CT and MRI of Uterine Sarcomas and Their Mimickers

Size: px

Start display at page:

Download "CT and MRI of Uterine Sarcomas and Their Mimickers"

Cecily Marshall
6 years ago
Views:

Downloaded from www.ajronline.org by 37.44.206.60 on 01/10/18 from IP address 37.44.206.60. Copyright RRS.

1 Downloaded from by on 01/10/18 from IP address Copyright RRS. For personal use only; all rights reserved CT and MRI of Uterine Sarcomas and Their Mimickers Sung Eun Rha 1, Jae Young yun 1, Seung Eun Jung 1, Soo Lim Lee 1, Song Mee Cho 1, Seong Su Hwang 1, Hae Giu Lee 1, Sung-Eun Namkoong 2, Jae Mun Lee 1 U terine sarcomas are rare tumors of mesodermal origin, constituting only 2 6% of uterine malignant tumors [1]. The three most common histologic variants of uterine sarcomas are malignant mixed müllerian tumors, leiomyosarcomas, and endometrial stromal sarcomas. Uterine sarcomas are, in general, the most malignant group of uterine tumors; they differ from endometrial cancers with regard to diagnosis, clinical behavior, pattern of spread, and management [1]. The distinction among the various subgroups of uterine sarcoma and between uterine sarcomas and other uterine tumors cannot be made on clinical grounds. Therefore, imaging studies, particularly CT and MRI, are important for evaluating the pelvic mass at presentation and for aid in staging the tumor. This pictorial essay discusses the clinical and imaging features of each of the subtypes of uterine sarcoma and their mimickers. Malignant Mixed Müllerian Tumors Malignant mixed müllerian tumors are the most common of the uterine sarcomas and constitute about 2% of all corpus malignant tumors. Malignant mixed müllerian tumors are quite malignant and contain both carcinomatous and sarcomatous components. The epithelial component is usually an adenocarcinoma, and stromal sarcoma is the most common type of sarcoma [1]. Interestingly, 4 38% of patients with malignant mixed müllerian tumors have received pelvic irradiation for unrelated pelvic malignancies several years before the symptoms [2]. malignant mixed müllerian tumor usually grows as a large solid mass replacing the endometrial cavity; necrosis and hemorrhage Fig year-old woman with malignant mixed müllerian tumor. Sagittal T2-weighted (TR/TE, 2,000/80) spin-echo image shows markedly distended endometrial cavity (E) with peripheral polypoid masses. Patient had history of pelvic irradiation for cervix carcinoma 12 years previously. These characteristic imaging features are probably caused by stenosis of cervical canal resulting from previous pelvic irradiation. Pictorial Essay are prominent features (Figs. 1 3). The myometrium is invaded to various degrees in almost all cases. Malignant mixed müllerian tumor is usually in the fundus but can involve the cervix and sometimes presents as a protruding mass from the cervical os. lthough malignant mixed müllerian tumors may metastasize hematogenously, local and lymphatic spread and intraperitoneal seeding are more common [3] (Figs. 2 and 3). Received February 3, 2003; accepted after revision March 18, Department of Radiology, College of Medicine, The Catholic University of Korea, 505, anpo-dong, Seocho-Ku, Seoul , South Korea. ddress correspondence to J. Y. yun. 2 Department of Obstetrics and Gynecology, Kangnam St. Mary s Hospital, Seoul , South Korea. JR 2003;181: X/03/ merican Roentgen Ray Society JR:181, November

Downloaded from www.ajronline.org by 37.44.206.60 on 01/10/18 from IP address 37.44.206.60. Copyright RRS. For personal use only; all rights reserved Rha et al. Fig. 2.

ulky peritoneal mass (arrows) is also present in cul-de-sac, suggesting peritoneal metastasis.

lmost all of these tumors occur after menopause, at median age of 62 years. Most frequent presenting symptom is bleeding. M = endometrial mass.

Leiomyosarcomas may arise either de novo from uterine musculature or the connective tissue of uterine blood vessels or in a preexisting leiomyoma.

