Received: 17 May 2001Revised: 7 September 2001Accepted: 30 November 2001Published online: 20 July 2002© Springer-Verlag 2002

Abstract Leiomyosarcomas are softtissue tumors that account for ap-proximately 15% of all soft tissuesarcomas. Leiomyosarcomas may belocated at almost any part of the ab-domen but especially are more com-mon in the retroperitoneum, fol-lowed by gastrointestinal tract andgenital system. They develop mainlyin adult life and are very rare in chil-dren. In this article, imaging findingsof leiomyosarcomas in various ab-dominal locations are presented. Ra-diologic studies are capable of pro-viding useful information on the lo-calization, size, changes in the inter-nal structure of the tumor, its exten-sion and invasion. Leiomyosarcoma

should be considered in the differen-tial diagnosis in case of detection ofa large, circumscribed, and heteroge-nous abdominal mass. Histopatho-logically, diagnosis of malignancydepends particularly on mitoticcounts, size, rate of necrosis, and in-filtrating margins.

Keywords Abdomen, neoplasms ·Leiomyosarcoma · CT · US · MR

Eur Radiol (2002) 12:2933–2942DOI 10.1007/s00330-002-1358-6 O N C O L O G Y

Sebuh KurugogluGunduz OgutIsmail MihmanliUgur KormanHaydar Durak

Abdominal leiomyosarcomas: radiologic appearances at various locations

Introduction

Leiomyosarcoma (LM) is a soft tissue sarcoma that isoriginated from smooth muscle cells. It accounts for ap-proximately 15% of all soft tissue sarcomas and is in-creasing in number. Leiomyosarcoma may occur in a va-riety of intra-abdominal locations and is more commonespecially in retroperitoneum, gastrointestinal (GI) tractand genital system when compared with other abdominalsites.

Leiomyosarcoma principally is a tumor of adult life.It affects patients in their fifth to seventh decades of lifewith a median age of 60 years [1]. Children rarely devel-op these tumors. Leiomyosarcoma is extremely rare inpediatric age group, comprising less than 2% of all softtissue sarcomas [2]. In general, it is more common inwomen than men (retroperitoneum, inferior vena cava);however, in some locations (i.e., skin, subcutis) the ratio

changes reversely. By the time LM is detected, it has al-ready reached to large sizes ranging from 5 to 35 cm [1].

Few predisposing or etiologic factors are recognizedin the development of LMs such as estrogen stimulation,immun-disordered conditions, and gene mutation(s) [3,4]. The role of radiation and leiomyomas undergoingmalignant degeneration are still controversial [1, 5]. Ma-lignant change in a pre-existing leiomyoma is difficult todemonstrate since benign smooth muscle can be indistin-guishable from well-differentiated areas of LM.

Histopathologically LM is composed of spindle cellswith eosinophilic cytoplasm having classic “cigar-shaped” blunt-ended nucleus [1]. Many tumors containlarge areas of hyalinization and necrosis. Hemorrhagecan also be found. Various stromal changes modify thepicture such as myxoid changes, fibrosis, and inflamma-tion. Some LMs, especially in the uterus and stomach,may partly or wholly be epitheloid; however, epitheloid

S. Kurugoglu (✉) · G. Ogut · I. MihmanliU. KormanDepartment of Radiology, Istanbul University, Cerrahpasa Medical Faculty, 34300 Istanbul, Turkeye-mail: sebuh.k@superonline.comTel.: +90-212-5861586Fax: +90-212-5861586

H. DurakDepartment of Pathology, Istanbul University, Cerrahpasa Medical Faculty, 34300 Istanbul, Turkey

tumors in nature does not imply malignancy. The malig-nant potential of these tumors are evaluated on the basisof their mitotic rate. The mitotic rate exceeding 5 in 10high-power field is malignant. A mitotic rate of 1–4 in10 high-power field is potentially malignant, especiallyif larger than 7.5 cm in diameter [1, 6, 7].

