Para ovarian mass of uncertain radiological morphology: A case report

A 45-year-old married female came to the hospital with lower abdominal pain and physical examination showed tenderness at that area. The pain was sudden and was not of chronic nature. There was no history of abnormal uterine bleeding, urinary or bowel complaints. Upon further history taking, there is no prior history of surgical interventions or underlying medical conditions. Specifically, there is no history of dysmenorrhea, dyspareunia or previous diagnosis of uterine adenomyosis or endometriosis. Furthermore, there is no history of hormonal treatment (oral contraceptives, gonadotropin-releasing hormone analogues). There is no family history of gynecological malignancies or endometriosis. She consequently had an abdominal ultrasonography which revealed a right solid adnexal lesion with no obvious calcifications or features of adnexal / ovarian torsion. No other pelvic abnormalities were detected ( Fig. 1 ). Unfortunately, no colored Doppler was obtained and the patient has not undergone vaginal ultrasound. Subsequent MR of the pelvis revealed a right adnexal lesion, measuring approximately 8 cm which demonstrates heterogeneous high signal on T2, Iso-intense on T1, with heterogeneous enhancement, no uterine abnormality noted ( Fig. 2 , Fig. 3 ). Upon laboratory investigations, the complete blood count showed no anemia with a normal hemoglobin count of 13 (the normal Hb level for females is 12 to 16 g/dL). Also, beta human chorionic gonadotropin was negative. Tumor markers such as CEA, CA19-9 and CA-125 were not taken. Ultimately, the treating team's approach was to opt for definitive treatment which was an open right oophorectomy and fallopian tube excision for the patient. The patient later reported the disappearance of symptoms. Histologically, the sections show numerous benign endometrial glands and endometrial stroma which are bordered by smooth muscle fibers with no evidence of cytologic atypia, mitoses or necrosis. ( Fig. 4 ). For the gross description, there were multiple smooth and irregular surfaces, soft, cystic, and rubbery consistency pieces of tissue measuring collectively 8 cm with whitish ray cut surfaces and focal hemorrhage right adnexal lesion. For the microscopic description, there were bundles of benign looking smooth muscle cells with edematous myxoid changes. Among these muscle fibers there are benign looking glands lined by columnar epithelial cells. Some of these glands are lined by tubal like columnar ciliated epithelial cells. The diagnosis was para ovarian adenomyoma. Fig. 1 US image shows a heterogenous isoechoic solid right adnexal lesion measuring 6.6 × 5.3 × 6.4 cm. Fig 1: Fig. 2 Sagittal T2 MR shows a normal sized uterus with normal endometrial stripe and junctional zone thickness. Intrauterine contraceptive device (IUCD) noted inside the endometrial cavity. Fig 2: Fig. 3 (A) Coronal T2. (B) Axial T2 fat-sat. (C and D) Axial T1 pre-and postcontrast MR of the pelvis. It is showing a right adnexal mixed solid and cystic lesion measuring 11.3 cm in the largest dimension, it demonstrates an isointense T1 and heterogenous high T2 signal. There is a heterogeneous enhancement postcontrast administration. (E and F) High b value diffusion-weighted image with corresponding ADC map show no restricted diffusion. Fig 3: Fig. 4 Histopathologic findings of paraovarian adenomyoma. (A) Benign endometrial glands and endometrial stroma (arrow in [A]) bordered by smooth muscle fibers. (B) Smooth muscle proliferation (arrow in [B]) surrounding endometrial island. (C) High power magnification of endometrial gland and endometrial stroma without cytologic atypia. (D) High power magnification of endometrial island and smooth muscle fibers without cytologic atypia, mitosis or necrosis (hematoxylin-eosin; original magnifications X100 [A], X200 [C], X200 [D]). Fig 4: US image shows a heterogenous isoechoic solid right adnexal lesion measuring 6.6 × 5.3 × 6.4 cm. Sagittal T2 MR shows a normal sized uterus with normal endometrial stripe and junctional zone thickness. Intrauterine contraceptive device (IUCD) noted inside the endometrial cavity. (A) Coronal T2. (B) Axial T2 fat-sat. (C and D) Axial T1 pre-and postcontrast MR of the pelvis. It is showing a right adnexal mixed solid and cystic lesion measuring 11.3 cm in the largest dimension, it demonstrates an isointense T1 and heterogenous high T2 signal. There is a heterogeneous enhancement postcontrast administration. (E and F) High b value diffusion-weighted image with corresponding ADC map show no restricted diffusion. Histopathologic findings of paraovarian adenomyoma. (A) Benign endometrial glands and endometrial stroma (arrow in [A]) bordered by smooth muscle fibers. (B) Smooth muscle proliferation (arrow in [B]) surrounding endometrial island. (C) High power magnification of endometrial gland and endometrial stroma without cytologic atypia. (D) High power magnification of endometrial island and smooth muscle fibers without cytologic atypia, mitosis or necrosis (hematoxylin-eosin; original magnifications X100 [A], X200 [C], X200 [D]).