62-year-old woman with malignant mixed müllerian tumor. Contrast-enhanced CT scan shows markedly enlarged uterus totally replaced by mass and focal disruption of uterine margin (arrowheads).

2 Downloaded from by on 01/10/18 from IP address Copyright RRS. For personal use only; all rights reserved Rha et al. Fig year-old woman with malignant mixed müllerian tumor., Sagittal T2-weighted spin-echo image (TR/TE, 2,000/ 80) shows polypoid endometrial mass (M) distending uterine cavity. ulky peritoneal mass (arrows) is also present in cul-de-sac, suggesting peritoneal metastasis., Sagittal gadolinium-enhanced fat-suppressed T1weighted spin-echo image (583/11) shows irregular central nonenhancing necrotic portion of peritoneal mass (arrows). lmost all of these tumors occur after menopause, at median age of 62 years. Most frequent presenting symptom is bleeding. M = endometrial mass. Leiomyosarcomas Leiomyosarcomas account for one third of uterine sarcomas. Leiomyosarcomas may arise either de novo from uterine musculature or the connective tissue of uterine blood vessels or in a preexisting leiomyoma. The incidence of sarcomatous change in benign uterine leiomyomas is reported to be % [4]. Leiomyosarcoma usually presents as a massive uterine enlargement with irregular Fig year-old woman with malignant mixed müllerian tumor. Contrast-enhanced CT scan shows markedly enlarged uterus totally replaced by mass and focal disruption of uterine margin (arrowheads). Note inhomogeneous contrast enhancement and calcification. Lobulated peritoneal mass (arrows) is noted anterior to main mass. scites (f) is also seen. Intraperitoneal metastases are more common than hematogenous metastases in malignant mixed müllerian tumors. Peritoneum is usually involved in more than half of cases extending beyond uterus. Extent of tumor at time of diagnosis is most important prognostic factor central zones of low attenuation, suggesting extensive necrosis and hemorrhage (Fig. 4). Foci of calcification may be present. The pattern of tumor spread is to the myometrium, pelvic blood vessels and lymphatics, contiguous pelvic structures, abdomen, and then distantly, most often to the lungs. lthough it has been suggested that an irregular margin of a uterine leiomyoma on MRI is suggestive of sarcomatous transformation, Fig year-old woman with uterine leiomyosarcoma. Sagittal gadolinium-enhanced T1-weighted spinecho image (TR/TE, 200/20) shows massive uterine enlargement (arrows) with irregular central zones of low signal intensity, suggesting extensive tumor necrosis (N). Uterine leiomyosarcoma should be suspected if rapid uterine enlargement occurs, especially in postmenopausal women. ecause most leiomyosarcomas are located in myometrium, endometrial biopsy is not as useful as in other sarcomas, but it may establish diagnosis in as many as one third of patients in whom lesion is submucosal. the specificity of this finding has not been established [5]. Endometrial Stromal Sarcomas Endometrial stromal sarcomas constitute 0.2% of all uterine malignancies and 15 23% of primary uterine sarcomas. There is no relationship to parity, associated disease, or prior pelvic radiotherapy. Pathologically, endometrial Fig year-old woman with low-grade endometrial stromal sarcoma. Contrast-enhanced CT scan shows relatively well-defined low-density myometrial mass (arrows) that cannot be differentiated from intramural myoma on this scan. Usual preoperative diagnosis of low-grade endometrial stromal sarcoma is leiomyoma with unusual degree of bleeding. On CT scans, some cases of low-grade endometrial stromal sarcoma cannot be clearly differentiated from myoma. However, on MRI, endometrial stromal sarcoma usually has infiltrative margin and shows high-signal intensity on T2-weighted images. = bladder. JR:181, November 2003

, xial fat-saturated T2-weighted spin-echo image (TR/ TE, 2,000/80) shows large relatively well-marginated hyperintense mass (solid arrows) in left lateral wall of

, xial gadolinium-enhanced T1-weighted image (417/ 11) shows multiple internal septalike enhanced areas in mass (arrows), which was located predominantly in

Presence of bands of low signal intensity in areas of myometrial involvement of low-grade endometrial stromal sarcoma on T2-weighted images may be important imaging

C Fig. 7. 58-year-old woman with high-grade endometrial stromal sarcoma. = bladder, C = ovarian cyst., Sagittal T1-weighted gradient-echo FLSH image (TR/TE,162.3/4.

