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abstract: A Clinicopathologic Study of 25 Cases
Neuroendocrine
carcinoma (NECa) of the endometrium is an uncommon tumor. In this
study, we present the clinicopathologic features of 25 such cases. The
patients ranged in age from 37 to 87 years (median, 57 y) and most
commonly presented with vaginal bleeding. The tumors were either pure
NECa (10) or mixed with other histotypes (15), most commonly
endometrioid carcinoma. The NECas were large cell type (15), small cell
type (4), or a mixture of both (6). NECa was underrecognized in 89% of
referral/consultation cases. All tumors were positive for ≥1
neuroendocrine marker (chromogranin, synaptophysin, CD56). Additional
immunohistochemical (IHC) studies were obtained in 18 cases, with
positive results as follows: keratin cocktail (17), diffuse p16 (6),
PAX-8 (6), CD117 (6), and TTF-1 (1). Mismatch-repair protein expression
by IHC was abnormal in 8 of 18 cases (6 MLH1/PMS2 loss; 1 MSH2/MSH6
loss; 1 MSH6 loss). According to FIGO staging, cases were distributed
as follows: I (6), II (2), III (10), and IV (7). All patients underwent
surgical treatment, and 20 patients received adjuvant therapy. Twelve
patients died of disease (mean survival 12.3 mo). Eleven patients were
alive 5 to 134 months after diagnosis, including 7 who achieved a 5-year
survival (3 stage I; 4 stage III). In summary, most of our endometrial
NECas were large cell type, mixed with other histotypes, and
underrecognized. These tumors tend to be PAX-8 negative and may be
associated with microsatellite instability. The recognition of NECa may
have an impact on the treatment of the patients affected by this
disease. Although NECa usually has an aggressive behavior, 28% of our
patients survived at least 5 years.
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