SIU 2017: SIU-ICUD Joint Consultation on Bladder Cancer - Non-urothelial Cancer of the Urinary Bladder

He reviewed each variant by histology and the major recommendations. Of note, more recent series have suggested that variants tend be diagnosed at later stages; but stage-matched, there is no evidence they do any worse than their UC counterparts.

• Bilharzial (predominantly in the Middle east, related to schistosomiasis infection)
• Non-bilharzial – risk factors include chronic catheter, infections, spinal cord injury, smoking
• Surgery remains the mainstay, even for NMIBC
• XRT not effective – though this generated controversy. Chemo-XRT is so effect for other SCC in the body, why not for bladder. The discussion pointed out that these patients may not be candidates for chemo, hence the inability to treat as other sites.
• Locoregional failure remains high despite treatment

• RC with wide local excision for patients with localized SCC of the bladder (LOE 2, Grade B)
  o Radiation reserved for palliation (LOE 3, Grade C)
  o Chemotherapy can be offered for metastatic disease (LOE 4, Grade C)

• RC should be offered as primary therapy for localized disease (LOE 2, Grade B)
• Neoadjuvant XRT +/- chemo may provide survival benefit following RC (LOE 3, Grade C)

• Urachal and non-urachal – managed slightly differently
• Will be negative for PSA, PAP, p63; positive for CK7, CK20, CDX2
• RC is the mainstay of therapy (LOE 3, Grade B); partial cystectomy with umbilectomy and LND is only an option for urachal adeno (LOE 2/3, Grade B)
• Adjuvant XRT and 5FU may help – no strong evidence (Level 4, Grade C)

• Similar to lung small cell cancer genotypically
• Primary therapy is cisplatin/etoposide followed by RC or XRT (LOE 2/3, Grade B)
• Carcinoid variant responds better to surgery first (more indolent)

• Even NMIBC micropapillary is aggressive
• % micropapillary in the specimen is associated with outcomes
• Consider early RC for patients with micropapillary variant
• Early data from MD Anderson suggests ddMVAC be efficacious in this patient cohort – not yet incorporated into the data analysis
• There are more rare variants that warranted their own guideline statements, but due to rarity not much time was spent on their discussion. Full guidelines will detail out all the variants and their management.