, Sagittal T2-weighted turbo spin-echo image (3,900/99) shows inhomogeneous increased signal intensity of mass (arrowheads).

Gross specimen (not shown) showed lobulated mass resembling fish flesh with hemorrhage and necrosis. Fig. 8.

3 Downloaded from by on 01/10/18 from IP address Copyright RRS. For personal use only; all rights reserved CT and MRI of Uterine Sarcomas Fig year-old woman with low-grade endometrial stromal sarcoma., xial fat-saturated T2-weighted spin-echo image (TR/ TE, 2,000/80) shows large relatively well-marginated hyperintense mass (solid arrows) in left lateral wall of uterus. Endometrial cavity is not distended (open arrows)., xial gadolinium-enhanced T1-weighted image (417/ 11) shows multiple internal septalike enhanced areas in mass (arrows), which was located predominantly in myometrium on pathologic examination. Presence of bands of low signal intensity in areas of myometrial involvement of low-grade endometrial stromal sarcoma on T2-weighted images may be important imaging finding for low-grade endometrial stromal sarcoma. Pathologically, this imaging finding is considered to be preserved myometrial bundles separated by tumor cells. C Fig year-old woman with high-grade endometrial stromal sarcoma. = bladder, C = ovarian cyst., Sagittal T1-weighted gradient-echo FLSH image (TR/TE,162.3/4.8; flip angle, 80 ) shows enlarged uterus with large homogeneous low-signal-intensity mass located mainly in endometrial cavity., Sagittal T2-weighted turbo spin-echo image (3,900/99) shows inhomogeneous increased signal intensity of mass (arrowheads). Note focal invasion of deep myometrium (arrow). C, Gadolinium-enhanced T1-weighted image (162.3/4.8) shows inhomogeneous contrast enhancement of mass. Gross specimen (not shown) showed lobulated mass resembling fish flesh with hemorrhage and necrosis. Fig. 8. High-grade endometrial stromal sarcoma in 20-year-old woman who had profuse vaginal bleeding. (Reprinted with permission from [9]), xial T2 -weighted turbo spin-echo image (TR/TE, 3,900/99) shows bulky lobulated intracavitary mass (arrows). Tumor invasion of entire thickness of myometrium at right-sided fundus (arrowhead) is well visualized., xial contrast-enhanced T1-weighted image (450/ 13) shows inhomogeneous contrast enhancement of uterine mass (straight arrows) that extends through entire thickness of myometrium (arrowhead). Endometrial cavity is mildly widened (curved arrow). Diagnosis can be confirmed by endometrial biopsy. JR:181, November

Hydrometra (E) is also present. Large amount of ascites () and peritoneal metastases (arrows) are also seen.

However, in practice, most patients with endometrial carcinomas have tumors that are confined to endometrium or that only superficially invade myometrium, and most malignant mixed müllerian tumors or

