To evaluate the feasibility of magnetic resonance imaging in predicting unusual site ectopic pregnancy: a retrospective cohort study.

OBJECTIVE: To evaluate the accuracy of pelvic MRI in the diagnosis of unusual ectopic pregnancy (EP), when ultrasound (US) examination is inconclusive. METHODS: We retrospectively reviewed the medical records of 150 patients with suspected EP. Clinical, US and MRI features of 15 unusual EPs were analysed. Two radiologists independently reviewed each case resolving by consensus any diagnostic discrepancy. Interobserver agreement was assessed using the Cohen κ test. RESULTS: MRI displayed a gestational sac-like structure surrounded by a thick wall in all cases. The thick wall displayed hyperintensity in 41 %, isointensity in 35 % and hypointensity in 24 % of cases on T1-weighted images. Diffusion- and fat saturation T1-weighted images were the most accurate sequences, as they enabled identification of 15/15 and 14/15 patients, respectively. Although US was false negative in detecting cervical and uterine infiltration underlying the caesarean scar, MRI was able to identify the invasion. Interobserver agreement was very good for all sequences (κ=0.892-1.0). CONCLUSIONS: MRI plays an important role in the early diagnosis of unusual EP. It should be considered after negative US findings, providing accurate evaluation of the site and the possible infiltration of these lesions, which help in the management of these patients. KEY POINTS: • MRI is being increasingly used as a problem-solving modality in ectopic pregnancy. • MRI plays an important role in early diagnosis of unusual ectopic pregnancy. • Knowledge of MRI features in EP is essential to determinate appropriate management.

Are Second Trimester Apparent Diffusion Coefficient Values of the Short Uterine Cervix Associated with Impending Preterm Delivery?

Purpose To prospectively determine whether the apparent diffusion coefficient (ADC) of the cervix is associated with preterm delivery in asymptomatic patients with a sonographic cervical length of 15 mm or less and positive fetal fibronectin test results between 23 and 28 weeks of gestation. Materials and Methods The institutional review board approved this prospective hypotheses-generating study. A total of 30 pregnant women (mean gestational age, 26 weeks) with a sonographic short cervix (≤15 mm) underwent pelvic 1.5-T magnetic resonance (MR) imaging. Oblique sagittal diffusion-weighted images were obtained with b values of 0, 400, and 800 sec/mm(2). ADC values at MR imaging of the subglandular and stromal cervix and the difference between both were correlated to the interval to delivery. Receiver operating characteristic curve analysis was performed to obtain sensitivity and specificity of ADC values in association with delivery within 7 days. Results Eight (27%) of 30 patients delivered within 6 or 7 days after MR imaging (impending delivery group), and 22 (73%) of 30 patients delivered between 18 and 89 days after imaging (mean, 55 days) (late delivery group). Mean subglandular ADC and mean ADC difference were higher (P < .001) in patients with impending delivery than in those with late delivery ([2406.3 ± 166.0] × 10(-6) mm(2)/sec vs [1708.9 ± 108.1] × 10(-6) mm(2)/sec and [657.3 ± 129.9] × 10(-6) mm(2)/sec vs [69.2 ± 70.2] × 10(-6) mm(2)/sec, respectively). Subglandular ADC inversely correlated with the interval between MR imaging and delivery (r = -0.75). Receiver operating characteristic curve analysis of subglandular ADC revealed 100% sensitivity (95% confidence interval: 63.1, 100) and 100% specificity (95% confidence interval: 84.6, 100) in association with impending delivery with a 1921 × 10(-6) mm(2)/sec threshold. Stromal ADC and sonographic cervical length showed no difference between groups (P = .072 and P = .511, respectively). Conclusion Cervical subglandular ADC at MR imaging is associated with impending preterm birth in patients with a short sonographic cervix. (©) RSNA, 2016.

Diagnosis of Small-Bowel Diseases: Prospective Comparison of Multi-Detector Row CT Enterography with MR Enterography.

