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Urachal cancer-current concepts of a rare cancer.
- Source :
-
Der Pathologe [Pathologe] 2019 Jun; Vol. 40 (Suppl 1), pp. 31-39. - Publication Year :
- 2019
-
Abstract
- Urachal cancer is a rare but aggressive disease. In addition to the non-glandular tumors, non-cystic urachal adenocarcinomas are nowadays distinguished from the primary cystic variant. (Immunohistochemical) markers are only of minor differential diagnostic value and, therefore, the diagnosis is primarily established in a multidisciplinary approach. The non-cystic variant accounts for the majority of cases (83%), is more common in men (63%), shows a median age at diagnosis of 51 years and has a 5-year survival rate of about 50%. In organ-confined disease, usually a partial cystectomy of the tumor in the bladder dome, including the median umbilical ligament and umbilicus, is performed. In advanced stages, systemic therapy is needed while 5‑fuorouracil (5-FU) containing regimes have been shown to be more effective. Due to the rarity of the tumor, targeted therapy approaches based on a biological rationale are becoming increasingly relevant. As molecular data are still sparse, we compiled and analyzed the largest urachal cancer cohort to date. In 31% of the cases, MAPK-/PI3K signaling pathway alterations were detected (especially in K-/NRAS) with implications for anti-EGFR therapy approaches. Further potentially therapeutic alterations were detected in FGFR1, MET, PDGFRA, and erbB2/HER2. Additionally, PD-L1 tumor cell expression (clone: 22C3) was demonstrated in 16% of cases, therefore making anti-PD-1/PD-L1 immuno-oncological approaches worth considering despite the absence of mismatch repair deficiency (MMR-d) and/or high microsatellite instability (MSI-h). Finally, urachal adenocarcinomas seem to be a distinct entity on the molecular level with closer resemblance to colorectal adenocarcinomas than to urothelial carcinomas.
- Subjects :
- Cystectomy
Humans
Phosphatidylinositol 3-Kinases metabolism
Urachus metabolism
Rare Diseases epidemiology
Rare Diseases metabolism
Rare Diseases pathology
Rare Diseases therapy
Urachus pathology
Urinary Bladder Neoplasms epidemiology
Urinary Bladder Neoplasms metabolism
Urinary Bladder Neoplasms pathology
Urinary Bladder Neoplasms therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1432-1963
- Volume :
- 40
- Issue :
- Suppl 1
- Database :
- MEDLINE
- Journal :
- Der Pathologe
- Publication Type :
- Academic Journal
- Accession number :
- 30895340
- Full Text :
- https://doi.org/10.1007/s00292-018-0516-9