Written, informed consent was obtained from the patient for the publication of this study.

In conclusion, Extrauterine adenomyoma is a rare entity and preoperative diagnosis is quite difficult. We present the first case of Para ovarian Adenomyoma within the Eastern region in Saudi Arabia. The radiological diagnosis of Extrauterine adenomyoma is inconclusive and histopathological examination is necessary to confirm the diagnosis. This case emphasizes the presence of this rare entity and such differential diagnosis should be considered when dealing with such an unusual appearance in an extrauterine mass.

Extra-uterine adenomyoma is a quiet rare diagnosis. It resembles uterine adenomyoma but in heterotopic location outside the uterine corpus [ 1 ]. The presence of endometrial stroma and glands within the myometrium resembles Adenomyosis. While endometriosis resembles the ectopic location of endometrium outside the uterus. These conditions originate are typically observed in reproductive age women and are from a menstruation-related dysfunction. Pelvic pain, infertility, dyspareunia, dysmenorrhea which results from progesterone resistance, inflammation and hyperestrogenism are observed in both of them [ 11 ]. In MRI, adenomyosis appears as focal or diffuse thickening of the junctional zone with ill-defined area of low signal intensity [ 1 ]. While endometriotic cysts usually appear as high signal intensity in T1 and it demonstrates shading in T2 weighted imaging [ 12 ]. Extra-uterine adenomyoma on the other hand is the focal and localized form of adenomyosis which resembles the presence of endometrial glands and stroma within the myometrium happening outside of the uterus [ 1 ]. Upon imaging, Extra-uterine adenomyoma usually appears as hypoechoic solid mass with internal vascularity in ultrasound while in magnetic resonance imaging, the lesion has a lobulated contour with well-defined borders. On T1 it is usually hypointense and on T2 it is isointense to the smooth muscle, resembling uterine fibroid. This pattern of signal intensity is different than most of the of the more common adnexal masses such as endometrioma which usually is bright on T1 and dark on T2 due to the presence of blood products. Furthermore, extrauterine adenomyomas do not usually demonstrate restrictive diffusion or calcification and they usually enhance after IV contrast administration. However, sonographic appearance and magnetic resonance (MR) features of extrauterine adenomyoma are inconclusive. Nevertheless, magnetic resonance imaging is the only modality that demonstrates somewhat reliable findings to differentiate adenomyomas from other masses of pelvic or adnexal origin. Differential diagnoses of such a lesion include endometrioma, fibroid, and sarcoma [ 6 , 8 ]. Surgical excision and histopathology examinations are required to reach the accurate diagnosis [ [1] , [2] , [3] , [4] , [5] , [6] , [7] ]. Furthermore, 18 similar cases were identified in the literature and the most common clinical presentation includes nonspecific abdominopelvic pain [ 6 ]. Surgical resection is the mainstay of treatment with malignant transformation of rare occurrence. Specifically, focal endometriod and clear cell adenocarcinomas occurred [ [8] , [9] , [10] ]. No reported cases noted within Al Hassa-Saudi Arabia. Hence, this is the first case of extrauterine adenomyoma within this region. In our case, unfortunately limitations include that tumor markers such as CEA, CA19-9 and CA-125 were not taken. Furthermore, no colored Doppler was obtained and the patient has not undergone vaginal ultrasound. The strengths include the rarity of the disease worldwide and the absence of it in the literature within this region.

Presence of endometrial glands within the myometrium represents adenomyosis [ 1 , 2 ]. On rare occasions, adenomyosis is located outside of the uterine tissue [ 1 , [3] , [4] , [5] ]. Consequently, the term of extrauterine adenomyoma is given. Definitive diagnosis is made by histopathology. However, imaging can provide a provisional diagnosis. Transvaginal and transabdominal sonography is used as an initial modality of imaging. Transvaginal sonography has better spatial resolution in comparison to the transabdominal approach. The higher imaging modality such as magnetic resonance imaging (MRI) is a noninvasive highly accurate modality to diagnose adenomyosis. The appearance of adenomyosis in MRI includes focal or diffuse thickening of the junctional zone which forms an ill-defined area of low signal intensity. Furthermore, it is also used to distinguish adenomyosis from a case of an enlarged uterus which has leiomyoma. The presence of adenomyoma outside of the uterus is very rare. Imaging features of extrauterine adenomyoma is nonspecific and are yet to be described in the literature [ 6 , 7 ]. If adenomyoma occurs outside of the uterus it mostly occurs at the ovary, broad ligament and pararectal space. It also occurs at the para ovarian, parametrial, pelvic wall and the round ligament. Furthermore, the appendix, small bowel mesentery and liver are other locations that are noted in the literature as well [ 1 ]. We report a case of an extrauterine adenomyoma located within the right adnexa confirmed by histopathology. The aim of the study is to highlight the presence of this disease. Furthermore, upon searching the literature, it showed 18 similar cases. However, there is no reported case noted within Al Hassa-Saudi Arabia. Hence, this is the first case of extrauterine adenomyoma within this region.