PURPOSE: To prospectively compare the accuracies of computed tomographic (CT) enterography and magnetic resonance (MR) enterography for the detection and characterization of small-bowel diseases. MATERIALS AND METHODS: The institutional review board approved the study protocol, and informed consent was obtained from all participants. From June 2009 to July 2013, 150 consecutive patients (81 men and 69 women; mean age, 38.8 years; range, 18-74 years), who were suspected of having a small-bowel disease on the basis of clinical findings and whose previous upper and lower gastrointestinal endoscopy findings were normal, underwent CT and MR enterography. Two independent readers reviewed CT and MR enterographic images for the presence of small-bowel diseases, for differentiating between inflammatory and noninflammatory diseases, and for extraenteric complications. The histopathologic findings of surgical (n = 23) and endoscopic (n = 32) biopsy specimens were used as the reference standard; the results of video-capsule endoscopy (n = 36) and clinical follow-up (n = 59) were used only to confirm the absence of small-bowel disease. RESULTS: MR and CT enterography were successfully performed in all 150 patients. Overall sensitivity, specificity, and accuracy, respectively, in identifying patients with small-bowel lesions were 75.9% (41 of 54), 94.8% (91 of 96), and 88.0% (132 of 150) for CT enterography and 92.6% (50 of 54), 99.0% (95 of 96), and 96.7% (145 of 150) for MR enterography. The sensitivity of MR enterography was significantly higher than that of CT enterography for the detection of both overall small-bowel diseases (P = .0159) and neoplastic diseases (P = .0412) but not for the detection of inflammatory diseases (P > .99) or noninflammatory and nonneoplastic diseases (P = .6171). CONCLUSION: MR enterography is more accurate than CT enterography in the detection of small-bowel diseases; MR enterography was more accurate in detecting neoplastic diseases in particular.

Magnetic resonance enterography, small-intestine contrast US, and capsule endoscopy to evaluate the small bowel in pediatric Crohn's disease: a prospective, blinded, comparison study.

BACKGROUND: Small-bowel (SB) disease is a severe clinical entity among the phenotypes of Crohn's disease (CD). OBJECTIVE: To assess sensitivity, specificity, and accuracy of magnetic resonance enterography (MRE), small-intestine contrast US (SICUS), and capsule endoscopy (CE) in the diagnosis of pediatric SB-CD. DESIGN: Prospective, blinded, comparison study. SETTING: Tertiary center for pediatric inflammatory bowel disease. PATIENTS: Children with known or suspected CD. Diagnosis of SB obstruction at SICUS or MRE excluded patients from the study. INTERVENTION: Patients underwent ileocolonoscopy, MRE, SICUS, and CE over a 7-day period. For the imaging evaluation, SB was divided into 3 segments: jejunum, proximal and mid ileum, and terminal ileum. MAIN OUTCOME MEASUREMENTS: The performance of each method was compared to a consensus reference standard for upper SB and to ileocolonoscopy for the terminal ileum. RESULTS: Twenty-five patients completed the study. In the jejunum, the sensitivity of SICUS and CE was 92%, which was not significantly higher than MRE (75%); the specificity of CE (61%) was significantly lower than that of MRE (P = .04). In the proximal and mid ileum, MRE and CE did not have significantly higher sensitivity (100%) than SICUS (80%), but CE was less specific (P > .05). At the terminal ileum, SICUS and MRE were slightly more sensitive than CE (94% vs. 81%); however, the latter was more specific. LIMITATIONS: Use of the consensus reference standard for upper SB. Small number of patients. CONCLUSION: SICUS, MRE, and CE are all effective options for imaging SB. An integrated use of different tools should be suggested to achieve a complete assessment of the SB in children with suspected or confirmed CD.

Errors in imaging the pregnant patient with acute abdomen.