In general, the signs and symptoms vary with the lo-cation, size, infiltration of the adjacent organs, and thepresence of metastases. Abdominal mass, pain, intestinalobstruction, and weight loss are the most common symp-toms and signs. Although 5-year survival rate is variable,the overall prognosis is often poor. The mode of spreadof LM is principally local and/or hematogenous [8].Lymphatic metastasis has occasionally been reported inpoorly differentiated tumors or LMs of GI tract origin. Inthis article imaging findings of histopathologically prov-en LMs in different abdominal locations are presentedand the radiologic findings are discussed.

Gastrointestinal tract

Gastric LM comprises 0.5–3.5% of all primary malig-nancies and 17–20% of all smooth muscle tumors of thestomach [9, 10]. There is no anatomical dominance inthe gastric location of LM. The tumor can be located atthe fundus, body, or the antrum (Fig. 1). Leiomyosarco-ma represents 2–3% of small bowel and 0.1% of largebowel tumors [11]. Ileal LM is more common than thoseof other small bowel sites. Leiomyosarcoma arising fromMeckel’s divericulum is also reported [1]. As for thelarge bowel, rectum and rectosigmoid regions are theusual sites (Fig. 2). Recent studies have shown that LMs

of GI tract differ from their abdominal counterparts. Anew terminology, GI stromal tumors (GIST), has beenapplied, as GIST are distinguished histopathologicallyand immunochemically from morphologically similarLMs of the abdomen. Although the two pathologiesseem to be distinct, their clinical courses are similar andcomplete surgical resection is the cornerstone of treat-ment for both [12, 13, 14].

In GI tract, LM may show four growing patterns [15].Intraluminal (toward the lumen) and subserosal (extra-mural) extension of the tumor are the two most commongrowing patterns. Leiomyosarcoma also can grow bidi-rectionally (dumbbell) or intramurally. Unless the tumorleads to obstruction and/or upper or lower GI tract bleed-ing, the tumor is diagnosed at an advanced stage. Earlydiagnosis is rarely achieved. Various radiologic modali-ties can be used in the detection or diagnosis of GI tractLMs. On double-contrast barium examination, the tumorcan show obstruction, extrinsic mass configuration, fill-ing defect, ulceration, intussuseption, luminal narrowing,and displacement (Fig. 3). Tumors which are growing to-ward the lumen present themselves as well-defined sub-mucosal mass lesions. The overlying mucosa can eitherbe intact or damaged, showing small or shallow ulcer-ations. On fluoroscopy, rigidity may be observed. On theother hand, extramurally growing LM shows double-contour images and/or displacement of adjacent organs,and is frequently indistinguishable from extrinsic masslesions. Combined form shows a combination of bothfindings. Multicentric or multifocal GI leiomyosarcoma-tosis is also reported [16].

On ultrasonography (US), LM presents as a hypo-echoic mass that can be heterogenous in echogenicityand may show centrally cystic areas corresponding to ne-crosis [17]. Internal hemorrhage appears as either hypo-,iso-, or hyperechogenic areas according to its stage(Fig. 4). Cross-sectional imaging is often used to demon-strate the tumor extent and the relation with the sur-

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Fig. 1 Gastric leiomyosarcoma. On double-contrast examinationof the stomach, at the diaphragmatic side of the gastric fundus, awell-defined, broad-based mural mass, growing both intra- andextraluminally (dumbbell), is seen (arrow). The extraluminal com-ponent of the mass is undulating the overlying diaphragm

Fig. 2 Rectal leiomyosarcoma. On contrast-enhanced CT image, awell-defined rectal mass, measuring 3.5 cm in diameter, indentingthe posterior wall of the bladder, is seen. Pararectal fatty planesare homogenous and no lymph node enlargement is present

rounding structures. Moreover, evaluation of the mor-phologic characteristics, such as size, shape, uniformityin density or intensity, enhancement pattern, presence ofcalcification, possible coexisting lymphadenopathy,and/or metastases, may provide a clue for differentiatingmalignant tumors from benign masses. Generally, LMsare detected when they reach a considerable size and are

