Ten cases of endometrial small cell neuroendocrine carcinoma are descr
ibed. The ages of the patients ranged from 50 to 75 years (mean, 64 ye
ars). Most of the tumors were bulky, intraluminal masses that invaded
at least half of the myometrial wall. Small cells were the only malign
ant element in two tumors. In the other eight, there were admixed elem
ents of adenocarcinoma (five), adenosquamous carcinoma (two), or heter
ologous mesodermal mixed tumor (one). Histologic examination of metast
atic deposits in six cases revealed solely small cells in all but one.
Immunohistochemical evidence of neuroendocrine differentiation was de
monstrated in all tumors using the markers chromogranin, synaptophysin
, leu-7, or neuron-specific enolase. Six of these tumors were original
ly interpreted as mesodermal mixed tumors with a homologous, stromal-t
ype sarcomatous component at initial pathologic examination, but were
reclassified as carcinoma. Clinical follow-up of these 10 patients and
an additional seven well-documented patients reported in the literatu
re provided strong evidence for the aggressive nature of this neoplasm
. Endometrial small cell neuroendocrine carcinoma is a rare, but aggre
ssive neoplasm that can commonly be mistaken for a homologous-type mes
odermal mixed tumor.