Synchronous bilateral endometrioid ovarian cancer and uterine adenocarcenoma in a young woman 51 T he pr esence of simulta neous c a rcinom as involving both the ovary and uterine corpus is rel- atively uncommon, and only 0.7–10% of patients with epithelial ovarian or uterine cancers have been found to have simultaneous tumours in large series.1 However, these synchronous tumours represent a diagnostic and ther- apeutic challenge, particularly if they have a similar histol- ogy. Here we present the case of a 34-year-old woman with endometrioid cancers of both ovaries and adenocarcinoma of the uterus. C A S E R E P O R T The patient was referred to our hospital because of metror- rhagia and lower abdominal pain of six months duration. She had started to menstruate at the age of 14 and her periods were irregular, occurring every 2–3 months, but with no associated abdominal pain. Five years earlier she had a spontaneous abortion during the12th week of gesta- tion and evacuation had been done. Six months earlier, she had begun to experience excessive vaginal bleeding with severe abdominal pain. Findings of clinical exami- nation were normal apart from mobile uterus just above the symphysis pubis. The vaginal examination revealed an ir regularly enlarged uterus of 10 weeks gestation size, with mobile fornices. Ultrasound scan of abdomen revealed a bulky, multifi broid uterus, with bilateral ovarian masses. Her laboratory investigations were all within the normal squ journal for scientific research: medical sciences 2001, 1, 51–53 ©2001 sultan qaboos university Synchronous bilateral endometrioid ovarian cancer and uterine adeno carcinoma in a young woman *Mansour Al-Moundhri1, Mariam Mathew2, Andrzej Krolikowski2 ABSTRACT. Synchronous carcinomas involving both the ovary and uterine corpus are relatively uncommon. These tumours repre- sent a diagnostic and therapeutic challenge, particularly if they have similar histology. Here we present the case of a 34-year-old woman with bilateral endometrioid cancers of both ovaries and adenocarcinoma of the uterus. 1Departments of Medicine and 2Obstetrics and Gynae cology, Sultan Qaboos University Hospital, P.O. Box: 38, Al-Khod 123, Muscat, Sultanate of Oman *To whom correspondence should be addressed Figure 1. The ruptured right ovarian tumour and the enlarged uterus a l - m o u n d h r i e t a l52 limits apart from high levels of cancer cell surface antigen 125 (CA125 ) of 322 I U /l. An explorative laparotomy showed a large, ruptured right ovarian tumour measuring 12 × 10 × 8 cm, a smaller but similar left ovarian tumour measuring 7 × 6 × 5 cm and a bulky uterus [Figure1]. There was a single deposit on the anterior wall of the uterus and multiple deposits on the omentum. The largest of these deposits was <2 cm in diameter. However, the bowels, paracolic gutter, liver, and diaphragm were clear. Frozen sections of both the ovaries revealed endometrioid carcinomas. Therefore, total abdo minal hysterectomy, bilateral salpingo-oophorectomy, appendectomy and total omentectomy were performed. Histopathological examination showed well-differ- entiated bilateral endometrioid ovarian carcinomas with focal squamoid differentiation in the right ovarian tumour [Figures 2 and 3] and moderately differentiated adeno- carcinoma of uterus infi ltrating up to two-thirds of the depth of uterine wall [Figure 4]. There were multiple neo- plastic omental lesions. Fallopian tubes and appendix were normal. The clinical and histological picture was suggestive of at least two synchronous primaries: (1) Fédération Internationale de Gynécologie et d’Obsté- trique (FIGO ) stage IIIb ovarian cancer with bilateral, large, diffuse, well differentiated endometrioid ovarian can- cers with focal squamoid differentiation in right ovary; (2) FIGO Stage Ic uterine adenocarcinoma extending up to two-thirds of myometrium, with no associated endometrial hyperplasia. The patient made an uncomplicated recovery follow- ing her surgery. Six weeks after surgery, her CA125 level decreased to 40 I U /l. She received six cycles of carbo- platinum and paclitaxel chemotherapy, which resulted in further decline in her CA125 levels [Figure 5]. Subse- quently she received 5000 cGy whole pelvis external radio- therapy, in 25 fractions through 15mV photons over fi ve weeks, followed by whole vaginal intracavitary radio therapy. Ten months after the diagnosis, she remains in clinical remission with CA125 of 1.7 I U /l. Figure 2. Photomicrograph of endometrioid carcinoma of the right ovary with squamoid