Pregnant women with an acute abdomen present a critical issue due to the necessity for an immediate diagnosis and treatment; in fact, a diagnostic delay could worsen the outcome for both the mother and the fetus. There is evidence that emergencies during pregnancy are subject to mismanagement; however, the percentage of errors in the diagnosis of emergencies in pregnancy has not been studied in depth. The purpose of this article is to review the most common imaging error emergencies. The topics covered are divided into gynecological and non-gynecological entities and, for each pathology, possible errors have been dealt with in the diagnostic pathway, the possible technical errors in the exam execution, and finally the possible errors in the interpretation of the images. These last two entities are often connected owing to a substandard examination, which can cause errors in the interpretation. Consequently, the systemization of errors reduces the possibility of reoccurrences in the future by providing a valid approach in helping to learn from these errors.

The key role of the radiologist in the management of polytrauma patients: indications for MDCT imaging in emergency radiology.

Trauma causes greater losses of life years and it is the most common cause of death for people under the age of 45. Time is one of the most relevant factors for the survival of injured patients, particularly the time elapsed from trauma until the resuscitation procedures. As a member of the trauma team, the radiologist contributes to the rapid diagnosis of traumatic disorders, with appropriate imaging modalities. Based on the evidence, the most appropriate diagnostic tool for severe/multiple trauma is computed tomography (CT). With the advent of multidetector CT (MDCT), radiologists are able to more effectively characterize life-threatening traumatic disorders within a few seconds in stable or stabilized patients. Considering the diagnostic potential of MDCT, conventional radiographs could be virtually abandoned in the diagnostic algorithms for adult polytraumatized patients. The radiologist helps to facilitate triage and to assess the optimal individual treatment for polytrauma patients, thus contributing to the improvement of patient outcomes. In this article, the indications for MDCT in the polytrauma setting are discussed.

Olfactory deficit and hippocampal volume loss for early diagnosis of Alzheimer disease: a pilot study.

BACKGROUND: The aim of this pilot study was to verify the role of olfactory test and volumetric magnetic resonance imaging measure of hippocampus to predict conversion from mild cognitive impairment to Alzheimer disease (AD). MATERIALS AND METHODS: Eighteen patients with amnesic mild cognitive impairment, broadly defined, were followed at 12 months. Hypothesized baseline predictors for follow-up conversion to AD were olfactory deficit and hippocampal volumes loss. RESULTS: In the 1-year follow-up, 5 patients converted to AD. The 2 clinical predictors olfactory test and hippocampal volume loss showed the same sensitivity of 92.3% but the olfactory test showed a higher specificity than the hippocampal volume loss (75% vs. 60%). CONCLUSIONS: Our findings suggest the potential utility of olfactory test and hippocampal volume loss for early detection of AD.

3T diffusion tensor imaging and electroneurography of peripheral nerve: a morphofunctional analysis in carpal tunnel syndrome.

OBJECTIVE: The aim of the study was to assess the diagnostic potential of diffusion tensor imaging (DTI) for pathologies of the peripheral nervous system (PNS) through clinical, electrophysiological and morphological evaluation of the median nerve. METHODS: The present work was a multilevel prospective study involving 30 subjects, 15 of whom had carpal tunnel syndrome (CTS) and 15 healthy controls. All subjects underwent clinical evaluation through administration of the Boston Carpal Tunnel Questionnaire (BCTQ), electroneurography (ENG), 3-Tesla magnetic resonance imaging with DTI, and calculation of fractional anisotropy (FA) and the apparent diffusion coefficient (ADC) at the flexor retinaculum. Tractography was also performed for three-dimensional reconstruction of the route of the median nerve through the carpal tunnel. The degree of functional impairment was compared with the anatomical damage to the median nerve according to ENG and DTI. RESULTS: FA and ADC were significantly correlated with ENG parameters of CTS and BCTQ data. Mean FA and ADC values in the CTS patients were 0.359±0.06 and 1.866±0.050×10(-3)mm(2)/s, respectively, vs 0.59±0.014 and 1.395±0.035×10(-3)mm(2)/s, respectively, in the controls. FA was decreased and ADC increased in patients with CTS compared with healthy controls (P<0.05). CONCLUSION: DTI parameters were clearly confirmed by both clinical and ENG data and, therefore, may be used for the diagnosis of CTS.

The alterations of the sigmoid-rectal junction in diverticular disease of the colon revealed by MR-defecography.

