Abstract
Malignant transformation is a rare complication of endometriosis. MR criteria for diagnosis include the presence of soft-tissue components, enhancement after contrast material administration in an endometriotic cyst. We present the multidetector CT and MR imaging findings in a case of an incidentally found endometrioid adenocarcinoma in a left-sided ovarian endometrioma, occurring in a 30-year old woman. MR imaging enabled the correct preoperative characterization of the lesion, by depicting a soft-tissue element, with strong and early enhancement after gadolinium administration. The same area had high signal intensity on diffusion-weighted images and low apparent diffusion coefficient values due to restricted diffusion, findings also strongly suggestive of malignancy.
Keywords: endometriosis, endometriotic cyst, carcinoma, imaging, multidetector CT, magnetic resonance
CASE REPORT
A 30-year old multiparous woman was referred to the Gynecology clinic, for a complex left adnexal mass, discovered incidentally during a sonographic examination (Figure 1). Cancer antigen CA 125 and CA 19-9 levels were elevated: 130 U/ml (normal values < 35 U/ml) and 43 U/ml (normal values < 39 U/ml), respectively).
Imaging findings
Multidetector CT (MDCT) examination of the abdomen was performed on a 16-row CT scanner (Mx8000 ID, Philips). The protocol included unenhanced CT scanning of the pelvis, using a detector collimation of 16 × 1.5 mm, followed by enhanced CT of the abdomen during the portal phase (scan delay: 70 sec), after the i.v. administration of iodinated contrast material (Visipaque, GE Healthcare) with a detector collimation of 16 × 0.75 mm. A large, multicystic left adnexal mass was revealed, composed mainly of hyperdense parts (CT density range: 45–85 HU), which were attributed to the presence of hemorrhagic content (Figure 2). No obvious lesion enhancement was detected (Figure 3, 4). Neither ascites nor lymphadenopathy was seen. Based on the MDCT findings, no obvious signs of malignancy were found.
MR imaging examination of the pelvis on a 1.5-Tesla MR unit (Intera, Philips), using a pelvic-phased array coil was followed. The protocol included turbo spin echo T2-weighted images in transverse, sagittal and coronal planes and axial spin echo T1-weighted images, before and after the application of a fat saturation prepulse. Dynamic contrast-enhanced fat-suppressed gradient-echo T1-weighted imaging was performed in the sagittal plane, after a rapid hand injection of gadolinium chelate components (Omniscan, GE Healthcare). Axial diffusion-weighted (DW) images, with a b-factor of 0 and 800 seconds/mm2 were also performed. The tumor had parts of high signal intensity on both T1 and T2-weighted images, suggestive for the presence of subacute haemorrhage. Others parts slightly hyperintense and extremely hypointense on T1 and T2-weighted images, respectively were considered as indicative for acute haemorrhage (Figure 5, 6, 7). The presence of a soft-tissue component, detected with intermediate signal intensity, similar to that of the normal myometrium on both T1 and T2-weighted images and early, strong enhancement on dynamic post-contrast MR images was revealed (Figure 5, 6, 7, 8). Subacute hemorrhagic components had intermediate signal intensity on diffusion-weighted images and a mean apparent diffusion coefficient value (ADC) of 1.37 × 10−3 mm2/second (Figure 9, 10). Acute hemorrhagic parts cause a dramatic signal drop in the area, due to T2* effect and had no measurable ADC values. The soft-tissue component was detected hyperintense on DW images, with low ADC values (1.05 × 10−3 mm2/second, Figure 9, 10), due to restricted diffusion. Ovarian malignancy was strongly indicated based on both conventional and diffusion-weighted MR imaging findings. The presence of an enhancing component within a blood-filled ovarian cyst was considered as suggestive of malignant transformation of a pre-existing endometrioma.
Fertility-preserving surgery was discussed with the patient and in this context initial laparotomy consisted of left salpingo-oophorectomy, biopsies of the right ovary, omenectomy, and peritoneal washings. Pathology showed a well-differentiated ovarian endometrioid adenocarcinoma arising from endometriosis (Figure 11, 12). Microscopic neoplastic foci of the same histologic type, and endometriotic implants were found on the contralateral ovary (Figure 13). Following this report the patient was counselled that fertility preservation was not possible given the presence of malignancy on the right ovary too. A second procedure was followed consisting of total abdominal hysterectomy and right salpingo-oophorectomy. The pathologic stage was IB by FIGO classification. The patient is now well, without signs of recurrence, 18 months after surgery on follow-up MR examination.
DISCUSSION
Endometriosis is a common disease affecting primarily women in the reproductive age group (1, 2, 3, 4, 5). Malignant transformation is a rare complication of endometriosis, with an incidence of malignancy in patients with ovarian endometriotic cysts reported 0.6–0.8% (6). Approximately 75% of tumors in patients with endometriosis arise in endometriotic cysts and less often in the rectovaginal septum, rectum and sigmoid colon (1). Endometrioid carcinoma of the ovary is the commonest histologic type of malignancy occurring in endometriomas, as it was in our case, followed by clear cell adenocarcinoma (1, 2, 6, 7, 8). Ovarian carcinomas associated with endometriosis are more often seen in younger women (30–50 years of age) when compared to ovarian carcinoma in women without endometriosis, as it was in this patient.
