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[Neuroendocrine tumors of the kidneys]

H Moch1

  • 1Institut für Klinische Pathologie, UniversitätsSpital Zürich, Schmelzbergstrasse 12, 8091, Zürich, Schweiz, holger.moch@usz.ch.

Der Pathologe
|April 23, 2015
PubMed

Insights

Primary renal neuroendocrine tumors, or carcinoids, share histological traits with those in other sites but are often misdiagnosed. Immunohistochemistry aids diagnosis, and WHO grading is recommended due to metastatic potential.

Area of Science:

  • Oncology
  • Pathology
  • Urology

Context:

  • The 2004 World Health Organization (WHO) classification categorizes renal carcinoid and neuroendocrine kidney cancers as primary renal neuroendocrine tumors.
  • Primary renal carcinoids exhibit histological similarities to neuroendocrine tumors in other anatomical locations.
  • These tumors are frequently misdiagnosed as other kidney cancers, including papillary renal cell carcinoma, mesonephric tumors, Wilms tumor (WT), and undifferentiated carcinoma.

Purpose:

  • To differentiate primary renal neuroendocrine tumors from other renal malignancies.
  • To highlight the diagnostic utility of immunohistochemical markers.
  • To recommend the application of the WHO classification system for neuroendocrine tumors to renal carcinoids.

Summary:

  • Immunohistochemical staining reveals characteristic neuroendocrine markers such as synaptophysin, chromogranin, CD56, and neuron-specific enolase (NSE), with potential CD99 positivity.
  • Absence of WT1, cytokeratin 7, cytokeratin 20, thyroid transcription factor (TTF1), and LCA helps rule out differential diagnoses.
  • Despite being considered low-grade, renal carcinoid tumors can metastasize, with increased mitotic activity, necrosis, and cytological atypia correlating with poorer prognosis.

Impact:

  • Accurate diagnosis of primary renal neuroendocrine tumors through characteristic histology and immunohistochemistry.
  • Improved differential diagnosis, reducing misclassification as other common kidney cancers.
  • Application of established WHO grading for neuroendocrine tumors to renal carcinoids may improve prognostic assessment and patient management.

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