This chapter should be cited as follows:
Ciccarone F, Dababou S, Glob Libr Women's Med
ISSN: 1756-2228; DOI 10.3843/GLOWM.419633

The Continuous Textbook of Women’s Medicine Series – Gynecology Module

Volume 10

Ultrasound in gynecology

Volume Editors: Professor Antonia Testa, Agostino Gemelli University Hospital, Rome, Italy
Professor Simona Maria Fragomeni, Agostino Gemelli University Hospital, Rome, Italy

Chapter

Malignant Myometrial Pathology Including STUMP and Sarcoma

First published: September 2025

AUTHOR(S)

Francesca Ciccarone, MD

Gynecologic Oncology Unit, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario “A. Gemelli” IRCCS, Rome, Italy

Susan Dababou, MD

Gynecologic Oncology Unit, Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario “A. Gemelli” IRCCS, Rome, Italy

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INTRODUCTION

Malignant myometrial pathology represents 3–7% of all uterine malignancies¹ and involves a group of rare uterine tumors arising from mesenchymal tissue. According to the latest World Health Organization (WHO) classification, they include uterine leiomyosarcoma (LMS), low- and high-grade endometrial stromal sarcoma (ESS), undifferentiated uterine sarcoma, adenosarcoma, epithelioid sarcoma, and other rare histotypes.²^,³ Smooth muscle tumors of uncertain malignant potential (STUMPs) are mesenchymal myometrial pathology that cannot be clearly classified as benign or malignant.²^,³ Despite their rarity, malignant myometrial tumors are clinically significant due to their aggressive behavior and diagnostic challenges. Their clinical presentation and imaging findings often mimic those of benign leiomyomas, complicating preoperative diagnosis. However, recognizing the potential for malignancy is essential to plan surgery, prevent tumor dissemination and influence prognosis.

This chapter provides a detailed analysis of the ultrasound features of STUMPs and uterine sarcomas. It explores both common and uncommon sonographic patterns and outlines key imaging features that may support the differentiation from benign myometrial lesions. A brief overview is also provided on epidemiology and clinical features, histopathological and molecular characterization, alternative imaging modalities, treatment and prognosis.

EPIDEMIOLOGY AND CLINICAL PRESENTATION

Among uterine sarcomas, LMS is the most common and aggressive subtype, arising from the smooth muscle of the myometrium and accounting for up to 60% of uterine sarcomas. ESSs are further divided into low-grade ESS, which tend to have an indolent course, and high-grade ESS, which behave more aggressively.⁴ Undifferentiated uterine sarcoma is another rare and highly malignant entity, which is characterized by poorly differentiated cells lacking specific stromal features.¹ Adenosarcomas, tumors composed of benign epithelial components and malignant stromal elements, occupy a distinct category within uterine sarcomas.¹ Finally, STUMPs represent a diagnostic gray-zone. These tumors exhibit atypical histopathological features that preclude classification as either benign leiomyomas or malignant leiomyosarcomas.² STUMPs are extremely rare and their incidence is not well established.⁵

Uterine sarcomas account for less than 10% of all uterine corpus cancers, with an incidence ranging from 1.55 to 1.95 per 100 000 women per year.⁶ Uterine LMS is thought to arise de novo in most cases, while malignant transformation from a pre-existing leiomyoma is considered a rare event.⁷ The prevalence of unexpected sarcoma in women undergoing hysterectomy or myomectomy for a myometrial lesion ranges between 1 in 2000⁸ and 1 in 350.⁹ Nevertheless, a correct identification of imaging features of malignancy is of utmost importance before surgery, to avoid procedures like morcellation that would lead to iatrogenic dissemination of occult malignancy. Managing uterine sarcomas remains challenging due to the difficulty in distinguishing benign from potentially malignant lesions preoperatively.¹⁰

