Dg. Huntsman et al., SMALL-CELL CARCINOMA OF THE ENDOMETRIUM - A CLINICOPATHOLOGICAL STUDYOF 16 CASES, The American journal of surgical pathology, 18(4), 1994, pp. 364-375
Sixteen cases of small-cell carcinoma of the endometrium were encounte
red in patients who ranged in age from 30 to 78 (mean. 57.4) years. Of
the 12 patients whose presenting features are known, eight had abnorm
al vaginal bleeding, three had pain related to metastatic tumor, and o
ne patient had both symptoms. On pelvic examination, adnexal masses we
re palpable in three patients, and vaginal involvement was evident in
two; one patient had a large palpable periumbilical mass. Thirteen pat
ients underwent total abdominal hysterectomy and bilateral salpingo-oo
phorectomy. Extrauterine spread was documented intraoperatively in eig
ht cases, including widespread intraabdominal and ovarian metastases i
n four cases, vaginal involvement in the two cases noted previously, p
araaortic lymph node involvement in one case, and tubal involvement in
one case. Three tumors were International Federation of Gynecology an
d Obstetrics (FIGO) stage I, four were stage II, two were stage III, a
nd six were stage IV; in one case, there was insufficient information
to allow staging. On gross examination, the tumors were usually descri
bed as bulky, ill-defined, and invasive of the myometrium; four were p
olypoid. Microscopic examination revealed sheets, cords, and nests of
small or intermediate-sized cells with scanty cytoplasm, hyperchromati
c nuclei, and a high mitotic rate. Single-cell and zonal necrosis and
vascular invasion were typically present. Synchronous grade 1 or grade
2 endometrial endometrioid adenocarcinoma was present in eight cases,
and complex atypical endometrial hyperplasia, in two others. In three
cases, the adenocarcinoma merged almost imperceptibly with the small-
cell component. None of the tumors contained argyrophil or argentaffin
cells, although nine of 11 tumors were immunoreactive for neuron-spec
ific enolase (one of these was also Leu-7 positive), and another was c
hromogranin positive. Of the 11 cases with follow-up information, seve
n patients died of disease (at least four with distant metastases) wit
h a median survival of 12 months, and another patient was alive with d
istant metastases at 18 months. The remaining patients were clinically
free of disease at postoperative intervals of less-than-or-equal-to 1
year (two cases) and 4.5 years (one case). This study confirms that s
mall-cell carcinomas of the endometrium are a histologically distincti
ve subtype of endometrial carcinoma, which, like their counterparts in
the uterine cervix, are aggressive tumors with a propensity for syste
mic s read and a poor prognosis.