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Fig. 3a–d Ileal leiomyosarcoma. a On enteroclysis, luminal nar-rowing in a 5-cm distal ileal segment (between arrows) with irreg-ular mucosal pattern showing spicular ulcerations (arrowheads) isseen (spot view). The plica circularis are destroyed and the in-volved segment is separated from the adjacent ileal segments(mass effect). b On contrast-enhanced CT image, a mainly extra-luminal growing, lobulated-contoured 7.5-cm mass originatingfrom the wall of distal ileum is present. The mass contains a punc-tate calcification (arrowhead). Luminal contrast material hasbulged on the mural component of the mass (umbilical ulceration,arrow). c Macroscopic appearance of the resected specimen show-ing a heterogenous bulky mass with necrotic, hemorrhagic, andpseudocystic areas. d Photomicrograph of the resected specimendemonstrating spindle-shaped hypercellularity with mild pleomor-phism and mitosis. (Hematoxylin and eosin stain, ×400)

Fig. 4 Ileal leiomyosarcoma. A hypoechoic heterogenous mass ofintestinal origin growing extramurally is seen on the US image.Hyperechogenicity within the mass corresponds to internal hemor-rhage (arrows)

bulky at presentation. They usually show internal necro-sis or cystic degeneration. Although calcification is not acommon finding, the incidence appears to vary. Comput-ed tomography (CT) is the best radiologic modality todetect dystrophic tumoral calcification. On CT, LM isseen as a large, well-defined, heterogenous, hypodensemass with or without central necrotic or cystic degenera-tion (Figs. 3b, 5, 6) [10, 18, 19]. Contrast-enhanced CTimages may show either homogenous or heterogenous

contrast enhancement. On magnetic resonance imaging(MRI), LM appears hypointense on T1-weighted imagesand shows intermediate signal intensity on T2-weightedimages. After contrast administration, heterogenous en-hancement of the mass is seen. Central necrotic areasshow strong T2 hyperintensity [20].

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Fig. 5a, b Duodenal leiomyosarcoma. a On double-contrast bari-um examination, irregular narrowing of the descending segment ofthe duodenum is seen (arrowheads). The contrast material extr-avasates from the lateral wall of the involved segment (arrow) andfills an irregular and large cavity. b Corresponding contrast-en-hanced CT image shows a big soft tissue density mass located lat-erally to the liver, posteriorly to the right kidney, medially to thesuperior mesenteric artery and vein, and anteriorly to the trans-verse colon. The mass contains many irregular necrotic areasfilled with gas/fluid levels and orally given contrast material

Fig. 6 Jejunal leiomyosarcoma. Contrast-enhanced CT image de-picts a large and bulky mass originating from the distal jejunum. Themass is mainly extraluminal and containing an irregular and largecentral cavity (arrow), filled with contrast extravasation and air

Fig. 7 Retroperitoneal leiomyosarcoma. On contrast-enhanced CTimage, a bulky, lobulated-contoured retroperitoneal soft tissuedensity mass is seen (electronic cursor represents the density ofthe mass, 63 HU). Abdominal aorta and inferior vena cava cannotbe separated from the mass. Note the collateral vessels on the an-terior abdominal wall

Retroperitoneum

Retroperitoneal LM is the second most common primaryretroperitoneal tumor in adults (30%) after liposarcoma(61%) [21, 22]. Women have a higher frequency for ret-

roperitoneal and inferior vena cava (80%) LM (possiblerelationship with estrogen) [1]. Retroperitoneal LM canbe extravascular, intravascular, or may show a combina-tion of both. These tumors can be huge, reaching up to35 cm in size. The usual clinical presentation is of a largeabdominal mass. Commonly on cross-sectional imaging alarge, bulky tumor with partially necrotic tissue is seen(Fig. 7). Both CT and MRI show a nonfatty soft tissuemass with heterogenous contrast enhancement [21, 23].Border of the tumor is often indistinct from the adjacentstructures. Intraspinal extension is very rarely appreciat-ed. Ultrasound shows the consistency of the mass, solidor cystic, and gives no additional information. Inferiorvena cava is the major retroperitoneal vessel from which

