We report what to our knowledge is the first case in the English-langu
age literature of a primary, pure, undifferentiated large-cell neuroen
docrine carcinoma of the urinary bladder. To date, only one case of a
large-cell neuroendocrine carcinoma was reported, and it was associate
d with an adenocarcinoma most likely of urachal origin. On the other h
and, slightly more than 100 cases of undifferentiated small-cell carci
noma of the urinary bladder were reported, approximately one-half of w
hich were associated with poorly differentiated transitional-cell carc
inoma of the conventional type. The patient in our case was a 73-year-
old man with a history of prostatic cancer treated with radiation ther
apy. He presented with hematuria, leading to the discovery of a solita
ry tumor on the dorsal wall of the urinary bladder. A diagnosis of lar
ge-cell neuroendocrine carcinoma was made, supported by immunohistoche
mical reactivity for chromogranin, neuron-specific enolase, and synapt
ophysin; a variety of other hormonal markers of neuroendocrine tumors
were negative. The radical cystoprostatectomy and bilateral pelvic lym
phadenectomy specimen showed a transmurally invasive tumor, without re
gional lymph node metastases, The patient died 2 months after surgery,
and the autopsy revealed disseminated metastases histologically ident
ical to the urinary bladder neoplasm, Awareness of the occurrence of l
arge-cell neuroendocrine carcinoma of the urinary bladder seems to be
important because of the possible aggressive outcome associated with t
his tumor and because of differential diagnostic considerations, which
include malignant lymphoma and metastasis from another primary, espec
ially in tumors occurring in a pure form.