4 Downloaded from by on 01/10/18 from IP address Copyright RRS. For personal use only; all rights reserved Rha et al. Fig year-old woman with advanced endometrial carcinoma. Sagittal T2-weighted turbo spin-echo image (TR/TE, 3,900/99) shows enlarged uterus and diffuse tumor involving entire endometrial surface. Hydrometra (E) is also present. Large amount of ascites () and peritoneal metastases (arrows) are also seen. dvanced endometrial carcinoma cannot be clearly differentiated from malignant mixed müllerian tumor on images. However, in practice, most patients with endometrial carcinomas have tumors that are confined to endometrium or that only superficially invade myometrium, and most malignant mixed müllerian tumors or endometrial stromal sarcomas tend to be massive with deep myometrial invasion on initial presentation. and 8). Continuous extension of the lesion into the adjacent structures is frequently found on imaging because of marked vascular and lymphatic involvement of the tumor. Mimickers of Uterine Sarcomas Endometrial Carcinoma Occasionally, advanced endometrial carcinoma cannot be differentiated from endometrial stromal sarcoma or malignant mixed müllerian tumors by imaging findings (Fig. 9). Lymphoma stromal sarcoma is further subdivided into low-grade stromal sarcoma and high-grade stromal sarcoma, each of which shows different clinical presentations and outcomes. Low-Grade Endometrial Stromal Sarcomas Low-grade endometrial stromal sarcomas tend to occur in a younger age group than do high-grade endometrial stromal sarcomas. Low-grade endometrial stromal sarcomas tend to invade extensively into the myometrium and surrounding structures, even with scant cytologic atypia. The imaging findings of low-grade endometrial stromal sarcomas are variable, from a polypoid endometrial mass to a myometrial mass mim- icking intramural myoma with degeneration [6] (Figs. 5 and 6). cystic High-Grade Endometrial Stromal Sarcomas High-grade endometrial stromal sarcomas have a much more aggressive course and a poorer prognosis than low-grade endometrial stromal sarcomas and tend to occur in an older age group. High-grade endometrial stromal sarcomas infiltrate the myometrium in a more destructive manner and are associated with areas of hemorrhage and necrosis [7]. MRI reveals a voluminous polypoid mass in an expanded endometrial cavity, characterized by heterogeneous signal intensity on both T1- and T2-weighted images (Figs. 7 Initial uterine involvement occurs in 1% of patients with lymphoma. However, uterine involvement is more common as part of a generalized process, as shown in 40 50% of these patients at autopsy. Imaging findings of lymphoma involving the uterus are nonspecific [8] (Fig. 10). IV Leiomyomatosis IV leiomyomatosis can mimic endometrial stromal sarcoma because both conditions have the tendency to extend as a contiguous tumor into the adjacent vascular structures (Fig. 11). denomyosis denomyosis is a diffuse lesion involving the myometrium and is similar to endometrial stromal sarcoma. However, adenomyosis Fig year-old woman with uterine lymphoma., Contrast-enhanced CT scan shows diffuse enlargement of uterus and several small low-attenuation nodules., Contrast-enhanced CT scan obtained at level above shows multiple small, nodular low-density lesions in both kidneys, suggesting multinodular form of renal lymphoma. lthough imaging findings of lymphoma involving uterus are nonspecific, diffuse uterine enlargement, somewhat lobular contour, and relatively homogeneous attenuation with multiple lymphadenopathy, can suggest lymphoma involvement of uterus JR:181, November 2003

Downloaded from www.ajronline.org by 37.44.206.60 on 01/10/18 from IP address 37.44.206.60. Copyright RRS. For personal use only; all rights reserved CT and MRI of Uterine Sarcomas Fig. 11.

, xial T2-weighted image (TR/TE, 2000/80) shows lobulated high-signal-intensity mass in right side of uterine body and another mass with same features in right adnexa.

IV leiomyomatosis is characterized by growth of histologically benign smooth muscle initially into venous channels within broad ligament and then into uterine and iliac veins.

IV extension of endometrial stromal sarcoma must be considered in differential diagnosis. Fig. 12. 35-year-old woman with focal adenomyosis.

Multiple punctate foci of high signal intensity (arrowheads) that are thought to represent hemorrhagic endometrial nests are scattered throughout mass.

