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Nat Rev Urol. 2010 Oct;7(10):583-7. doi: 10.1038/nrurol.2010.140. Epub 2010 Sep 14.

Neuroendocrine carcinoma in a patient with Birt-Hogg-Dubé syndrome.

Author information

1
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.

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.

INVESTIGATIONS:

CT and MRI, physical examination, measurement of serum markers, urinalysis, transrectal prostate biopsy, histopathological and genetic examination of tumor specimens, postmortem immunohistochemical analysis.

DIAGNOSIS:

Neuroendocrine carcinoma of prostate or bladder origin.

MANAGEMENT:

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.

PMID:
20842188
DOI:
10.1038/nrurol.2010.140
[Indexed for MEDLINE]

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