Signs and symptoms of uterine malignant mesenchymal tumors may mimic benign leiomyomas such as heavy uterine bleeding, bulk symptoms, dysmenorrhea, infertility, pelvic pressure, abnormal vaginal discharge and intermenstrual bleeding.¹^,¹¹ Most uterine sarcomas are symptomatic at presentation¹²^,¹³ and occur in older women, with postmenopausal status being a significant risk factor, with an incidence four times higher than in younger women.⁷ Notably, a retrospective study found that ESS, more commonly occurs in younger, premenopausal women.¹³ The reported median ages of women at diagnosis are 50–56 years for LMS, 40–55 years for ESS, 55–60 years for undifferentiated uterine sarcoma (USS) and 41–48 years for STUMP.¹³^,¹⁴ Black race is associated with a twofold increased risk of LMS compared to white women, while no similar association has been observed for other uterine sarcoma subtypes.¹⁵ Previous pelvic radiation therapy is a risk factor,¹⁶^,¹⁷ along with obesity, nulliparity and excess endogenous estrogen, all of which are also associated with other uterine cancers.¹⁶^,¹⁷

HISTOPATHOLOGICAL AND MOLECULAR FEATURES

Histological diagnosis is often controversial and frequently requires expert reassessment at referral centers. According to the WHO classification, STUMPs are defined as tumors that cannot be histologically diagnosed as unequivocally benign or malignant.¹⁸^,¹⁹

The Stanford criteria of Bell et al.²⁰ are commonly used to classify LMS, including the frequency of mitotic figures (≥ 10 per 10 high-power fields [HPFs]), extent of nuclear atypia and presence of coagulative tumor cell necrosis.¹⁸^,¹⁹^,²⁰^,²¹^,²²

The histopathological criteria remain inconsistent, hence immunohistochemistry and advanced molecular tests to detect fusion transcripts or assess mutation status may help in the diagnosis and to identify possible therapeutic targets.²³^,²⁴

ULTRASOUND

Ultrasound remains the primary diagnostic tool for evaluating myometrial lesions, thanks to its cost-effectiveness, accessibility and reliable diagnostic performance, especially in expert centers.¹³^,²⁵ However, ultrasound has limited accuracy in characterizing smooth muscle uterine tumors, and no clinical or radiological criteria have demonstrated sufficient sensitivity and specificity to reliably distinguish malignant lesions, making preoperative diagnosis challenging. Findings on ultrasound examination should be reported using the terms, definitions and measurements provided by the Morphological Uterus Sonographic Assessment (MUSA) group.²⁶ The use of MUSA terminology helps reduce intra- and interobserver variability, facilitates the assessment of treatment effects (both medical and surgical), enables comparison across different imaging modalities and ensures reproducibility and reliability across research protocols.

Ultrasound features of malignant myometrial pathology (STUMP and sarcoma)

Uterine sarcomas and STUMP are not frequently encountered, and most data on their ultrasound features stem from retrospective studies. Only a few emerging prospective studies have begun to provide more refined and potentially reliable diagnostic criteria.²⁷^,²⁸

In 2019, an international group of experts conducted one of the first retrospective analyses of a relatively large cohort of histologically confirmed uterine sarcomas (n = 195; 116 LMS, 48 ESS and 31 USS), describing their most common ultrasound features.¹³ Malignant lesions appeared as large isolated solid masses (mean diameter, 91 mm) with inhomogeneous echogenicity of the solid tissue (77.4%), sometimes containing cystic areas (44.6%) (77% were irregular), mostly without fan-shaped shadowing or calcification (Figures 1 and 2). Although moderate-to-rich vascularization (color score 3–4) was common (67.9%), about one-third of sarcomas showed minimal or absent vascularization (color score 1–2), likely due to tumor necrosis (Figure 3). Notably, 20% were misclassified as benign, with ESS often showing normal endometrial appearance, regular margins and sparse vascularization, while USS displayed irregular margins, hemorrhagic cystic areas and absence of shadowing.¹³ One sarcoma was multilocular without solid components.¹³ The ‘cooked appearance’ of solid tissue, indicating necrosis, was observed in 21.7% of sarcoma cases¹³ but also appeared in leiomyomas and STUMPs, limiting its specificity.⁵ This sonographic feature refers to areas of inhomogeneous, hypoechoic solid tissue with absent internal vascularization, resembling the texture of cooked meat.

Transvaginal ultrasound images with Doppler evaluation of cases of STUMP. The images depict heterogeneous echotexture, irregular vascularization patterns and varying degrees of cystic and solid components.

