Synchronous primary carcinomas of the endometrium and ovary

Clinical History

A 73-year-old woman was referred for vaginal bleeding and anaemia (Hct: 32.5). Transvaginal sonography revealed a large partly cystic-solid left adnexal mass and a moderate amount of ascites.

Imaging Findings

MDCT revealed a large multicystic pelvic mass, probably originating from the left adnexa, with contrast-enhancing solid components and thick, irregular walls or septa, of more than 3 mm thickness, demonstrating enhancement (Fig. 1), strongly suggestive of malignancy. A widened endometrial cavity was also detected (Fig. 1e). Pelvic MRI demonstrated the pelvic mass with solid parts, as areas of restricted diffusion, enhancing after gadolinium administration (Fig. 2). Endometrial carcinoma was detected on MRI, as a heterogeneous lesion, with restricted diffusion (Fig. 2). The tumour interrupted the junctional zone and invaded the myometrium in less than 50% (Fig. 2c). Both CT and MRI revealed the presence of ascites, thickening and enhancement of the peritoneum (Fig. 1d, 2e), a feature suggestive of peritoneal carcinomatosis. The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy and epiploectomy. Histology reported synchronous endometrioid adenocarcinomas of endometrium (FIGO IB) and both ovaries (FIGO IB), of moderate differentiation (Fig. 3).

Discussion

Background
Although synchronous multiple primary tumours are extremely rare, they usually involve the genitourinary and gastrointestinal tract, followed by both breast and genitourinary tract and breast and gastrointestinal tract [1]. Among gynaecologic malignancies, synchronous primary tumours of the endometrium and ovary are the most common. The incidence is reported as about 5% of patients with endometrial cancer and 10% of patients with ovarian cancer [2-4]. The pathogenesis of synchronous endometrial and ovarian carcinomas is unclear. The theory of “secondary Mullerian system” proposed that the epithelia of cervix, uterus, fallopian tubes, ovaries, and peritoneal surface share molecular receptors responding to carcinogenic stimulus, leading to the development of multiple primary malignancies synchronously. This hypothesis could provide explanation for synchronous malignancies of similar histology, but not in cases of dissimilar histology [2]. Various studies have reported that women with synchronous primary cancers of the endometrium and ovary have a better overall prognosis compared to those with a single-organ primary cancer and metastasis.
Simultaneous detection of malignancies of endometrium and ovary often challenges the clinicians and pathologists to make correct diagnosis and arrange appropriate management. These patients can be classified into three groups: primary endometrial cancer with ovarian metastasis, primary ovarian cancer with endometrial metastasis and synchronous primary endometrial and ovarian carcinoma. Several pathologic criteria have been proposed for distinguishing metastatic tumour from synchronous primary cancer, although this may remain challenging [1-5]. Among them, histologic dissimilarity of carcinomas, absence of vascular space invasion, ovarian tumour located in the parenchyma, without surface implants or other evidence of spread, endometrial cancer invading less than 50% of the myometrium, without evidence of spread, coexistence of atypical endometrial hyperplasia or ovarian endometriomas suggest the presence of synchronous primary carcinomas. In this patient, histologic diagnosis was based on absence of vascular space invasion, absence of any spread of ovarian malignancies and presence of endometriotic cysts.
Imaging perspective
Imaging findings that are strongly suggestive of ovarian malignancy include a mass partly cystic-solid, with solid parts as areas of restricted diffusion, enhancing after contrast administration, presence of necrosis within a solid tumour, cystic or solid-cystic lesions with thick and irregular walls or septa and/or with papillary projections, demonstrating contrast enhancement. Ancillary findings such as pelvic organ invasion, ascites, peritoneal metastases and adenopathy increase the diagnostic confidence of malignancy [6-11]. Ovarian carcinomas associated with endometrial hyperplasia or endometrial carcinoma include endometrioid carcinoma, granulosa cell tumour and rarely, fibroma or fibrothecoma [6-10].Background
Although synchronous multiple primary tumours are extremely rare, they usually involve the genitourinary and gastrointestinal tract, followed by both breast and genitourinary tract and breast and gastrointestinal tract [1]. Among gynaecologic malignancies, synchronous primary tumours of the endometrium and ovary are the most common. The incidence is reported as about 5% of patients with endometrial cancer and 10% of patients with ovarian cancer [2-4]. The pathogenesis of synchronous endometrial and ovarian carcinomas is unclear. The theory of “secondary Mullerian system” proposed that the epithelia of cervix, uterus, fallopian tubes, ovaries, and peritoneal surface share molecular receptors responding to carcinogenic stimulus, leading to the development of multiple primary malignancies synchronously. This hypothesis could provide explanation for synchronous malignancies of similar histology, but not in cases of dissimilar histology [2]. Various studies have reported that women with synchronous primary cancers of the endometrium and ovary have a better overall prognosis compared to those with a single-organ primary cancer and metastasis.
Simultaneous detection of malignancies of endometrium and ovary often challenges the clinicians and pathologists to make correct diagnosis and arrange appropriate management. These patients can be classified into three groups: primary endometrial cancer with ovarian metastasis, primary ovarian cancer with endometrial metastasis and synchronous primary endometrial and ovarian carcinoma. Several pathologic criteria have been proposed for distinguishing metastatic tumour from synchronous primary cancer, although this may remain challenging [1-5]. Among them, histologic dissimilarity of carcinomas, absence of vascular space invasion, ovarian tumour located in the parenchyma, without surface implants or other evidence of spread, endometrial cancer invading less than 50% of the myometrium, without evidence of spread, coexistence of atypical endometrial hyperplasia or ovarian endometriomas suggest the presence of synchronous primary carcinomas. In this patient, histologic diagnosis was based on absence of vascular space invasion, absence of any spread of ovarian malignancies and presence of endometriotic cysts.
Imaging perspective
Imaging findings that are strongly suggestive of ovarian malignancy include a mass partly cystic-solid, with solid parts as areas of restricted diffusion, enhancing after contrast administration, presence of necrosis within a solid tumour, cystic or solid-cystic lesions with thick and irregular walls or septa and/or with papillary projections, demonstrating contrast enhancement. Ancillary findings such as pelvic organ invasion, ascites, peritoneal metastases and adenopathy increase the diagnostic confidence of malignancy [6-11]. Ovarian carcinomas associated with endometrial hyperplasia or endometrial carcinoma include endometrioid carcinoma, granulosa cell tumour and rarely, fibroma or fibrothecoma [6-10].

Differential Diagnosis List

Final Diagnosis

Synchronous primary endometrioid adenocarcinomas of the ovary and endometrium.

URL:	https://www.eurorad.org/case/10745
DOI:	10.1594/EURORAD/CASE.10745
ISSN:	1563-4086