Adnexal Masses in Female Pediatric Patients

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1 Pediatric Imaging Clinical Perspective nthony et al. dnexal Masses in Female Pediatric Patients Pediatric Imaging Clinical Perspective Downloaded from by on 12/27/17 from IP address Copyright RRS. For personal use only; all rights reserved FOCUS ON: Evelyn Y. nthony 1 Melanie P. Caserta Jasmeet Singh Michael Y. M. Chen nthony EY, Caserta MP, Singh J, Chen MYM Keywords: adnexal pathology, infection, neoplasms, pediatric imaging, vascular compromise DOI: /JR Received September 2, 2011; accepted after revision November 7, ll authors: Department of Radiology, Wake Forest University School of Medicine, Medical Center lvd, Winston-Salem, NC ddress correspondence to E. Y. nthony (eanthony@wfubmc.edu). WE This is a Web exclusive article. JR 2012; 198:W426 W X/12/1985 W426 merican Roentgen Ray Society dnexal Masses in Female Pediatric Patients OJECTIVE. This article reviews the range of adnexal masses that present in pediatric females. The preferred imaging modalities, the appearance of the normal ovaries, and the epidemiology of ovarian diseases and abnormalities are discussed. The illustrated abnormalities include simple and complex ovarian and paraovarian cysts, neoplasms, ovarian torsion, ectopic pregnancy, and tuboovarian abscess, with attention to the imaging features and vascular flow patterns that help distinguish surgical from nonsurgical cases, malignant from benign lesions, and ovarian abnormalities from mimickers. CONCLUSION. The critical clinical questions to the radiologist in the setting of adnexal lesions are the site of origin, benign versus malignant features, and presence of infection or abscess. Pairing clinical presentation and imaging findings will direct appropriate management, whether it is reassurance, follow-up imaging, or surgery. dnexal abnormalities in pediatric patients are uncommon but not rare. In fact, the breadth of adnexal disease diagnosed in adult females is seen in the pediatric population, albeit with a different incidence profile. Diagnosis may be more difficult, even delayed or missed, because of low index of suspicion; nonspecific complaints; or consideration of more common, acute abdominal processes that mimic adnexal issues. This article will review the imaging options and features of adnexal lesions, including neoplasms, vascular compromise, infection, and some important paraovarian mimickers in pediatric patients. Technique The preferred initial imaging modality of the female pelvis remains gray-scale ultrasound with additional color and pulsed wave Doppler imaging. Ultrasound is safe, inexpensive, and free of ionizing radiation. With the patient s bladder distended, a sonographic window is created by which the ovaries can generally be identified. The ovaries may be particularly prominent in infant females because of the lingering presence of maternal hormones, and small follicles are often noted even in prepubertal girls (Fig. 1). The typical mean ovarian volume is roughly 1 cm 3 in girls up to 5 years old; the ovaries at least double in size by the age of 12 years [1]. t puberty, ovaries increase in size to approximately 4 cm 3 [2], with growth paralleling uterine maturation. When lesions are discovered, the sonographer gathers information about the size and internal characteristics of the lesions and presence and quality of vascular flow. If further imaging assessment is required, both CT and MRI are available and provide specific benefits. MRI, like ultrasound, functions without ionizing radiation. It also offers superb soft-tissue contrast, including dynamic enhancement for evaluation of vascular integrity. However, MRI takes longer to perform and may require sedation of the patient, making it more cumbersome in emergency situations. Thus, CT remains important in this setting because of its speed, spatial resolution, and ready global assessment of the abdomen and pelvis, especially for surgical planning. Mimickers of adnexal abnormalities and diseases can be excluded, and staging of malignancy can be performed. Ovarian Cysts The most common adnexal abnormality in the pediatric population is an ovarian cyst. Ovarian cysts are the most frequent cause of an abdominal mass in the fetus and in the newborn [3]. n anechoic focus in an ovary is considered a follicle if it is smaller than 3.0 cm. mature dominant follicle may fail to involute W426 JR:198, May 2012

dnexal Masses in Female Pediatric Patients Downloaded from www.ajronline.org by 37.44.196.146 on 12/27/17 from IP address 37.44.196.146. Copyright RRS. For personal use only; all rights reserved Fig.