5 Downloaded from by on 01/10/18 from IP address Copyright RRS. For personal use only; all rights reserved CT and MRI of Uterine Sarcomas Fig year-old woman with IV leiomyomatosis., xial T2-weighted image (TR/TE, 2000/80) shows lobulated high-signal-intensity mass in right side of uterine body and another mass with same features in right adnexa., xial T2-weighted image (2,000/80) caudal to shows that parametrial mass extends out from uterine mass. Pathology confirmed diagnosis of IV leiomyomatosis. IV leiomyomatosis is characterized by growth of histologically benign smooth muscle initially into venous channels within broad ligament and then into uterine and iliac veins. This IV growth takes form of visible, wormlike projections extending out from a myomatous uterus into parametria toward pelvic sidewalls. IV extension of endometrial stromal sarcoma must be considered in differential diagnosis. Fig year-old woman with focal adenomyosis. Sagittal T2-weighted turbo spin-echo image (TR/TE, 3,900/99) shows ill-defined circumscribed area of low signal intensity in posterior wall of uterine body. Multiple punctate foci of high signal intensity (arrowheads) that are thought to represent hemorrhagic endometrial nests are scattered throughout mass. These foci were also seen on T1-weighted images (not shown). In contrast to adenomyosis, endometrial stromal sarcoma exhibits high signal intensity on T2-weighted images. Fig year-old woman who has intramural leiomyoma with myxoid and cellular degeneration., Sagittal T2-weighted turbo spin-echo image (TR/TE, 3,900/99) shows well-defined intramural mass (arrows) with high signal intensity peripherally and irregular isointensity of signal in myometrium centrally., Gadolinium-enhanced T1-weighted image shows poor enhancement of periphery of mass and homogeneous enhancement of central portion of mass (arrows). Pathologically, mass was identified as intramural leiomyoma, its central portion showed cellular degeneration, and its periphery showed myxoid degeneration. Degenerated myoma may mimic imaging findings of uterine sarcomas. JR:181, November

6 Rha et al. Downloaded from by on 01/10/18 from IP address Copyright RRS. For personal use only; all rights reserved exhibits ill-defined low signal intensity with multifocal punctate high-signal-intensity spots on T2-weighted images (Fig. 12). Myoma with Secondary Degeneration lthough nondegenerated myomas have a typical appearance at MRI, degenerated myoma may mimic the imaging findings of uterine sarcoma (Fig. 13). Conclusion The imaging findings of uterine sarcomas are large uterine masses with extensive hemorrhage and necrosis. However, the findings may be nonspecific, and the diagnosis is based on pathologic findings. Knowledge of the imaging spectrum and the clinical features of uterine sarcomas is important for radiologists because imaging plays a crucial role in staging and management. cknowledgment We thank onnie Hami, Department of Radiology, University Hospitals of Cleveland, Cleveland, OH, for her editorial assistance in the preparation of this manuscript. References 1. Lurain JR. Uterine cancer. In: erek JS, dashi EY, Hillard P, eds. Novak s gynecology. Philadelphia: Williams & Wilkins, 1996: Shapeero LG, Hricak H. Mixed müllerian sarcoma of the uterus: MR imaging findings. JR 1989;153: Smith T, Moy L, Runowicz C. Müllerian mixed tumors: CT characteristics with clinical and pathologic observations. JR 1997;169: Janus CJ, White M, Dottino P, rodman M, Goodman H. Uterine leiomyosarcoma: magnetic resonance imaging. Gynecol Oncol 1989;32: Pattani SJ, Kier R, Deal R, Luchansky E. MRI of uterine leiomyosarcoma. Magn Reson Imaging 1995;13: Koyama T, Togashi K, Konishi I, et al. MR imaging of endometrial stromal sarcoma: correlation with pathologic findings. JR 1999;173: Ueda M, Otsuka M, Hatakenaka M, et al. MR imaging findings of uterine endometrial stromal sarcoma: differentiation from endometrial carcinoma. Eur Radiol 2001;11: Glazer HS, Lee JKT, alfe DM, Mauro M, Griffith R, Sagel SS. Non-Hodgkin lymphoma: computed tomographic demonstration of unusual extranodal involvement. Radiology 1983;149: Jung NY, Rha SE, Jung SE, et al. CT and MR imaging findings of endometrial stromal sarcomas. J Korean Radiol Soc 2003;48:59 64 The RRS foundation, The Roentgen Fund, sponsors worthy educational, scientific, and research initiatives that contribute to excellence in radiology and medicine. gift to The Roentgen Fund means progress in radiology. For more information call JR:181, November 2003

Endometrial Stromal Sarcoma

May 26, 2011 By Sushila Ladumor, MD [1] Endometrial stromal sarcoma (ESS) is a rare malignant tumor of the endometrium, occurring in the age group of 40-50 years. History The 50-year-old, female patient