • Davis, C. J., Jr., Mostofi, F. K., & Sesterhenn, I. A. (1995). Renal medullary carcinoma. The seventh sickle cell nephropathy. The American Journal of Surgical Pathology, 19, 1–11.

    Article  PubMed  Google Scholar 

  • Ohe, C., Smith, S. C., Sirohi, D., et al. (2018). Reappraisal of morphologic differences between renal medullary carcinoma, collecting duct carcinoma, and fumarate hydratase-deficient renal cell carcinoma. The American Journal of Surgical Pathology, 42, 279–292.

    Article  PubMed  Google Scholar 

  • Sirohi, D., Smith, S. C., Ohe, C., et al. (2017). Renal cell carcinoma, unclassified with medullary phenotype: Poorly differentiated adenocarcinomas overlapping with renal medullary carcinoma. Human Pathology, 67, 134–145.

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Diagnostic and Public Health, Section of Pathology, University of Verona, Verona, Italy

    Anna Caliò & Guido Martignoni

  2. Department of Pathology, Pederzoli Hospital, Peschiera del Garda, Italy

    Diego Segala & Guido Martignoni

Authors
  1. Anna Caliò
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  2. Diego Segala
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  3. Guido Martignoni
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Corresponding author

Correspondence to Guido Martignoni .

Editor information

Editors and Affiliations

  1. Histopathology and Molecular Diagnostics, University Hospital Careggi, Florence, Italy

    Maria Rosaria Raspollini

  2. Pathology Service, Champalimaud Clinical Center, Lisbon, Portugal

    Antonio Lopez-Beltran

  3. Unit of Anatomic Pathology, Department of Surgery, Cordoba University Medical School, Cordoba, Spain

    Antonio Lopez-Beltran

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Cite this entry

Caliò, A., Segala, D., Martignoni, G. (2020). Renal Medullary Carcinoma. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4924

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  • DOI: https://doi.org/10.1007/978-3-030-41894-6_4924

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-41893-9

  • Online ISBN: 978-3-030-41894-6

  • eBook Packages: MedicineReference Module Medicine

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