Small follicles/cysts can be seen in prepubertal girls, especially in infants who have residual maternal hormones. appropriately and may enlarge into a functional cyst or corpus luteum.

ased on the degree of complexity associated with blood products, clot formation, lysis, and retraction, appearances are variable on all cross-sectional imaging modalities (Fig. 2). Fig.

Macroscopic fat within mass on CT is diagnostic of teratoma, and more than 50% also show coarse calcifications, as in this case. Fig. 2 Hemorrhagic ovarian cyst.

, Color Doppler ultrasound image shows complex cystic mass with fluid and layering echogenic material (arrow).

Ultrasound appearances include reticular, fishnet, or lacy pattern; retracting hyperechoic clot; fluid-debris level; and diffuse low-level echoes, with the latter being more typical of endometrioma.

2 dnexal Masses in Female Pediatric Patients Downloaded from by on 12/27/17 from IP address Copyright RRS. For personal use only; all rights reserved Fig. 1 6-month-old female infant with elevated estrogen levels. Transabdominal image of pelvis shows normal ovary containing small follicles (arrow). Small follicles/cysts can be seen in prepubertal girls, especially in infants who have residual maternal hormones. appropriately and may enlarge into a functional cyst or corpus luteum. Rupture or hemorrhage often brings these patients to medical attention. ased on the degree of complexity associated with blood products, clot formation, lysis, and retraction, appearances are variable on all cross-sectional imaging modalities (Fig. 2). Fig year-old girl with acute-onset abdominal pain and vomiting. xial CT image shows left-sided mass that contains fat and calcification (arrow). Macroscopic fat within mass on CT is diagnostic of teratoma, and more than 50% also show coarse calcifications, as in this case. Fig. 2 Hemorrhagic ovarian cyst., 12-year-old girl with adnexal mass by outside referral report. CT scan reveals complex right adnexal cyst. Layering high-density material (arrow) is characteristic of hemorrhage., Color Doppler ultrasound image shows complex cystic mass with fluid and layering echogenic material (arrow). Note absence of vascular flow within solid components, which is key feature in differentiating hemorrhagic cyst from neoplasm. Hemorrhagic cysts vary in appearance related to age of lesion. Ultrasound appearances include reticular, fishnet, or lacy pattern; retracting hyperechoic clot; fluid-debris level; and diffuse low-level echoes, with the latter being more typical of endometrioma. Whether simple or complex, these lesions are followed with ultrasound to confirm resolution and exclude cystic neoplasm. Fig. 4 8-year-old girl with intermittent abdominal pain, worsening 2 days before admission. Color Doppler ultrasound image shows cystic mass with calcifications (not shown) and echogenic mural nodule with shadowing (arrow), highly suggestive of teratoma. Shadowing echogenic mural nodule is known as Rokitansky plug and represents sebaceous material. This lesion shows no color flow, which is concerning for torsion; torsion was subsequently confirmed at surgery. Sonographic appearance of teratoma is highly variable; teratomas are usually heterogeneous with highly echogenic components secondary to fat and calcifications and possibly fat-fluid level. Torsion is common association with teratomas. Ovarian Neoplasms Ovarian neoplasms commonly have a cystic component and may be benign or malignant. The solid component is the most statistically significant predictor of malignancy [4]. Categories are defined by the cell of origin: germ cell tumors, epithelial tumors, and stromal tumors. These neoplasms generally present in postpubertal girls as a result of pain, increasing abdominal girth, and symp- Fig. 5 3-year-old girl with history of immature teratoma diagnosed at 11 months of age who presented with metastatic disease. Coronal CT reconstruction image shows peritoneal carcinomatosis encasing organs within abdomen. Note calcifications and fat (arrow) within mature components of tumor. JR:198, May 2012 W427

nthony et al. Downloaded from www.ajronline.org by 37.44.196.146 on 12/27/17 from IP address 37.44.196.146. Copyright RRS. For personal use only; all rights reserved Fig.

xial CT image shows large right-sided mass with cystic and solid enhancing (arrow) components without fat or calcification. Pathologic diagnosis was yolk sac tumor. Fig.