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Fig. 8 Inferior vena cava leiomyosarcoma. On contrast-enhancedCT image, a well-defined mass originating from the middle-thirdsegment of inferior vena cava is seen (collateral vessels are domi-nant in the upper sections; not shown). The superior mesenteric ar-tery and vein are displaced anteriorly. The gallbladder is hydropicsecondary to pressure effect of the mass on extrahepatic bile duct.Duodenum is also compressed by the mass (the umbilication ofthe contrast material on the medial wall of the duodenum is sec-ondary to previously performed sphincterotomy)

Fig. 9 Renal leiomyosarcoma. Contrast-enhanced CT imageshowing a mass originated peripherally from the left renal capsule,extending anteriorly and filling the left half of the abdominal cavi-ty. The tumor is very large, reaching 12 cm in diameter, containinglarge areas of central necrosis

Fig. 10 Renal leiomyosarcoma. On T2-weighted MR image, an 8-cm mass replacing the left renal parenchyma is seen. The tumoris heterogenous in signal intensity, containing many small hyper-intense areas, and cannot be differentiated from hypernephroma

Fig. 11 Bladder leiomyosarcoma. On contrast-enhanced CT im-age, a bladder mass (M) originating from the left anterior wall andgrowing extramurally is seen

LM originates [24, 25]. The tumor can arise from the in-frarenal, middle third or upper suprahepatic segments, thelatter being more common (Fig. 8). Multiplanar imagingcapacity, inherent signal intensity differences of tissues,flow void, and flow enhancement techniques to assessvessel patency are the advantages of MRI over other ra-diologic modalities [21, 26]. Angiography and US mayalso be useful for the evaluation of vessel patency. As acomplication, venous occlusion can be a part of inferiorvena cava LM and collateral vascularisation develops.

Urinary tract

Primary sarcoma of the kidney is very rare accounting for1–3% of all renal malignancies [27, 28]. Leiomyosarcomais the most common histologic subtype of renal sarcomas,which can arise either from the renal parenchyma, renalpelvis, or renal artery, but is usually peripherally situated(Fig. 9) [1, 29]. Renal LMs give imaging findings charac-teristic of a malignant tumor but without histologic speci-ficity and cannot be differentiated from renal cell carcino-ma (Fig. 10). Radiologic findings are often non-specificand diagnosis is often established post-operatively. Cross-sectional imaging, however, is capable of providing infor-mation of the exact localization, morphology, and extentof the tumor [30]. On CT and MRI, LM appears as a well-defined mass of varying density and signal intensity, re-spectively. Detection of low signal intensity regions onT2-weighted MR images which also show delayed en-hancement is consistent with more fibrous tissue whichmay be a clue for the differentiation. Leiomyosarcomas ofthe bladder (Fig. 11) and ureter are very rare [31, 32].

Reproductive tract

As with other LMs of abdominal origin, genital LMs aregenerally large at presentation. Among women, corpusuteri (Fig. 12), and among men spermatic cord (Fig. 13),are common locations for genital LMs [1]. Pure ovarianLM is extremely rare and more common in post-meno-

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Fig. 12a, b Uterine leiomyosarcoma. a Axial and b sagittal T2-weighted MR images depict a very large lobulated-contouredmass, reaching 15 cm in diameter, filling the entire pelvis (be-tween arrows). The mass is very heterogenous containing hypoin-tense (fibrotic) and hyperintense (degenerative) areas. A smallamount of intraperitoneal, peritumoral effusion is present

Fig. 13 Spermatic cord leiomyosarcoma. Contrast-enhanced con-tinuous CT images show a centrally necrotic mass located at theleft inguinal canal (the mass was extending high into the pelvis).The non-necrotic peripheral tumor is strongly enhanced with thecontrast material

pausal women. Leiomyosarcoma is the least commonprimary neoplasm of the uterine cervix (Fig. 14), com-prising less than 1% of all cervical cancers with an inci-dence of 0.2–0.4% [33]. Like ovarian LM, it tends to oc-cur in the perimenopausal period and thereafter. Uterinemyomas, especially those with myxomatous changes, arereported to be prone to changing to LMs [34].

