Tubulocystic carcinoma of the kidney: Clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma

Mahul B. Amin, Gregory T. MacLennan, Ruta Gupta, David Grignon, Francois Paraf, Annick Vieillefond, Gladell P. Paner, Mark Stovsky, Andrew N. Young, John R. Srigley, John C. Cheville

Research output: Contribution to journalArticle

137 Scopus citations

Abstract

A distinctive tumor described under the terms Bellini duct carcinoma and low-grade collecting duct carcinoma has been referred to by us and others as tubulocystic carcinoma. This renal cell carcinoma subtype is not recognized in the World Health Organization 2004 classification. Herein, we present a detailed study of 31 cases to further characterize this rare subtype of renal cell carcinoma. The tumor occurred in adults (mean age, 54 years) with a strong male predominance (7:1). Grossly, the tumors ranged from 0.7 to 17 cm, and exhibited a spongy or "bubble wrap" appearance reflecting the microscopic presence of variably sized cystically dilated tubules lined by a single layer of epithelium. The lining varied with a cuboidal, flat, and hobnail cell appearance, and the neoplastic cells had abundant eosinophilic cytoplasm and enlarged nuclei with prominent nucleoli. The cysts were closely spaced with an intervening variably fibrotic stroma. Immunohistochemistry and ultrastructural examination showed features of proximal convoluted tubules (Pax 2 immunoreactivity and short microvilli with brush border organization) and distal nephron (kidney-specific cadherin immunoreactivity and cytoplasmic interdigitation). Gene expression profiling showed that tubulocystic carcinoma displayed a unique molecular signature. Twenty-four tumors were stage pT1, 4 stage pT2, and 3 stage pT3. Disease progression (median follow-up of 56 months) occurred in 3 patients; 1 with local recurrence, and 2 with distant metastasis to bone and liver. In light of the distinctive clinicopathologic features and a low but definite metastatic potential, this unique subtype of renal cell carcinoma deserves formal recognition in the contemporary classification of renal neoplasms.

Original languageEnglish (US)
Pages (from-to)384-392
Number of pages9
JournalAmerican Journal of Surgical Pathology
Volume33
Issue number3
DOIs
StatePublished - Mar 1 2009

Keywords

  • Gene expression profiling
  • Histologic subtypes
  • Immunohistochemistry
  • Metastasis
  • Pathologic stage
  • Pathology
  • Renal cell carcinoma
  • Tubulocystic carcinoma

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Fingerprint Dive into the research topics of 'Tubulocystic carcinoma of the kidney: Clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma'. Together they form a unique fingerprint.

  • Cite this

    Amin, M. B., MacLennan, G. T., Gupta, R., Grignon, D., Paraf, F., Vieillefond, A., Paner, G. P., Stovsky, M., Young, A. N., Srigley, J. R., & Cheville, J. C. (2009). Tubulocystic carcinoma of the kidney: Clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma. American Journal of Surgical Pathology, 33(3), 384-392. https://doi.org/10.1097/PAS.0b013e3181872d3f