- Clear-cell RCC
- Papillary RCC
- Chromophobe RCC
- Collecting duct carcinoma
- Renal medullary carcinoma
- Translocation tumors
- Tubulocystic RCC
- Clear Cell (Tubulo) Papillary RCC
- Acquired Cystic Disease–associated RCC
- Multilocular Cystic Renal Cell Neoplasm of Low Malignant Potential (Multilocular Cystic RCC)
- Hybrid Oncocytic/Chromophobe Tumors
Clear-Cell Renal Cell Carcinoma
|Clear-cell RCC under the microscope.|
Papillary Renal Cell CarcinomaPapillary RCC accounts for 10-15% of RCC tumors, making it the second most common tumor subtype. Papillary RCC was previously known as "chromophilic" RCC. Papillary RCC have a few important clinical correlations:
- commonly found in patients with end-stage renal disease
- commonly found in patients with acquired cystic disease
- often multifocal, upwards of 40% of papillary RCC are found in more than one site in the kidney
|Papillary RCC (webpathology.com)|
- more common form
- dark cells with scant cytoplasm
- associated with Hereditary Papillary RCC Syndrome
- less common
- eosinophilic (red) cells with abundant cytoplasm
- sporadic forms of Papillary Type 2 are not necessarily dangerous
- these tumors are potentially aggressive when associated with the hereditary Leiomyomatosis and RCC Syndrome -- these tumors are now given their own distinction and are no longer lumped with Type 2 tumors.
Chromophobe Renal Cell Carcinoma
Collecting Duct CarcinomaAs the name suggests, collecting duct carcinoma derives from the collecting duct (or Bellini's duct) of the nephron and are also known as Bellini tumors. They account for less than 1% of RCC. Collecting duct carcinoma often presents in younger patients with advanced disease and is unresponsive to most therapies, leading to a poor prognosis.
Renal Medullary CarcinomaAlso a rare and aggressive form of cancer, renal medullary carcinoma often presents in young, African-Americans with sickle-cell trait with a locally advanced tumor and metastatic disease. The prognosis is poor.
Translocation TumorsTranslocation tumors are a relatively new diagnostic entity and describes a relatively common form of RCC in children. While RCC is less than 5% of renal tumors in children (Wilms and neuroblastoma are much more common), >50% of the RCC are translocation tumors. These tumors are rare in adults, but may be more common in patients exposed to chemotherapy for a prior malignancy. Under the microscope, these tumors represent a combination of both clear-cell and papillary RCC. The term "translocation" defines these tumors as they uniformly demonstrate chromosomal translocations involving the TFE3 transcription factor gene (maps to Xp11.2 locus).
PrognosisAs these tumors are a relatively new entity, data regarding outcomes is still premature. Children have a relatively good prognosis, even with nodal (but not distant) metastases with >90% alive at about 5 years. Data suggests that adults have a worse prognosis, more often presenting with advanced disease and with an average survival of 1-2 years. Interestingly, these tumors can metastasize 20 to 30 years after an initial diagnosis - so long-term follow-up is required.
 American Cancer Society. Cancer Facts & Figures 2014. Atlanta: American Cancer Society; 2014.
Campbell SC, Lane BR. "Malignant Renal Tumors" in Campbell-Walsh Urology, 10th Edition. Wein, Kavoussi, Novick, Partin and Peters (Eds.). Philadelphia: Elsevier, 2012. chapter 49, page 1413-1474.
Srigley JR, Delahunt B, Eble JN, Egevad L, Epstein JI, Grignon D, Hes O, Moch H, Montironi R, Tickoo SK, Zhou M, Argani P; ISUP Renal Tumor Panel.The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia. Am J Surg Pathol. 2013 Oct;37(10):1469-89. doi: 10.1097/PAS.0b013e318299f2d1.