title: Neuroendocrine carcinoma in a patient with Birt-Hogg-Dubé syndrome.
abstract: BACKGROUND
  A patient with Birt-Hogg-Dubé syndrome (BHD) presented with gross hematuria of 6 months' duration. Imaging revealed the presence of a mass in the left prostatic lobe, in addition to a previously observed renal mass. Prostate biopsy and imaging findings indicated an inflammatory etiology, and the patient was discharged. 5 months later, the patient presented once again with urinary retention. During transurethral resection of the prostate, a mass adjacent to the bladder was observed. Postoperative imaging revealed a large pelvic mass, a second mass impinging on the rectum, and extensive lymphadenopathy. The patient died 2 weeks later.
  
  
  METHODS
  CT and MRI, physical examination, measurement of serum markers, urinalysis, transrectal prostate biopsy, histopathological and genetic examination of tumor specimens, postmortem immunohistochemical analysis.
  
  
  METHODS
  Neuroendocrine carcinoma of prostate or bladder origin.
  
  
  RESULTS
  The patient died before planned chemotherapy or radiation therapy could be implemented. More-frequent monitoring of the patient might have led to earlier diagnosis and allowed treatment to be started before widespread tumor metastasis and invasion.
authors.affiliation: Department of Dermatology, Research Institute Growth and Development (GROW), School for Oncology and Developmental Biology, University of Maastricht, P. O. Box 616, 6200 MD Maastricht, The Netherlands.
authors.forename: Tijs
authors.initials: T
authors.lastname: Claessens
authors.forename: Sherry A
authors.initials: SA
authors.lastname: Weppler
authors.forename: Michel
authors.initials: M
authors.lastname: van Geel
authors.forename: David
authors.initials: D
authors.lastname: Creytens
authors.forename: Maaike
authors.initials: M
authors.lastname: Vreeburg
authors.forename: Bradley
authors.initials: B
authors.lastname: Wouters
authors.forename: Maurice A M
authors.initials: MA
authors.lastname: van Steensel
mesh_terms: D058249:Birt-Hogg-Dube Syndrome / Q000150:complications*; D018278:Carcinoma, Neuroendocrine / Q000209:etiology*; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008875:Middle Aged; D011471:Prostatic Neoplasms / Q000209:etiology*; D001749:Urinary Bladder Neoplasms / Q000209:etiology*