Management of uterine papillary serous carcinoma

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Management of uterine papillary serous carcinoma
KAVITA SINGH, MD., JÁNOS BÁLEGA, MD.

Pan-Birmingham Gynaecological Cancer Centre, Birmingham

ABSTRACT Uterine papillary serous carcinoma (UPSC) is an aggressive variant of endometrial cancer accounting for 10% of all uterine cancers, but it is responsible for 40% of diseaserelated
deaths. It has got an aggressive biological and clinical behaviour with the propensity to spread even in early stage. Since its relative rarity, there are only limited prospective randomised data available helping clinicians in planning the management of patients with UPSC. The aim of this paper is to summarise the evidence on the management of UPSC.

Key words serous papillary carcinoma, endometrium, prognosis, management



INTRODUCTION
Uterine papillary serous carcinoma (UPSC) is an aggressive variant of endometrial cancer (EC), is nonoestrogen dependent and occurs more commonly in elderly,
non-obese, parous women. It accounts for 10% of all uterine cancers but is responsible for 40% of disease-related deaths (1-2).

It was first identified as a distinct histological entity in 1981 by Lauchlan (3). Hendrickson and colleagues (4) in 1982 correlated this histological subtype with their aggressive biological behaviour and identified their association with increased myometrial infiltration, increased metastatic spread to lymphatics, and higher incidence of upper abdominal relapse compared to cancers with endometrioid histology. Bokhman (5) in 1983 sub-classified endometrial cancer into two types. The most common subtype is endometrioid adenocarcinoma (Type I), which accounts for 80% of uterine cancers and are oestrogen-dependent, and have a relatively indolent course on the background of endometrial hyperplasia. They occur more frequently in obese, nulliparous women with diabetes and polycystic ovaries. They are usually oestrogen (ER) and progesterone receptor (PR) positive unlike the Type II uterine cancer (UPSC) which arises in atrophic endometrium and is ER- and PR- negative but always stain positive for p53 mutation (6). Histologically, UPSC resembles ovarian papillary serous carcinoma (OPSC), and psammoma bodies might be present (7). Its biological behaviour and clinical presentation is similar to ovarian, tubal, and peritoneal serous carcinomas, though the outcome is even poorer as it has a lower response rate to chemotherapy, which is 25-30% compared to 60-70% for ovarian cancer. In African-Americans, there is a higher preponderance of Type II uterine cancers as compared
to Caucasians (8).

UPSC metastasises early by haematogenous, lymphatic, contiguous surface spread or by exfoliation of tumour emboli directly into the peritoneal cavity. Extra-pelvic spread may be present even without any myometrial invasion, and therefore appropriate staging is vital for assessment of prognosis and selection of cases for adjuvant treatment (9).

Till date, the management of UPSC has not been standardised. Most studies have been retrospectively generated from single institutions and represent small case series of cases collected over a long period of time and with no uniform surgical or adjuvant treatment protocols. Majority of the studies reported also consist of heterogeneous group of patients treated with various forms of adjuvant treatment. The aim of this paper is to summarise the available evidence on the management of UPSC.

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