Multiple primary tumors in patients with carcinosarcoma of bladder: A coincidence or an etiological association? A study of 221 carcinosarcoma of bladder cases

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e16138-e16138
Author(s):  
J. Wang ◽  
F. Wang

e16138 Background: Carcinosarcoma of the bladder are rare; As a result, few studies of carcinosarcoma of the bladder have included sufficient number of patients to define their natural history and to determine the factors that impact survival. The objective of this study was to examine the epidemiology, natural history, and prognostic factors that affect the survival for carcinosarcoma of the bladder by using population-based registries. Methods: The Surveillance, Epidemiology, and End Results (SEER) Program database (1973–2004) was used to identify cases of carcinosarcoma of the bladder by tumor site and histology codes. The association between clinical and demographic characteristics and survival of carcinosarcoma of the bladder was examined. Results: A total of 221 cases of histology confirmed carcinosarcoma of the bladder were identified, this account for approximately 0.11% of primary bladder tumors during same period. By using linked population files, we calculated the incidence of carcinosarcoma of the bladder as a rate 0.22 per 100,000 per year, age-adjusted to year 2000 U.S. standard population. Median age of all patients were 75 years (range 41–96). In about 40% of study population, more than one or multiple primary were also indentified. Majority of (212, 95.9 %) patients received cancer directed surgery. For survival analyses, we excluded the cases that were identified at autopsy or on the basis of death certificates only and the patients have more than one primary. Total 132 patients were included in survival analysis. The median overall survival for all cases was 9 months (range 6–12 months). In multivariate analysis, only tumor stage was found to be significant prognostic factors for disease-specific survival. Conclusions: Carcinosarcoma of the bladder are rare tumors that are challenging, the survival is poor even in patients underwent surgical resection. Better therapy is needed to improve patient's outcome. A significant fraction of carcinosarcoma patients are actually affected by multiple primary tumors, the potential association between carcinosarcoma and other neoplasms remain to be investigated. No significant financial relationships to disclose.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. e15015-e15015
Author(s):  
Jue Wang

e15015 Background: Micropapillary bladder carcinoma (MPBC) is a rare variant of urothelial carcinoma. The objective of this study was to examine the epidemiology, natural history, and prognostic factors of MPBC using population-based registry. Methods: The Surveillance, Epidemiology, and End Results (SEER) Program database was used to identify cases of MPBC by tumor site and histology codes. The clinical, demographic characteristics, treatment and survival of MPBC were examined. Results: A total of 98 cases of histology confirmed MPBC were identified between 2001 and 2007, this accounted for approximately 0.01% of all primary bladder tumors during the study period. Median age of the patients was 72 years (range 26-95). Of all the patients with MPBC, 56.1% had muscle invasive disease; 75.5% of patients had poorly or undifferentiated histology. A total of 30.6% of all patients (2.3% superficial disease; 52.7% of muscle-invasive MPBC) had radical or partial cystectomy. By logistic regression analysis, nonmuscle-invasive MPBC (OR 62.5, 95% CI 7.2-542.3) was associated with lower rate of utilization of cystectomy. The 1-, 3- and 5-year overall survival rate of MPBC were 84.5%, 57.3% and 42.3%. In multivariate analysis, tumor stage (HR 4.7, 95% CI 1.0-21.8) was found to be the only significant predictor for cancer-specific survival. Conclusions: The optimal therapy for MPBC remains undermined. Emphasis on early detection is needed to improve the outcome for patients with this malignancy.


2018 ◽  
Vol 141 (1) ◽  
pp. 213-221 ◽  
Author(s):  
Ha Son Nguyen ◽  
Ninh B. Doan ◽  
Michael Gelsomino ◽  
Saman Shabani ◽  
Ahmed J. Awad ◽  
...  

Sarcoma ◽  
2010 ◽  
Vol 2010 ◽  
pp. 1-7 ◽  
Author(s):  
Jue Wang ◽  
Fen Wei Wang ◽  
Chad A. LaGrange ◽  
George P. Hemstreet III ◽  
Anne Kessinger