Sex cord stromal tumors are morphologically similar to granulosa theca cell tumor, and clinical factors are critical to diagnosis.

Similarly, fibrothecomas may secrete estrogen with secondary thickening of endometrial stripe, but fibrothecomas may have marked posterior acoustic attenuation on ultrasound owing to dense nature of

enign teratomas comprise 67% of pediatric ovarian neoplasms and are bilateral in up to 25% of cases [5].

3 nthony et al. Downloaded from by on 12/27/17 from IP address Copyright RRS. For personal use only; all rights reserved Fig year-old girl with acute-onset abdominal pain and vomiting (same patient as in Fig. 3). xial CT image shows large right-sided mass with cystic and solid enhancing (arrow) components without fat or calcification. Pathologic diagnosis was yolk sac tumor. Fig. 8 Granulosa theca cell tumor in 14-year-old girl with progressive abdominal distention. xial CT image shows mostly solid mass (arrow) with cystic components (asterisk) arising from right adnexa. Sex cord stromal tumors are morphologically similar to granulosa theca cell tumor, and clinical factors are critical to diagnosis. Granulosa cell tumors secrete estrogen and can cause precocious puberty and menorrhagia; tumors have spongelike appearance on ultrasound, and thickening of endometrial stripe is often present. Similarly, fibrothecomas may secrete estrogen with secondary thickening of endometrial stripe, but fibrothecomas may have marked posterior acoustic attenuation on ultrasound owing to dense nature of solid mass. Sertoli-Leydig cell tumors present clinically with virilization. toms derived from hormonal effects when masses are functional. The first category of neoplasms is germ cell tumors. enign teratomas comprise 67% of pediatric ovarian neoplasms and are bilateral in up to 25% of cases [5]. Mature teratomas contain tissue from all three primitive lines endoderm, mesoderm, and ectoderm. The presence of fat or calcification in the lesion is diagnostic and easily distinguished by CT (Fig. 3) or MRI. The diagnosis may be slightly more challenging by ultrasound (Fig. 4), but several signs are helpful including the tip-of-the-iceberg sign (coarse, shadowing calcification), dermoid mesh (linear interfaces representing hair), and dermoid plug (echogenic nodule with fat, hair, and teeth). Some teratomas contain immature elements Fig. 7 CT appearance of epithelial neoplasms., 15-year-old girl with benign serous cystadenoma of right ovary who presented with increased abdominal girth. Sagittal CT reconstruction image shows large cystic mass arising from pelvis. Cystadenomas can be quite large at presentation; however, absence of enhancing septations or solid components suggests benign cause. Serous neoplasms represent 25% of benign neoplasms and 50% of malignant lesions. Psammomatous calcifications may be present in malignant tumors., 18-year-old woman with mucinous cystadenocarcinoma arising from mature teratoma. Fat and calcifications (not shown) were noted in portion of mass. xial contrast-enhanced CT image shows large cystic mass with solid enhancing components (arrows). Note cystic portions of mass have varying densities of fluid (asterisks); this feature is seen with mucinous neoplasms because of differences in concentrations of mucin within loculi. Fig year-old girl with small cell carcinoma of ovary. xial CT image highlights large solid and cystic mass (arrow) centrally within abdomen. This rare neoplasm of uncertain cell origin occurs in young adolescents and women and has a poor prognosis. lthough this tumor is not associated with RC gene mutation, genetic proclivity exists given that this patient s mother and aunt were unsuccessfully treated for same disease. W428 JR:198, May 2012

xial CT scan shows enlarged ovary lined with peripheral follicles (arrows), thereby giving string-of-pearls sign.

On CT or MRI, deviation of uterus toward affected side and beak sign with congested vessels may also be present. Fig.

, xial CT image illustrates large, predominantly solid mass (arrow).