PURPOSE: The sigmoidorectal junction (SRJ) has been defined as an anatomical sphincter with particular physiological behavior that regulates sigmoid and rectum evacuation. Its function in clinical conditions, such as diverticular disease has been advocated. The aim of our study is to identify the SRJ and to compare the morphometric and dynamic features of the SRJ between patients with diverticular disease and healthy subjects using MR-defecography. METHODS: Sixteen individuals, eight with uncomplicated diverticular disease and eight healthy subjects, were studied using MR-defecography to identify the SRJ and to compare the morphometric and dynamic features observed. RESULTS: In each subject studied, MR-defecography was able to identify the SRJ. This resulted in the identification of a discrete anatomical entity with a mean length of 31.23 mm, located in front of the first sacral vertebra (S1) and at a mean distance of 15.55 cm from the anal verge, with a mean wall thickness of 4.45 mm, significantly different from the sigmoid and rectal parietal thickness. The SRJ wall was significantly thicker in patients with diverticular disease than the controls (P = 0.005), showing a unique shape and behavior in dynamic sequences. CONCLUSION: Our findings support the hypothesis that SRJ plays a critical role in patients with symptomatic diverticular disease; further investigation may clarify whether specific SRJ analysis, such as MR-defecography, would predict inflammatory complications of this diffuse and heterogenic disease.

68Ga-PSMA PET/CT in Recurrent Prostate Cancer after Radical Prostatectomy Using PSMA-RADS Version 2.0.

BACKGROUND: 68Ga-PSMA PET/CT is superior to standard-of-care imaging for detecting regional and distant metastatic recurrent prostate cancer. The objective of our study was to evaluate the performance of 68Ga-PSMAPET/CT in our patient population, using the new PSMA-RADS version 2.0. METHODS: A total of 128 patients scanned with 68Ga-PSMA PET/CT for detection of recurrence after RP were analyzed with PSMA-RADS version 2.0. For the analysis of the detection rate, categories PSMA-RADS 3 to 5 were considered as "positive for malignancy" and 1-2 as "negative". RESULTS: According to PSMA-RADS v2.0, we classified patients as follows: 23 patients without PSMA-RADS because they were negative; PSMA-RADS 1: 10 patients; PSMA-RADS 2: 4 patients; PSMA-RADS 3A: 11 patients; PSMA-RADS 3B: 2 patients; PSMA-RADS 3C: 2 patients; PSMA-RADS 3D: 2 patients; PSMA-RADS 4: 13 patients; PSMA-RADS 5: 61 patients. CONCLUSIONS: The overall detection rate of 68Ga-PSMA PET/CT was 71%. By dividing the patients into fourgroups according to PSA level before examination, we obtained the following detection rates: PSA < 0.2 ng/mL 38%; 0.2 ≤ PSA < 0.5 ng/mL 57%; 0.5 ≤ PSA ≤ 1 ng/mL 77%; and PSA > 1 ng/mL 95%. CONCLUSION: Using PSMA-RADS version 2.0, we obtained detection rate values comparable with recent literature both in absolute terms and in relation to different PSA levels.

The role of cardiac magnetic resonance in sports cardiology: results from a large cohort of athletes.

