Serous Carcinoma of the Uterus

Background

Uterine serous carcinomas (USC) are characterized by aggressive and bulky disease. Unlike most other uterine carcinoma cases, these tumors usually arise from endometrium which is atrophic rather than hyperplastic.

EPIDEMIOLOGY CHARACTERIZATION

SYNONYMS Serous papillary adenocarcinoma of the endometrium, uterine serous carcinoma

INCIDENCE 10% of endometrial carcinomas

AGE-RANGE AND MEDIAN 39-93 years Median 68 years

GEOGRAPHIC DISTRIBUTION Worldwide though may be lesser incidence in Norway and Australia

GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION

Classic
Most tumors are small and atrophic but have a papillary exophytic appearance

Occasionally, the tumor may be confined to the polyp

HISTOLOGICAL TYPES CHARACTERIZATION

Precursor lesions Precursor lesions have been sought and have been classified as minimal uterine serous carcinoma (MUSC). This term has been applied to cases of endometrial intraepithelial carcinoma (EIC) and superficial serous carcinoma (SSC). EIC is defined as replacement of the uterine surface epithelium and/or underlying glands by high grade malignant cells which represent the in situ phase of USC. SSC is defined as USC without myometrial or lymphovascular invasion. In a study of 21 cases of MUSC, 13 of 14 cases (93%) of MUSC confined to the uterus were disease free and one died from unrelated causes. The other 7 women all presented with extrauterine disease, including microscopic deposits, and were dead or alive with recurrences.

Classic Complex papillary architecture with cuboidal to hobnailed shaped cells having marked cytologic atypia
High mitotic activity
Psammoma bodies in 1/3 of cases
In almost all cases, the adjacent endometrium is atrophic and may show the precursor lesions
Tend to invade deeply into myometrium with extensive lymphatic invasion

SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER CHARACTERIZATION

p53 Overexpression in 71% of cases

Ki-67 High proliferation rate

WT1

WT1 immunoreactivity in uterine papillary serous carcinomas is different from ovarian serous carcinomas.

Goldstein NS, Uzieblo A.

Department of Anatomic Pathology, William Beaumont Hospital, Royal Oak, MI 48073, USA.
Am J Clin Pathol 2002 Apr;117(4):541-5 Abstract quote
WT1 diffusely stains most ovarian serous carcinomas; reactivity of uterine papillary serous carcinomas has not been evaluated.

We studied WT1 expression in 13 International Federation of Gynecology and Obstetrics stage 1 and 5 stage 3 or 4 uterine papillary serous carcinomas without ovarian metastases and compared their reactivity with the WT1 staining of 30 ovarian serous carcinomas. WT1 reactivity was evaluated with the C19 and 6F-H2 antibody clones. All 18 uterine papillary serous carcinomas were nonreactive for WT1. The nonovarian metastases of the 5 high-stage uterine papillary serous carcinomas also were nonreactive for WT1. In contrast, 29 (97%) of 30 ovarian serous carcinomas were reactive for WT1. WT1 reactivity in an unknown primary serous carcinoma would suggest it is from a nonuterine site.

The mechanisms underlying these findings are unknown. They raise the possibility of genetic differences between the 2 morphologically similar neoplasms.

PROGNOSIS AND TREATMENT CHARACTERIZATION

Prognostic Factors Most important feature which determines prognosis is the presence or absence of extrauterine disease at initial presentation
The surgeon must do a careful staging and the pathologist must do a diligent search for microscopic deposits of disease.

5 Year Survival 36% for all stages

Metastasis May extensively involve the lymphatics of the myometrium, cervix, broad ligament, fallopian tube, and ovaries
Extends over the peritoneal surfaces of the pelvis and abdomen

Treatment Surgery with chemotherapy

Uterine papillary serous carcinoma: a study on 108 cases with emphasis on the prognostic significance of associated endometrioid carcinoma, absence of invasion, and concomitant ovarian carcinoma.

Carcangiu ML, Chambers JT.

Department of Pathology, Yale University School of Medicine, New Haven, Connecticut 06510.
Gynecol Oncol 1992 Dec;47(3):298-305 Abstract quote
One hundred eight cases of uterine papillary serous carcinoma (UPSC) were analyzed to characterize its histologic features and biologic behavior. Special situations that could conceivably modify the behavior and therapeutic approaches were considered: (1) the occurrence of areas of endometrioid carcinoma in otherwise typical UPSC; (2) the confinement of UPSC to an otherwise benign endometrial polyp or the endometrial mucosa or absence of residual tumor at the time of hysterectomy; and (3) the coexistence of a superficial UPSC and a serous ovarian carcinoma.

There was coexistence of endometrioid and UPSC in 22 cases, and tumor was confined to an endometrial polyp or endometrium in 19 cases. There was simultaneous pathologic stage I UPSC and papillary serous ovarian carcinoma in 10 cases. In patients with pathologic stages I and II UPSC the presence of areas of endometrioid carcinoma intermixed with the UPSC did not improve survival. Patients with stage I disease and no residual tumor or tumor confined to an endometrial polyp/endometrial mucosa and without vascular invasion had a survival not statistically different from those with stage I disease but with myometrial and/or vascular invasion.