In men intratesticular LM is extremely rare. Leiomyo-sarcoma is more common in the spermatic cord in whichsmooth muscle is abundant [35, 36]. Ultrasound is mosthelpful in demonstrating the extratesticular origin of the

mass and evaluating scrotal extension. Both CT and MRIare necessary for pelvic staging and searching for distantmetastases [37]. Prostatic sarcoma comprises 0.2–0.5%of all prostatic malignancies, with LM representing 25%of sarcomas. When diagnosed, LM is always bulky(Fig. 15). Prostatic LM shows diffuse prostatic enlarge-ment of heterogenous signal intensity and contrast en-hancement on MRI [38].

Liver

The mode of spread of LM is principally local or hema-togenous, liver being the most common site [39, 40].Metastatic LM is much more common than primary he-patic LM (Figs. 16, 17); the latter may have associationwith hepatitis and cirrhosis [41, 42]. On MRI, it is seenas a well-delineated large mass, hypointense on T1- andmarkedly hyperintense on T2-weighted images, whichmay be either homogenous or non-homogenous, contain-ing necrotic or hemorrhagic areas [40, 41]. On contrast-enhanced sequences central necrotic areas do not en-hance.

Abdominal wall (Fig. 18), omentum (Fig. 19), andpancreas (Fig. 20) are exceptional locations for abdomi-nal LMs [43, 44]. Pancreatic head is the most commonlocalization; however, the whole organ can be occupiedwhen LM is detected. On cross-sectional imaging, pan-creatic LM shares the same characteristic features of oth-er abdominal LMs [45].

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Fig. 14a, b Uterine cervix leiomyosarcoma. On T2-weighted a sagittal and b axial MR images, a well-defined tumor of theuterine cervix (arrows) growing exophitically and filling the upperthird of the vagina is seen. The hypointense rim around the tumorcorresponds to the intact vaginal wall. Parametrium, rectum, andbladder are normal

Fig. 15 Prostatic leiomyosarcoma. On T2-weighted sagittal MRimage a bulky prostatic mass is present, which almost occupies theentire pelvis (between arrows), containing both solid (upper) andcystic (lower) components. The bladder is anterosuperiorly dis-placed by the mass

Although the radiologic appearances are not specificfor abdominal LMs, radiologic studies are capable ofproviding useful information for the localization, size,changes in the internal structure of the mass, extention,and invasion of the tumor which are beneficial to the dif-

ferential diagnosis and management. No matter wherethe abdominal location, in case of detection of a large,circumscribed, and heterogenous abdominal mass, LMshould be considered in the differential diagnosis.

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Fig. 16 Gastric leiomyosarco-ma with liver metastasis. Oncontrast-enhanced continuousCT images, a gastric mass (g)growing extramurally towardsthe left lobe of the liver is seen.A large and well-defined hepat-ic metastasis (m) is also presentin the posterior segment of theright lobe, showing the samefeatures as the gastric mass

Fig. 17 Metastatic hepatic leiomyosarcoma. On contrast-en-hanced CT, a large, 18-cm lobulated-contoured, non-homogenoushepatic mass, extending medially toward the pancreas, duodenum,and filling the lesser sac, even crossing the midline, is seen. Notethe compressed, slit-like inferior vena cava (arrow)

Fig. 18 Abdominal wall leiomyosarcoma. On contrast-enhancedCT image, a fusiform-shaped right anterior abdominal wall mass(m) originating from the right rectus muscle is seen

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Fig. 19 Omental leiomyosarcoma. On contrast-enhanced CT im-age a lobulated-contoured large mass of omental origin is present.The mass is invading the serosal surfaces of the adjacent small andlarge intestine (arrows)

Fig. 20 Pancreatic leiomyosarcoma. On contrast-enhanced CTimage, centrally necrotic mass originated from the pancreatic tailis present. The necrotic area does not enhance with contrast mate-rial. Note the superior mesenteric vein invasion by the mass (ar-rowhead)

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