Transvaginal ultrasound images with color Doppler of uterine sarcoma, demonstrating heterogeneous echotexture and irregular margins. The Doppler assessment reveals variable vascularization patterns, ranging from moderate (a–b) to extensive (c) intralesional blood flow. Images (b) and (c) show the 'cooked appearance' of solid tissue, a sonographic feature defined by the lack of structure of the solid component and the absence of acoustic shadowing.

Transvaginal (a) and transabdominal (b) ultrasound images of uterine sarcoma. The lesion exhibits heterogeneous echotexture, irregular margins and peripheral vascularity on color Doppler. While uterine sarcomas are often highly vascularized, cases with lower vascularization can pose challenges in the differential diagnosis from benign myometrial lesions, such as atypical leiomyomas.

The presence of central necrosis, defined as irregular anechoic areas within an otherwise solid lesion, has also been described as a sonographic feature of uterine sarcomas in a separate retrospective study.²⁹ Irregular intralesional cysts have been reported in the literature as a typical ultrasound feature of uterine sarcoma and are considered to reflect the end stage of central necrosis.¹³^,²⁹^,³⁰^,³¹ A retrospective study assessing the level of interobserver agreement in identifying ultrasound features of malignant myometrial lesions found that the agreement was moderate for most features of uterine sarcoma, including irregular tumor borders, non-uniform echogenicity, cystic areas, central necrosis, absence of calcifications and high intralesional vascularity.²⁹

A prospective single-center study by Ciccarone et al. evaluated, in 2268 women with myometrial lesions ≥ 3 cm, the accuracy of a clinical and ultrasound-based algorithm in predicting mesenchymal uterine malignancies, including STUMP.²⁷ Among the 52 malignancies identified (23 LMS, 17 STUMP, seven USS and five ESS), lesions with irregular margins (48.1%), non-uniform echostructure (78.8%), mixed echogenicity (44.2%), moderate-to-rich vascularization (color score 3–4 (57.7%)) and irregular cystic areas (51.9%) were significantly more frequent.²⁷ Moreover, malignant lesions were more often solitary (61.5%), lacked acoustic shadowing (50%) and appeared significantly larger (mean diameter, 107.1 mm, P <0.0001) compared to benign tumors.²⁷ These sonographic patterns, validated in a large prospective cohort study, represent a key step toward improving preoperative risk stratification in clinical practice.

Heterogeneous echostructure along with moderate-to-high vascularity are useful ultrasound features that have been associated extensively with uterine sarcoma.¹³^,²⁹^,³² Doppler ultrasound often reveals irregular vascular patterns with thin, scattered or dilated vessels, predominantly in marginal and central regions. LMS is also characterized by low resistance index (RI) (0.37 ± 0.03) and high peak systolic velocity (PSV) (71 cm/s vs 22.5 cm/s in leiomyomas).¹⁶^,³³

There are no specific ultrasound features of STUMPs, which are often described with imaging patterns similar to those of LMS. In a recent multicenter retrospective study (including 35 STUMPs, 50 LMS and 200 leiomyomas), STUMPs and LMS shared several features such as absence of normal myometrium, multilocular appearance, hyperechogenicity relative to the surrounding myometrium, and absence of posterior shadowing, echogenic areas and hyperechogenic rim.⁵

Interestingly, STUMPs were more strongly associated with FIGO Type 6–7, absence of internal shadows, and, in case of cystic areas, the presence of a smooth internal wall. A color score of 1 was more typical of leiomyomas, a color score of 2 was mainly reported in leiomyomas and STUMPs, while color score 4 was significantly associated with LMS. Combining both color score 3 and 4, it was found that both LMS and STUMPs had a high percentage of both circumferential and intralesional vascularization, compared to leiomyomas.⁵

A retrospective series of 20 STUMPs further described most lesions as well-defined (85%), with non-uniform echogenicity, isoechoic (60%) or mixed echogenicity (30%) and microcystic anechoic areas (70%). No shadowing and no calcifications were reported. No calcifications or shadowing were observed, and the vascularization was predominantly poor-to-moderate (69%), with both circumferential and intralesional blood flow patterns being common (90%)²⁸ (Figure 4).

Grayscale (a) and color Doppler (b) transvaginal ultrasound images of a STUMP showing a heterogeneous mass with irregular echotexture. (b) Doppler imaging highlights scattered vascularization, with a color score of 3, indicating moderate vascularity and a potentially atypical neovascularization pattern.

