title: Primary Ureteral Thyroid Transcription Factor 1-Positive Small Cell Neuroendocrine Carcinoma: Case Report and Review of the Literature.
abstract: Upper urinary tract neoplasms comprise only 5% of urinary malignancies overall, the overwhelming majority showing an urothelial histology. Nonurothelial lesions represent 1.9% of upper tract neoplasms, and small cell carcinomas (SmCCs) only account for a minute fraction of them, with few cases reported in the literature to date. In the genitourinary tract, these lesions are most frequently found in the urinary bladder, where they tend to show a mixed histology with a characteristic small cell neuroendocrine component that is positive for chromogranin A, synaptophysin, and CD56 immunostains. Urinary SmCCs are almost invariably aggressive, with a propensity to infiltrate surrounding tissues and vascular structures. Unfortunately, clinical stage at presentation is often advanced, with early dissemination to lymph nodes or distant organs in a significant proportion of patients. For the most part, prognosis is dismal and can be predicted by the pathologic stage at diagnosis. Recently, new chemotherapeutic regimens have shown some potential benefit for the treatment of urinary SmCC, albeit not achieving curative results. Here we present the case of a 71-year-old male patient with a history of treated bladder urothelial carcinoma that presented with subacute flank pain and was diagnosed with a primary pure SmCC of the ureter. Besides the usual neuroendocrine immunomarkers, this tumor showed intense positivity for TTF-1 (thyroid transcription factor-1), a phenomenon that has been described for extrapulmonary SmCC of other locations.
authors.affiliation: University of Illinois at Chicago Hospital and Health Sciences System, Chicago, IL, USA amacosta@uic.edu.
authors.forename: Andrés Martin
authors.initials: AM
authors.lastname: Acosta
authors.affiliation: University of Illinois at Chicago Hospital and Health Sciences System, Chicago, IL, USA.
authors.forename: Farid Saei
authors.initials: FS
authors.lastname: Hamedani
authors.affiliation: Feinberg School of Medicine, Northwestern University, Chicago, IL, USA Jesse Brown VAMC, Chicago, IL, USA.
authors.forename: Joshua James
authors.initials: JJ
authors.lastname: Meeks
authors.affiliation: Jesse Brown VAMC, Chicago, IL, USA.
authors.forename: Shoujin
authors.initials: S
authors.lastname: Wu
mesh_terms: D000368:Aged; D014408:Biomarkers, Tumor / Q000378:metabolism*; D018278:Carcinoma, Neuroendocrine / Q000378:metabolism / Q000473:pathology*; D018288:Carcinoma, Small Cell / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008297:Male; D009687:Nuclear Proteins / Q000378:metabolism*; D000074482:Thyroid Nuclear Factor 1; D014157:Transcription Factors / Q000378:metabolism*; D014516:Ureteral Neoplasms / Q000378:metabolism / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor; C000614412:NKX2-1 protein, human; D009687:Nuclear Proteins; D000074482:Thyroid Nuclear Factor 1; D014157:Transcription Factors
keywords: TTF-1; genitourinary neoplasms; neuroendocrine tumor; small cell carcinoma; upper urinary tract; ureter; ureteral