Consider hemangioma when ovarian lesion is highly vascular. Calcifications and hemorrhage may also be present. Fig.

, Transabdominal ultrasound image depicts marked enlargement of left ovary (arrow), which is larger than uterus (arrowhead).

Other ultrasound features of torsion include high-resistance arterial flow with loss of venous flow, twisted vascular pedicle, and

4 dnexal Masses in Female Pediatric Patients Downloaded from by on 12/27/17 from IP address Copyright RRS. For personal use only; all rights reserved Fig year-old girl with ovarian torsion. xial CT scan shows enlarged ovary lined with peripheral follicles (arrows), thereby giving string-of-pearls sign. lso, note central low attenuation concerning for infarcting tissue. On CT or MRI, deviation of uterus toward affected side and beak sign with congested vessels may also be present. Fig year-old girl referred for imaging of abdominal mass. Mass was diagnosed as ovarian hemangioma at surgery., xial CT image illustrates large, predominantly solid mass (arrow)., Next sequential image nicely demarks enlarged vessels (arrow) within mass. Ovarian hemangiomas are rare benign tumors. Consider hemangioma when ovarian lesion is highly vascular. Calcifications and hemorrhage may also be present. Fig year-old premenarchal girl with ovarian torsion., Transabdominal ultrasound image depicts marked enlargement of left ovary (arrow), which is larger than uterus (arrowhead). UT = uterus, LT OV = left ovary., Color power Doppler image shows absence of flow to left ovary. Other ultrasound features of torsion include high-resistance arterial flow with loss of venous flow, twisted vascular pedicle, and corkscrew vessels. Fig year-old girl with right ovarian torsion and infarction at surgery. Initial imaging interpretation from outside institution defined cause of pain as uterine mass., Coronal fat-suppressed T2-weighted image highlights enlarged ovary with multiple peripheral follicles as evidenced by small hyperintense structures (arrowheads)., Gadolinium-enhanced image shows no enhancement within ovary (asterisk), which is consistent with infarction. JR:198, May 2012 W429

, xial CT image shows calcified adnexal mass in right pelvis (arrow)., Coronal CT reconstruction image shows ovoid mass with calcifications (arrow) in left upper quadrant.

Wandering ovary is mobile, calcified structure that results from autoamputation of torsed ovary. and have potential for recurrence and metastasis.

Other categories of germ cell tumors include dysgerminoma, yolk sac tumor (Fig. 6), choriocarcinoma, and mixed varieties [7].