Differentiating leiomyoma variants from uterine sarcoma/STUMP

The differentiation between benign and malignant uterine mesenchymal tumors represents one of the most complex challenges in gynecologic imaging. This difficulty arises when encountering atypical variants of leiomyomas (e.g. mitotically active, cellular or bizarre leiomyomas), which, despite their benign nature, may exhibit ultrasound features typically associated with malignancy. Among these, the most concerning findings include cystic areas, rich vascularization, irregular or ill-defined margins and absence of acoustic shadowing³⁴ (Tables 1 and 2, Figure 5).

Sonographic features suspicious of uterine mesenchymal malignancy.

Feature	Typical suspicious appearance	Clinical relevance
Tumor size	> 8 cm	Frequent in LMS, STUMP and other sarcomas²⁷
Margins	Irregular, ill-defined	May suggest malignancy²⁷^,³⁸
Echogenicity	Heterogeneous, mixed, non-uniform	Suggests necrosis or atypical cellularity¹³^,²⁷
Cystic degeneration	Irregular cysts, hemorrhagic or microcystic areas	May reflect necrosis or atypical degeneration¹³^,²⁷
'Cooked' appearance	Inhomogeneous, hypoechoic with no internal vascularity	Correlates with necrosis¹³^,²⁷
Posterior shadowing	Absent	Seen in benign rather than malignant lesions¹³^,²⁷
Calcifications	Absent	Absence raises suspicion in large solid masses¹³
Vascularization (color score)	Moderate-to-high (color score 3–4)	Common in LMS and STUMP⁵^,¹³^,¹⁶^,²⁷^,³²
Vascular pattern	Irregular, intralesional, circumferential	Disorganized neovascularization⁵^,¹³^,¹⁶^,³²
Lesion distribution	Solitary mass	More typical of malignant than benign lesions¹³

Diagnostic ultrasound features of uterine mesenchymal malignancies: main evidence from the literature.

Author, year	Study design	Ultrasound features	Clinical features
Ciccarone, 2025²⁷	Prospective	Irregular margins; non-uniform echostructure; color score 4; cystic areas; absence of shadowing; single lesion; tumor diameter > 8 cm.	AUB; age > 45; symptomatic
Borella, 2024⁵	Multicenter retrospective	Absence of normal myometrium; multilocular structure; non-uniform echogenicity; irregular cystic areas; absence of shadowing; color score 3–4; intra- and circumferential vascularization.	Older/postmenopausal women; AUB; pelvic pressure
De Bruyn, 2024²⁹	Retrospective	Irregular tumor borders; non-uniform echogenicity; cystic areas; central necrosis; absence of calcifications and moderate-to-abundant intralesional vascularity.	AUB; postmenopausal women
Ludovisi, 2019¹³	Retrospective	Isolated large solid masses; inhomogeneous echogenicity; cystic areas (usually irregular); usually not manifesting shadowing or calcifications; moderately or well vascularized.	AUB; postmenopausal women.

AUB, abnormal uterine bleeding.

Sonographic features that raise suspicion for malignancy include: rich vascularization (a), presence of cystic areas (b), irregular or ill-defined margins (c) and absence of acoustic shadowing (d).

It is important to underline, however, that these features are not pathognomonic for malignancy. For example, high vascularization can also be present in benign tumors. In fact, a prospective study on 70 women with highly vascularized smooth muscle tumors demonstrated that the vast majority were benign (93%), with only 7% diagnosed as malignant.³² Therefore, vascularity alone is not sufficient to establish a diagnosis. Patient age, on the other hand, remains an important risk factor.¹³^,²⁷^,³² The same study³² showed that age and ultrasound features combined can help improve diagnostic performance. In particular, women over 40 years with highly vascularized lesions, irregular margins and no visible endometrium are at higher risk of malignancy.³² Moreover, a large retrospective study involving 2075 patients undergoing myomectomy found that the probability of unexpected uterine sarcoma varies substantially with age, ranging from approximately 1 in 100 in women aged 75–79 years, to less than 1 in 500 in those under 30 years of age.³⁵

Another key point to consider is that sarcomas and STUMPs are not always highly vascularized. Some may present with minimal or absent blood flow on Doppler, emphasizing the heterogeneity of these tumors and the limits of relying on vascularity alone.¹³