Background. Urinary bladder sarcomatoid carcinoma (carcinosarcoma) is rare. The objective of this study was to examine the epidemiology, natural history, and prognostic factors of urinary bladder carcinosarcoma using population-based registry.Methods. The Surveillance, Epidemiology, and End Results (SEER) Program database was used to identify cases by tumor site and histology codes. The association between clinical and demographic characteristics and long-term survival was examined.Results. A total of 221 histology confirmed cases were identified between 1973 and 2004, this accounted for approximately 0.11% of all primary bladder tumors during the study period. Median age of the patients was 75 years (range 41–96). Of the patients with a known tumor stage , 72.5% had a regional or distant stage; 98.4% of patients with known histology grade , had poorly or undifferentiated histology. Multiple primary tumors were indentified in about 40% of study subjects. The majority of patients (95.9%) received cancer directed surgery, 35.8% had radical or partial cystectomy, 15.8% of patients received radiation therapy combination with surgery. The median overall survival was 14 months (95% CI 7–21 months). 1-, 5-, and 10-year cancer specific survival rate were 53.9%, 28.4% and 25.8%. In a multivariate analysis, only tumor stage was found to be a significant prognostic factor for disease-specific survival.Conclusions. Urinary bladder carcinosarcoma commonly presented as high grade, advanced stage and aggressive behavior with a poor prognosis. Emphasis on early detection, including identification of risk factors is needed to improve the outcome for patients with this malignancy.


2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Jue Wang ◽  
Fen Wei Wang ◽  
Anne Kessinger

Background. The objective of this paper was to examine the epidemiology, natural history, and prognostic factors of carcinosarcoma of the kidney and renal pelvis (CSKP) using population-based registry.Patients and Methods. Forty-three patients with CSKP, diagnosed between January 1973 and December 2007, were identified from the national Surveillance, Epidemiology, and End Results (SEER) database and reviewed.Results. 79% of all patients with known SEER stage were classified as having regional or distant stage; almost all the patients with known histology grade had poorly or undifferentiated histology. The median cancer specific survival was 6 months (95% CI 4–9). The 1-year cancer-specific survival rate for entire cohort was 30.2%. There were no differences in terms of age at diagnosis, histological grade, tumor stage on presentation, and frequency of nephrectomy between carcinosarcoma of kidney (CSK) or renal pelvis (CSP). In multivariate analysis, age at diagnosis, tumor stage, and year of diagnosis were found to be significant predictors for cancer-specific survival.Conclusion. CSKP commonly presented as high-grade, advanced stage disease, and was associated with a poor prognosis regardless of location.


2016 ◽  
Vol 31 (4) ◽  
pp. 461-465 ◽  
Author(s):  
Valentina Zampiga ◽  
Rita Danesi ◽  
Gianluca Tedaldi ◽  
Michela Tebaldi ◽  
Ilaria Cangini ◽  
...  

Li-Fraumeni syndrome (LFS) is an autosomal dominant disorder occurring at a young age that predisposes individuals to multiple forms of cancer and to a heterogeneous spectrum of malignancies. We describe the clinical history of a patient who had 5 primary malignant cancers and a familiar history consistent with LFS. We analyzed the genomic DNA of the proband and her relatives by next-generation sequencing (NGS) technology using an enrichment protocol for the simultaneous sequencing of 94 genes involved in hereditary cancers. Genetic analysis of the proband revealed a TP53 germline mutation in exon 5 determining a nucleotide alteration at codon 175 (R175H), a hot spot mutation site related to LFS and a reported pathogenic mutation. The proband daughter's and brother's DNA did not carry the TP53 mutation but they had some rare variants in common with the proband, in addition to other variants with a still unclear role. In conclusion, we identified a TP53 mutation in a patient with multiple primary tumors and a family history characterized by a severe susceptibility to cancer. The genetic analysis by targeted NGS led to the identification of the genetic background and to the exclusion of a cancer risk for the family members. Targeted NGS represents an efficient approach for the identification of mutations in families with a heterogeneous phenotype.


Author(s):  
Jennifer Seyffert ◽  
Alysa Herman ◽  
Brian Katz ◽  
Daniel Rivlin

<p>Merkel cell carcinoma (MCC) is an aggressive and uncommon neuroendocrine tumor which clinically presents as a rapidly growing solitary nodule or plaque located in sun exposed areas on the head, neck, and extremities. Merkel cell carcinomas can be UV-induced or result from viral infection with the Merkel cell polyomavirus (MCV). We present a unique case of bilateral symmetric Merkel cell carcinomas located on the dorsal hands and briefly discuss options for genomic investigation to delineate if the tumors are multiple primary tumors or result of metastatic disease.</p>


2018 ◽  
Vol 9 ◽  
Author(s):  
Mathias Cavaillé ◽  
Flora Ponelle-Chachuat ◽  
Nancy Uhrhammer ◽  
Sandrine Viala ◽  
Mathilde Gay-Bellile ◽  
...  

1994 ◽  
Vol 56 (3) ◽  
pp. 320-323 ◽  
Author(s):  
Andreas Jovanoic ◽  
Ignaz G. H. van der Tol ◽  
Engelbert A. J. M. Schulten ◽  
Pieter J. Kostense ◽  
Nico de Vries ◽  
...  

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