Patients with stage I UPSC with concomitant ovarian serous surface papillary carcinoma had survival not statistically different from patients with stage IV UPSC.

Am J Surg Pathol 2000;24:797-806

Commonly Used Terms

Endometrial carcinoma
Uterus and cervix

Last Updated 4/10/2002

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EPIDEMIOLOGY	CHARACTERIZATION
SYNONYMS	Serous papillary adenocarcinoma of the endometrium, uterine serous carcinoma
INCIDENCE	10% of endometrial carcinomas
AGE-RANGE AND MEDIAN	39-93 years Median 68 years
GEOGRAPHIC DISTRIBUTION	Worldwide though may be lesser incidence in Norway and Australia

HISTOLOGICAL TYPES	CHARACTERIZATION
Precursor lesions	Precursor lesions have been sought and have been classified as minimal uterine serous carcinoma (MUSC). This term has been applied to cases of endometrial intraepithelial carcinoma (EIC) and superficial serous carcinoma (SSC). EIC is defined as replacement of the uterine surface epithelium and/or underlying glands by high grade malignant cells which represent the in situ phase of USC. SSC is defined as USC without myometrial or lymphovascular invasion. In a study of 21 cases of MUSC, 13 of 14 cases (93%) of MUSC confined to the uterus were disease free and one died from unrelated causes. The other 7 women all presented with extrauterine disease, including microscopic deposits, and were dead or alive with recurrences.
Classic	Complex papillary architecture with cuboidal to hobnailed shaped cells having marked cytologic atypia High mitotic activity Psammoma bodies in 1/3 of cases In almost all cases, the adjacent endometrium is atrophic and may show the precursor lesions Tend to invade deeply into myometrium with extensive lymphatic invasion

SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER	CHARACTERIZATION
p53	Overexpression in 71% of cases
Ki-67	High proliferation rate
WT1
WT1 immunoreactivity in uterine papillary serous carcinomas is different from ovarian serous carcinomas. Goldstein NS, Uzieblo A. Department of Anatomic Pathology, William Beaumont Hospital, Royal Oak, MI 48073, USA.	Am J Clin Pathol 2002 Apr;117(4):541-5 Abstract quote WT1 diffusely stains most ovarian serous carcinomas; reactivity of uterine papillary serous carcinomas has not been evaluated. We studied WT1 expression in 13 International Federation of Gynecology and Obstetrics stage 1 and 5 stage 3 or 4 uterine papillary serous carcinomas without ovarian metastases and compared their reactivity with the WT1 staining of 30 ovarian serous carcinomas. WT1 reactivity was evaluated with the C19 and 6F-H2 antibody clones. All 18 uterine papillary serous carcinomas were nonreactive for WT1. The nonovarian metastases of the 5 high-stage uterine papillary serous carcinomas also were nonreactive for WT1. In contrast, 29 (97%) of 30 ovarian serous carcinomas were reactive for WT1. WT1 reactivity in an unknown primary serous carcinoma would suggest it is from a nonuterine site. The mechanisms underlying these findings are unknown. They raise the possibility of genetic differences between the 2 morphologically similar neoplasms.

PROGNOSIS AND TREATMENT	CHARACTERIZATION
Prognostic Factors	Most important feature which determines prognosis is the presence or absence of extrauterine disease at initial presentation The surgeon must do a careful staging and the pathologist must do a diligent search for microscopic deposits of disease.
5 Year Survival	36% for all stages
Metastasis	May extensively involve the lymphatics of the myometrium, cervix, broad ligament, fallopian tube, and ovaries Extends over the peritoneal surfaces of the pelvis and abdomen
Treatment	Surgery with chemotherapy
Uterine papillary serous carcinoma: a study on 108 cases with emphasis on the prognostic significance of associated endometrioid carcinoma, absence of invasion, and concomitant ovarian carcinoma. Carcangiu ML, Chambers JT. Department of Pathology, Yale University School of Medicine, New Haven, Connecticut 06510.	Gynecol Oncol 1992 Dec;47(3):298-305 Abstract quote One hundred eight cases of uterine papillary serous carcinoma (UPSC) were analyzed to characterize its histologic features and biologic behavior. Special situations that could conceivably modify the behavior and therapeutic approaches were considered: (1) the occurrence of areas of endometrioid carcinoma in otherwise typical UPSC; (2) the confinement of UPSC to an otherwise benign endometrial polyp or the endometrial mucosa or absence of residual tumor at the time of hysterectomy; and (3) the coexistence of a superficial UPSC and a serous ovarian carcinoma. There was coexistence of endometrioid and UPSC in 22 cases, and tumor was confined to an endometrial polyp or endometrium in 19 cases. There was simultaneous pathologic stage I UPSC and papillary serous ovarian carcinoma in 10 cases. In patients with pathologic stages I and II UPSC the presence of areas of endometrioid carcinoma intermixed with the UPSC did not improve survival. Patients with stage I disease and no residual tumor or tumor confined to an endometrial polyp/endometrial mucosa and without vascular invasion had a survival not statistically different from those with stage I disease but with myometrial and/or vascular invasion. Patients with stage I UPSC with concomitant ovarian serous surface papillary carcinoma had survival not statistically different from patients with stage IV UPSC.