Finally, the traditional concern regarding rapid tumor growth as a sign of malignancy has been reconsidered. Recent studies have shown that growth patterns in leiomyomas are highly variable between individuals. A retrospective analysis found no reliable predictors of which tumors would grow or regress over time, and concluded that growth alone, whether fast or slow, should not be used as a sole indicator of malignancy.³⁶

Ultrasound diagnostic algorithm

A recent prospective study stratified 2268 patients into different risk classes using a traffic light algorithm including sonographic and clinical variables. Among 52 malignancies (23 LMS, 17 STUMPs, seven USS and five ESS), LMS patients were typically postmenopausal and symptomatic, with abnormal uterine bleeding (55.8%) being the most common symptom.²⁷ Key ultrasound findings included ill-defined borders, heterogeneous echogenicity, moderate-to-rich vascularization, a ‘cooked’ appearance, irregular cystic areas and solitary lesions. Multivariable analysis confirmed that age, tumor diameter > 8 cm, irregular margins and color score 4 were independent risk factors for malignancy, while the presence of acoustic shadowing acted as a protective factor.²⁷ Based on these five key parameters, a predictive traffic light algorithm was developed, which provides a score that allows stratification of patients into different risk classes (Figure 6). This predictive model demonstrated a sensitivity of 98.1%, specificity of 58.3% and area under the receiver-operating-characteristics curve (AUC) of 0.87, reinforcing its diagnostic value. Compared to other scoring systems, this approach emphasizes a dynamic follow-up strategy based on risk classes.²⁷ By prospectively validating this approach in a large, real-world population, the study represents a key contribution toward safer and more personalized decision-making in the evaluation of myometrial lesions.

Traffic light algorithm for risk stratification of myometrial lesions. The traffic light system integrates one clinical (age) and four sonographic parameters (lesion diameter >8 cm, irregular borders, color score 4 and absence of acoustic shadowing) into a predictive model for mesenchymal uterine malignancies. Based on the calculated probability of malignancy, patients are stratified into: low-risk class (green): predicted risk <0.39%; intermediate-risk class (yellow): predicted risk 0.40–2.2%; or high-risk class (red): predicted risk ≥2.3%. This stratification supports personalized decision-making, ranging from conservative follow-up to immediate surgical management.

Radiomics

Advances in radiomics and machine learning are further enhancing diagnostic precision. In the ADMIRAL pilot study, analysis of ultrasound radiomic features in 70 patients (20 sarcomas, 50 leiomyomas) achieved 85% accuracy, 80% sensitivity, 87% specificity and an AUC of 0.86.³⁷ While still at an early stage, these technologies offer the potential to quantify objectively sonographic features that are currently assessed subjectively, enhancing reproducibility and interobserver agreement.

DIFFERENTIAL DIAGNOSIS

The distinction between benign uterine tumors and sarcomas remains a diagnostic challenge, hence other imaging techniques, serum markers and preoperative biopsy may help in the differential diagnosis in selected cases.

Magnetic resonance imaging

Magnetic resonance imaging (MRI) may help in the preoperative evaluation of uterine masses, when ultrasound findings are inconclusive. MRI offers higher accuracy in soft-tissue contrast, a larger field of view, diffusion imaging and multiplanar capabilities.¹⁵ A recent consensus statement has provided a standardized approach to MRI assessment, emphasizing its role in distinguishing uterine sarcomas/STUMP from benign leiomyomas.¹⁵ Features suggestive of malignancy include irregular margins; heterogeneous and high signal on T2 weighted imaging; and hemorrhagic and necrotic changes, with central non-enhancement, hyperintensity on diffusion-weighted imaging (DWI), and low values for apparent diffusion coefficient (ADC)¹⁵^,²⁵^,³⁹^,⁴⁰^,⁴¹ (Figure 7). Diagnostic algorithms have been proposed to support MRI interpretation in differentiating between benign and malignant uterine lesions, and recent studies have explored the use of artificial intelligence to further enhance diagnostic accuracy.⁴²^,⁴³^,⁴⁴ Emerging technologies, including radiomics and machine learning, show promise in improving diagnostic precision, although standardization and external validation remain essential.³⁹^,⁴⁵

Magnetic resonance imaging (MRI) of a STUMP. (a) Contrast-enhanced MRI showing heterogeneous enhancement of the mass. (b) T2-weighted MRI demonstrating a hyperintense lesion with heterogeneous signal intensity. (c) Diffusion-weighted imaging (DWI) highlighting areas of restricted diffusion. (d) Apparent diffusion coefficient (ADC) map, showing low ADC values suggestive of increased cellularity, aiding in the differentiation of STUMP from benign leiomyomas and malignant leiomyosarcomas.