5 nthony et al. Downloaded from by on 12/27/17 from IP address Copyright RRS. For personal use only; all rights reserved Fig month-old girl discovered to have bilateral calcified cystic masses on CT scan obtained for evaluation of trauma. Surgical resection revealed congenital ovarian torsion., xial CT image shows calcified adnexal mass in right pelvis (arrow)., Coronal CT reconstruction image shows ovoid mass with calcifications (arrow) in left upper quadrant. Ovoid nature of mass with calcifications is clue to diagnosis of wandering ovary, which is rare cause of abdominal calcification in child. Wandering ovary is mobile, calcified structure that results from autoamputation of torsed ovary. and have potential for recurrence and metastasis. This subtype may even present in infants and may result in peritoneal spread of disease. Chemotherapy can lead to maturation of the implants to a benign form, despite persistent bulk [6] (Fig. 5). Other categories of germ cell tumors include dysgerminoma, yolk sac tumor (Fig. 6), choriocarcinoma, and mixed varieties [7]. These tumors are indistinguishable by imaging, but imaging features direct the operative approach and provide preoperative staging. Final diagnosis and staging are the purview of pathology. The second category of neoplasms is epithelial tumors cystadenomas and cystadenocarcinomas. Most tumors in this category are divided into serous and mucinous subtypes, both of which are often quite large at presentation (Fig. 7). Imaging highlights internal septations, papillary projections, and/ Fig. 15 Ectopic pregnancy., 17-year-old girl with history of ectopic pregnancy treated with methotrexate. CT scan shows large amount of high-density fluid (arrow) within pelvis, which is concerning for rupture of ectopic pregnancy. Surgery confirmed dilated and focally ruptured right fallopian tube., Ultrasound image of right adnexa in young adult female shows classic findings of ectopic pregnancy. Thickwalled echogenic structure (arrow) contains gestational sac (asterisk) and yolk sac (arrowhead) next to right ovary (C). Yolk sac outside uterus is diagnostic of ectopic pregnancy. or larger solid components. On ultrasound, mucinous tumors may have low-level internal echoes from mucoid material. The overall rate of malignancy ranges from 7.5% to 30% [4, 8]. Imaging descriptions address the probability of malignancy, lesion vascularity, ovary of origin, and signs of lesion rupture. The uterus is usually rotated toward the side of tumor origin [9]. The third category of primary neoplasms is tumors of stromal cell origin. These tumors include granulosa-thecal cell tumors, fibromas, thecomas, Sertoli-Leydig cell tumors, and undifferentiated sex cord stromal tumors. Many are hormonally active and, therefore, present earlier than those without function [10]. Typically, these tumors are solid, unilateral, and contained (Fig. 8). They may appear similar to germ cell tumors without fat or calcium, but presentation may foreshadow cell origin. Other rare ovarian neoplasms range from aggressive malignancies, such as small cell carcinoma (Fig. 9), which is usually associated with hypercalcemia, to benign entities, such as hemangiomas (Fig. 10). Metastatic disease can also involve the ovaries, either from hematogenous or contiguous spread. Within this group of malignancies are adenocarcinoma of the colon, urkitt lymphoma, alveolar rhabdomyosarcoma, Wilms tumor, neuroblastoma, and retinoblastoma [11]. Ovarian Surgical Emergencies ny ovarian or tubal mass predisposes a patient to ovarian torsion. Of course, ovarian torsion can occur without a lead point secondary to excessive mobility of the ovary or fallopian tube (Fig. 11). In either situation, torsion is a high-stakes imaging diagnosis and surgical emergency. The diagnosis may be particularly challenging if torsion is intermittent. The W430 JR:198, May 2012

dnexal Masses in Female Pediatric Patients Fig. 16 16-year-old girl with right tuboovarian abscess that was confirmed by surgery.