differentiation Figure 3. Photomicrograph of endometrioid carcinoma of left ovary Figure 4. Photomicrogaph of moderately differentiated adenocarcinoma of uterus s y n c h r o n o u s o va r i a n a n d u t e r i n e c a n c e r s 53 D I S C U S S I O N The si multaneous presence of carcinoma in the endometrium and in the ovary may indicate either metastatic disease or independently developing neoplasms. The classifi cation of these lesions either as two separate primary tumours, or as a single primary tumour with a metastasis has implications for patient prognosis and recommendations for therapy. Several large retrospective studies of ovarian endometrioid cancers have demonstrated that the presence of co-existing endometrial adenocarcinoma was not detrimental to the prognosis of patients: in fact it has been suggested that they may indicate better prognosis.2,3,4 Although several morphological criteria have been proposed as guidelines for classifi cation of these lesions, certain cases remain diffi cult to classify. Eifel et al sug- gested that if both the tumours were of endometrioid type, the neoplasms represented two separate primaries, and the patient had good prognosis. In contrast, histology of papil- lary, clear-cell or mucinous type suggested two separate primaries of different morphology, with poorer prognosis. Ulbright showed that concomitant endometrioid carcinoma of the ovary and adenocarcinoma of the endometrium, if moderately or well differentiated, was possibly independ- ent in origin, whereas the poorly differentiated ones were possibly metastatic.6 More recently it was suggested that molecular analysis might be useful in determining the rela- tionship of synchronous uterine and ovarian endometrioid neoplasms. Emmert-Buck et al reported loss of heterozygos- ity in chromosomes 17q21.3-22 or 11q13 in 10 out of 13 patients who presented with endometrioid tumours in both uterus and ovary. However, eight cases had selective local osteolytic hypercalcemia (LOH ) for one tumour site only, suggesting two separate primary tumours.7 Similarly, Lin et al reported high incidence mutations of the human puta- tive protein tyrosine phosphatase (PTEN / M M AC1) gene at chromosome in synchronous endometrial and ovarian carcinomas.8 The mainstay of the treatment is aggressive surgical cytoreduction with total abdominal hysterectomy, bilateral salpingo-oophorectomy, appendectomy and total oment- ectomy, followed by adjuvant treatment. As with other types of ovarian tumours, the treatment with platinum based chemotherapy has been shown to improve survival in simul- taneous tumours. On the other hand, the admini stration of adjuvant radiotherapy to isolated FIGO Stage Ic uterine adenocarcinoma has been shown to improve outcome. However, it is not clear from the literature whether admin- istration of radiotherapy infl uences prognosis where con- comitant lesions exist C O N C L U S I O N This case illustrates the issues raised by presentation of concomitant ovarian and uterine endometrioid cancers and the fact that it may affect relatively younger women, often requiring extensive surgery and adjuvant chemotherapy. R E F E R E N C E S 1. Zaino RJ, Ungar ER, Whitney C. Synchronous carcino- mas of the uterine corpus and ovary. Gynecol Oncol 1984, 19, 329–35. 2. Tidy J, Mason WP. Endometrioid carcinoma of ovary: a retrospective study. Br J Obstet Gynaecol 1988, 95, 1165–9. 3. Czernobilsky B, Silverman BB, Mikuta JJ. Endometrioid carcinoma of the ovary. A clinicopathologic study of 75 cases. Cancer 1970, 26, 1141–52. 4. Pearl ML, Johnson CM, Frank TS, Roberts JA. Synchro- nous dual primary ovarian and endometrial carcinomas. Int J Gynaecol Obstet 1993, 43, 305–12. 5. Eifel P, Hendrickson M, Ross J, Ballon S, Martinez A, Kempson R. Simultaneous presentation of carcinoma involving the ovary and the uterine corpus. Cancer 1982, 50, 163–70. 6. Ulbright TM, Roth LM. Metastatic and independent can- cers of the endometrium and ovary: a clinicopathologic study of 34 cases. Hum Pathol 1985, 16, 28–34. 7. Emmert-Buck MR, Chuaqui R, Zhuang Z, Nogales F, Liotta LA, Merino MJ. Molecular analysis of synchronous uterine and ovarian endometrioid tumors. Int J Gynecol Pathol 1997, 16, 143–8. 8. Lin WM, Forgacs E, Warshal DP, Martin JS, Ashfaq R et al. Loss of heterozygosity and mutational analysis of the PTEN/MMAC1 gene in synchronous endometrial and ovarian carcinomas. Clin Cancer Res 1998, 4, 2577–83. 0 1 0 2 0 3 0 0 1 0 2 0 3 0 4 0 5 0 CA 1 25 U /m l T i m e ( w e e k s ) Figure 5. Decline in CA125 with chemotherapy