Lactate dehydrogenase

The preoperative imaging assessment when combined with clinical findings and serum lactate dehydrogenase (LDH) levels, could help to differentiate between leiomyosarcomas and degenerated uterine leiomyomas.⁴⁶ While total LDH levels may increase in both benign and malignant uterine masses, specific isoenzymes, especially LDH3, LDH4 and LDH5, tend to be elevated in sarcomas, whereas LDH1 and LDH2 are typically reduced.⁴⁷ A recent large retrospective study proposed a simple and effective risk model based on the combination of LDH3 and LDH1 isoenzymes, known as the uterine mass Magna Graecia (UMG) index.⁴⁷ When applied to a cohort of 2254 patients with benign fibroids and 43 with confirmed sarcoma, the UMG index achieved 100% sensitivity and 99.6% specificity, with nine false positives among the 2211 benign cases and no false negative amongst 43 sarcomas. Its ability to distinguish between benign and malignant uterine masses suggests that, once prospectively validated, the UMG index could become a valuable tool in clinical practice, particularly useful when imaging is inconclusive.

Biopsy

In selected patients, biopsy can represent an additional tool to support differential diagnosis. However, the interpretation of biopsy specimens is not always straightforward. A retrospective French study involving centralized histopathological review reported a 14% rate of diagnostic discordance in patients initially diagnosed with sarcoma, mostly involving benign tumors mistaken for malignant, or vice versa. These data highlight the importance of expert pathology review and the potential consequences of diagnostic errors on patient management.⁴⁸

Recent advances have improved the reliability of biopsy in this setting. ESGO/EURACAN/GCIG guidelines recommend that biopsy be utilized in specific scenarios, such as advanced-stage disease or cases in which hysterectomy is not immediately feasible.²⁴ In such cases, image-guided core-needle biopsy (≥14–16G), performed in expert centers with access to advanced pathology, may provide useful preoperative information. Patients should always be informed of the possibility of false-negative results, particularly in the case of low-grade tumors, the histologic interpretation of which is inherently difficult.²⁴^,⁴⁹

TREATMENT AND PROGNOSIS

The prognosis for patients diagnosed with uterine sarcoma is poor,⁵⁰ with a 5-year survival rate of less than 50%.⁵¹ Conversely, most patients with STUMP experience favorable outcomes, although, in approximately 10% of cases, the lesion exhibits aggressive behavior.¹⁴

The optimal management of STUMP and uterine sarcoma varies widely due to differences in biological behavior and limited high-quality evidence. For STUMP, total hysterectomy with or without bilateral salpingo-oophorectomy is generally recommended in women who do not wish to retain their fertility, while myomectomy may be considered in younger patients seeking fertility preservation.¹⁴^,²⁰^,⁵²^,⁵³ However, certain surgical techniques, such as unprotected morcellation (without the use of a containment bag), have been significantly associated with increased risk of recurrence (relative risk = 2.94; P = 0.001) and are considered independent predictors of recurrence.⁵⁴ Despite these findings, the available literature shows no definitive survival benefit associated with more radical surgery.¹⁴^,²⁰^,⁵²^,⁵³ Postoperative management of STUMP remains inconsistent, with no established consensus on surveillance protocols. Reported recurrence rates range from 0% to 36%, with a mean of approximately 13%. In a large retrospective multicenter study including 87 patients, 18 cases (20.7%) recurred, 11 as LMS and seven as STUMP, with a mean time to recurrence of 79 months.⁵² Risk factors for recurrence and shorter recurrence-free survival included fragmentation or morcellation during surgery, epithelioid features, high mitotic count, Ki-67 ≥ 20%, progesterone receptor < 83% and diffuse p16 expression.⁵²