The typical appearance of an endometriosis-related carcinoma is that of a large ovarian cyst with hemorrhagic fluid and soft-tissue components, enhancing on postcontrast T1-weighted images (6, 7, 8). The above findings were seen in this patient. Both MDCT and MR imaging detected a large, multicystic adnexal mass, mainly composed of hemorrhagic content. The detection of the enhancing soft-tissue element was difficult on CT, due to the lesion hyperdensity. MR imaging, on the other hand clearly identified the solid tissue component, detected with intermediate signal intensity on T2-weighted images and enhancing strongly during the early phase, on dynamic contrast-enhanced images. Dynamic contrast-enhanced MR imaging has proved useful in differentiating malignancies from benign tumors due to the differences in contrast tissue behavior caused by neoangiogenesis (9, 10, 11). Ovarian tumors usually demonstrate an early and strong enhancement, as was the case in our patient. Based on MR imaging features, a reliable preoperative characterization of the malignant nature of the mass was possible.
Diffusion-weighted (DW) MR imaging has recently been used in the evaluation of the female pelvic malignancies (12, 13, 14, 15, 16, 17, 18). It has been reported that high b value DW imaging depicted high signal intensity for malignant tumors when compared to normal tissues and benign lesions with lower ADC values for the former, because malignancies have a larger cell diameter and denser cellularity, therefore restricting water diffusion. These criteria were also met in our patient, although the MR diagnosis was readily made by the conventional MR imaging. The tumorous component was hyperintense on DW images, with a low ADC value (1.05 × 10−3 mm2/second), a finding suggestive of restricted diffusion. Hemorrhagic components on the other hand had intermediate signal intensity on DW imaging, with ADC values of 1.37 × 10−3 mm2/second. Endometriomas are reported with low ADC values, when compared with benign and malignant cystic ovarian malignancies, due to the presence of thick proteinaceous fluid or blood products (16, 17, 18).
Differential diagnosis of an endometriosis-related carcinoma should include intracystic blood clot and decidual changes of the ectopic endometrium within an endometrioma during pregnancy (6, 7, 19). The absence of contrast enhancement enables the reliable recognition of a blood clot within an adnexal cystic lesion (6). Ectopic endometrial stroma can undergo decidual change during pregnancy and close follow-up of pregnant women with such changes in an endometrioma is mandatory to rule out malignancy (6, 19).
TEACHING POINT
Endometriosis is considered a precancerous condition for ovarian malignancy, although rarely. The presence of an enhancing soft-tissue component within a blood-filled ovarian cystic mass is suggestive of malignancy and ovarian carcinoma arising in an endometriotic cyst should be considered. Early and strong enhancement of the soft-tissue element on dynamic contrast-enhanced MR imaging and restricted diffusion on DW MR images confirm the diagnosis of malignancy.
Table 1.
Etiology | Unopposed estrogen therapy |
Incidence | 0.6–0.8% |
Age predilection | 4–5th decade |
Treatment | Staging laparotomy including radical hysterectomy and bilateral salpingo-oophorectomy |
Prognosis | Depends mainly on the stage of the disease at the time of diagnosis |
Imaging findings | MDCT-plain images: hyperdense parts (hemorrhagic content) MDCT-contrast-enhanced images: hyperdense, non-enhancing parts (hemorrhagic content): difficult to appreciate the contrast-enhancing elements MRI-T1: hyperintense (hemorrhagic content) and isointense parts (tumorous component), when compared to normal myometrium MRI-T2: hyperintense and extremely hypointense components (acute and subacute hemorrhagic content, respectively) and isointense parts (tumorous component), when compared to normal myometrium MRI-DW: isointense and slightly hyperintense components (hemorrhagic content) and hyperintense parts (tumorous component) MRI-contrast-enhanced images: strong and early enhancement on dynamic MRI (tumorous component) |
Table 2.
US | CT | MRI | |
---|---|---|---|
Carcinoma developed in a pre- existing ovarian endometrioma | Complex adnexal mass, with cystic and solid elements. Color and pulsed Doppler may show presence of blood vessels within solid components; a low resistive index and pulsatility index may also be detected | Multicystic adnexal mass, mainly with hyperdense parts, due to hemorrhagic content. The detection of solid, contrast-enhancing elements may be difficult | Hemorrhagic component T1: hyperintense T2: hyperintense or hypointense DWI: may show restricted diffusion Pattern of contrast enhancement: none or wall and septa enhancement (smooth, usually of thickeness < 3mm) Neoplastic component T1: intermediate T2: intermediate DWI: restricted diffusion Pattern of contrast enhancement: strong, early, heterogeneous enhancement |
Intracystic blood clot | Complex adnexal mass, with cystic elements and solid echogenic parts. Color Doppler may show absence of blood vessels within solid elements | Multicystic adnexal mass, predominantly hyperdense | T1: hyperintense T2: hyperintense or hypointense DWI: may show restricted diffusion Pattern of contrast enhancement: none or wall and septa enhancement (smooth, usually of thickeness < 3mm) |
Decidual changes developed within endometrioma during pregnancy | Complex adnexal mass, with cystic-solid parts or nodular projections. Color Doppler may show presence of vascularity within solid elements or nodular projections | Hemorrhagic component T1: hyperintense T2: hyperintense or hypointense DWI: may show restricted diffusion Pattern of contrast enhancement: no gadolinium is administered Solid component T1: intermediate T2: intermediate Serial monitoring is mandatory |
ABBREVIATIONS
- MDCT
multidetector CT
- MR
magnetic resonance
- DW
diffusion-weighted
- ADC
apparent diffusion coefficient
- FIGO
International Federation of Gynecology and Obstetrics
- i.v.
intravenous.
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