6 dnexal Masses in Female Pediatric Patients Fig year-old girl with right tuboovarian abscess that was confirmed by surgery. CT scan shows thick-walled, rim-enhancing fluid collection (arrow) with adjacent inflammatory stranding in right adnexa. Tuboovarian abscess represents advanced pelvic inflammatory disease and imaging may show thickwalled fallopian tube distended by fluid, enlarged edematous ovaries, and pelvic abscess. Identification of normal appendix is important to exclude appendicitis with complicating features. Downloaded from by on 12/27/17 from IP address Copyright RRS. For personal use only; all rights reserved most important finding is an enlarged, rounded ovary compared with the contralateral side. Follicles often migrate to the periphery, the so-called string-of-pearls sign [12] (Fig. 12). ecause a normal Doppler examination cannot exclude early or intermittent torsion at the time of the study, abnormal ovarian morphology is suspicious for intermittent torsion. Poor vascular flow or apparent lack of flow by ultrasound without morphologic changes is indeterminate. In pediatric patients, ultrasound via a transabdominal approach through a distended bladder is a complete examination. If advanced imaging is required, MRI without and with contrast material (Fig. 13) can confirm the diagnosis and may highlight any underlying mass. Torsion can occur in utero. nonviable ovary in an infant ranges in appearance from a complex cystic and solid mass without vascular flow (Fig. 14) to a small calcified mass visible on radiographs. torsed ovary in an infant may lie in the abdomen rather than the pelvis. The other adnexal emergency, which occurs in postpubertal girls, is ectopic pregnancy hence, the importance of the pregnancy test in the clinical evaluation. ny complex adnexal mass with an empty uterus and positive pregnancy test is highly concerning. The presence of complex ascites strengthens the case (Fig. 15). Risk factors include pelvic inflammatory disease, previous ectopic pregnancy, intrauterine device, in vitro fertilization, and tubal surgery [13]. Pelvic Infections Pelvic infection may emanate from the adnexa or may involve the adnexal regions from adjacent structures, creating an imaging appearance similar to that of neoplasms with complicating features, torsion, or ectopic pregnancy. Classic imaging findings of tuboovarian abscess and pyosalpinx are complex fluid collections with thick walls and rim enhancement or increased peripheral vascular flow, often with adjacent inflammatory change and free fluid (Fig. 16). Ultrasound depicts septations well, but CT and MRI offer a global assessment, which is often required preoperatively [14]. CT and MRI are also better than ultrasound at excluding appendicitis, a much more common diagnosis in this population, and phlegmon or abscess associated with inflammatory bowel disease. Importantly, Crohn disease without abscess may not be treated surgically so correct diagnosis of this entity affects management. Other nongynecologic pelvic abnormalities are beyond the scope of this article. The critical clinical questions to the radiologist in the setting of adnexal lesions are the site of origin, benign versus malignant features, and presence of infection or abscess. Pairing clinical presentation and imaging findings will direct appropriate management, whether it is reassurance, follow-up imaging, or surgery. Ultrasound remains the imaging workhorse for the detection and diagnosis of pediatric pelvic abnormalities and diseases, and CT and MRI provide complementary information when more detail is required or when questions persist. References 1. Servaes S, Victoria T, Lovrenski J, Epelman M. Contemporary pediatric gynecologic imaging. Semin Ultrasound CT MR 2010; 31: Ziereisen F, Guissard G, Damry N, vni EF. Sonographic imaging of the paediatric female pelvis. Eur Radiol 2005; 15: Heling KS, Chaoui R, Kirchmair F, Stadie S, ollmann R. Fetal ovarian cysts: prenatal diagnosis, management and postnatal outcome. Ultrasound Obstet Gynecol 2002; 20: Oltmann SC, Garcia N, arber R, Huang R, Hicks, Fischer. Can we preoperatively risk stratify ovarian masses for malignancy? J Pediatr Surg 2010; 45: Outwater EK, Siegelman ES, Hunt JL. Ovarian teratomas: tumor types and imaging characteristics. RadioGraphics 2001; 21: Choudhary S, Fasih N, McInnes M, Marginean C. Imaging of ovarian teratomas: appearances and complications. J Med Imaging Radiat Oncol 2009; 53: Smith HO, erwick M, Verschraegen CF, et al. Incidence and survival rates for female malignant germ cell tumors. Obstet Gynecol 2006; 107: Morowitz M, Huff D, von llmen D. Epithelial ovarian tumors in children: a retrospective analysis. J Pediatr Surg 2003; 38: Hiller N, ppelbaum L, Simanovsky N, Lev-Sagi, haroni D, Sella T. CT features of adnexal torsion. JR 2007; 189: Cecchetto G, Ferrari, ernini G, et al. Sex cord stromal tumors of the ovary in children: a clinicopathological report from the Italian TREP project. Pediatr lood Cancer 2011; 56: McCarville M, Hill D, Miller E, Pratt C. Secondary ovarian neoplasms in children: imaging features with histopathologic correlation. Pediatr Radiol 2001; 31: Servaes S, Zurakowski D, Laufer MR, Feins N, Chow JS. Sonographic findings of ovarian torsion in children. Pediatr Radiol 2007; 37: Perlman S, Hertweck P, Fallat ME. Paratubal and tubal abnormalities. Semin Pediatr Surg 2005; 14: Kim MY, Rha SE, Oh SN, et al. MR imaging findings of hydrosalpinx: a comprehensive review. RadioGraphics 2009; 29: JR:198, May 2012 W431

A Practical Approach to Adnexal Masses

A Practical Approach to Adnexal Masses Darcy J. Wolfman, MD Section Chief of Genitourinary Imaging American Institute for Radiologic Pathology Clinical Associate Johns Hopkins Community Radiology Division