In contrast, the standard treatment for early-stage uterine sarcomas is en-bloc total hysterectomy with bilateral salpingo-oophorectomy.¹⁰^,⁵⁵^,⁵⁶ Lymphadenectomy is not recommended unless macroscopic lymph node involvement is present.⁵⁵ In premenopausal women with LMS, ovarian-sparing surgery may be considered, as evidence suggests that bilateral salpingo-oophorectomy does not improve survival.⁵⁶ For early-stage LMS, adjuvant chemotherapy lacks strong supporting evidence;⁵⁷^,⁵⁸ however, regimens including gemcitabine, docetaxel and doxorubicin may be considered for selected patients. In contrast, patients with low-grade (LG)-ESS may benefit from adjuvant hormonal therapy due to ER/PR receptor positivity, while data on high-grade (HG)-ESS and high-grade undifferentiated sarcoma (HG-USS) remain insufficient.⁴ Adjuvant radiotherapy, including external beam radiotherapy (EBRT) or intensity-modulated radiotherapy (IMRT), may reduce pelvic relapse rates, particularly in LMS, but has no proven impact on overall survival.¹⁰

For advanced or recurrent sarcomas (LMS, HG-ESS, HG-USS, adenosarcomas), surgical cytoreduction is crucial, especially following a favorable response to chemotherapy. Prognosis varies by histological subtype: LMS are typically high-grade tumors with poor prognosis even when confined to the uterus, while LG-ESS has a favorable prognosis with potential for long-term survival. HG-ESS and HG-USS behave more aggressively, particularly in the absence of nuclear uniformity. Adenosarcoma is generally associated with a good prognosis unless there is myometrial invasion or sarcomatous overgrowth.¹ Follow-up protocols depend on recurrence risk and tumor grade. For low-grade sarcomas, monitoring every 4–6 months for the first 3–5 years, followed by annual visits, is recommended, while high-grade sarcomas require closer surveillance: every 3–4 months for the first 2–3 years, every 6 months for the following 2–3 years and then annually.¹

CONCLUSION

Uterine sarcomas and STUMP lesions, although rare, are of substantial clinical importance due to their potential aggressive behavior.

The preoperative differentiation between benign and malignant myometrial lesions remains a diagnostic challenge, in particular because of overlapping ultrasound features with variants of leiomyomas. Ultrasound remains the first-line diagnostic tool. Ultrasound features including irregular margins, heterogeneous echogenicity, absence of acoustic shadows and rich vascularization can raise suspicion, especially in symptomatic patients over 45 years of age. Predictive models based on algorithms, such as the traffic light algorithm, as well as emerging techniques, including radiomics and machine learning, are progressively improving diagnostic accuracy and risk stratification.

PRACTICE RECOMMENDATIONS

Ultrasound remains the first-line diagnostic tool for assessing myometrial lesions due to its cost-effectiveness, accessibility and reliable diagnostic yield, although there are no established clinical or radiological criteria.
The MUSA terminology is recommended for the description of myometrial findings on ultrasound examination. Using standardized terminology facilitates a uniform diagnostic approach and reduces intra- and interobserver variability.
Differentiating between benign and malignant myometrial lesions can be challenging in the presence of atypical variants of leiomyoma (e.g. cellular, bizarre or mitotically active leiomyomas), which, despite their benign nature, may display ultrasound features commonly associated with malignancy.
Typical ultrasound features of malignant mesenchymal tumors include irregular margins, heterogeneous echogenicity, absence of acoustic shadowing, rich vascularization and irregular cystic areas.
The traffic light algorithm includes five key parameters (age, tumor diameter, margin regularity, color score and presence/absence of acoustic shadows) to stratify the patients into three risk classes (low, intermediate or high).
Radiomics and machine learning algorithms applied to ultrasound and MRI are emerging tools for improved diagnostic precision and risk stratification.
Diagnosis of malignant myometrial pathology requires a multidisciplinary approach of experts combining histological evaluation, immunohistochemistry and molecular profiling in referral centers.
The prognosis for patients diagnosed with uterine sarcomas is poor. Conversely, most patients with STUMP experience favorable outcomes, although lesions may exhibit aggressive behavior.

CONFLICTS OF INTEREST

The author(s) of this chapter declare that they have no interests that conflict with the contents of the chapter.

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