BACKGROUND: Cardiac magnetic resonance (CMR) provides information on morpho-functional abnormalities and myocardial tissue characterisation. Appropriate indications for CMR in athletes are uncertain. OBJECTIVE: To analyse the CMR performed at our Institute to evaluate variables associated with pathologic findings in a large cohort of athletes presenting with different clinical conditions. METHODS: All the CMR performed at our Institute in athletes aged > 14 years were recruited. CMR indications were investigated. CMR was categorised as "positive" or "negative" based on the presence of morphological and/or functional abnormalities and/or the presence of late gadolinium enhancement (excluding the right ventricular insertion point), fat infiltration, or oedema. Variables associated with "positive" CMR were explored. RESULTS: A total of 503 CMR were included in the analysis. "Negative" and "positive" CMR were 61% and 39%, respectively. Uncommon ventricular arrhythmias (VAs) were the most frequent indications for CMR, but the proportion of positive results was low (37%), and only polymorphic ventricular patterns were associated with positive CMR (p = 0.006). T-wave inversion at 12-lead ECG, particularly on lateral and inferolateral leads, was associated with positive CMR in 34% of athletes (p = 0.05). Echocardiography abnormalities resulted in a large proportion (58%) of positive CMR, mostly cardiomyopathies. CONCLUSION: CMR is more efficient in identifying a pathologic cardiac substrate in athletes in case of VAs (i.e., polymorphic beats), abnormal ECG repolarisation (negative T-waves in inferolateral leads), and borderline echocardiographic findings (LV hypertrophy, mildly depressed LV function). On the other hand, CMR is associated with a large proportion of negative results. Therefore, a careful clinical selection is needed to indicate CMR in athletes appropriately.

MR imaging of the placenta: what a radiologist should know.

Imaging of the placenta can have a profound impact on patient management, owing to the morbidity and mortality associated with various placental conditions. Placental conditions affecting the mother and fetus include molar pregnancies, placental hematoma, abruption, previa, accreta, vasa previa, chorioangioma, and retained products of conception. Although uncommon, abnormalities of the placenta are important to recognize owing to the potential for maternal and fetal morbidity and mortality. Sonography remains the first imaging modality for evaluation of the placenta. Magnetic resonance (MR) imaging has many unique properties that make it well-suited for imaging of the placenta: the multi-planar capabilities, the improved tissue contrast that can be obtained using a variety of pulse sequences and parameters and the lack of ionizing radiation; MR imaging can be of added diagnostic value when further characterization is required. In this article, we review the appearances and the role of MRI in diagnosis and management of these conditions. We present our clinical perspective on diagnosing these challenging problems with MRI and review the imaging findings that can lead to a correct diagnosis.

CT and MR enterography in evaluating small bowel diseases: when to use which modality?

MR and CT techniques optimized for small bowel imaging are playing an increasing role in the evaluation of small bowel disorders. Several studies have shown the advantages of these techniques over traditional barium fluoroscopic examinations due to improvements in spatial and temporal resolution combined with improved bowel distending agents. The preference of MR vs. CT has been geographical and based on expertise and public policy. With the increasing awareness of radiation exposure, there has been a more global interest in implementing techniques that either reduce or eliminate radiation exposure. This is especially important in patients with chronic diseases such as inflammatory bowel disease who may require multiple studies over a lifetime or in studies that require sequential imaging time points such as in assessment of gastrointestinal motility. MRI has many properties that make it well suited to imaging of the small bowel: the lack of ionizing radiation, the improved tissue contrast that can be obtained by using a variety of pulse sequences, and the ability to perform real time functional imaging. Moreover, MR modalities allow visualization of the entire bowel, without overlapping bowel loops, as well as the detection of both intra- and extraluminal abnormalities.The intra- and extraluminal MR findings, combined with contrast enhancement and functional information, help to make an accurate diagnosis and consequently characterize small bowel diseases.

Imaging of stone disease in pregnancy.

Renal colic is the most frequent non-obstetric cause for abdominal pain and subsequent hospitalization during pregnancy. Intervention is necessary in patients who do not respond to conservative treatment. Ultrasound (US) is widely used as the first-line diagnostic test in pregnant women with nephrolithiasis, despite it is highly nonspecific and may be unable to differentiate between ureteral obstruction secondary to calculi and physiologic hydronephrosis. Magnetic resonance imaging (MRI) should be considered as a second-line test, when US fails to establish a diagnosis and there are continued symptoms despite conservative management. Moreover, MRI is able to differentiate physiologic from pathologic dilatation. In fact in the cases of obstruction secondary to calculi, there is renal enlargement and perinephric edema, not seen with physiological dilatation. In the latter, there is smooth tapering of the middle third of the ureter because of the mass effect between the uterus and adjacent retroperitoneal musculature. When the stone is lodged in the lower ureter, a standing column of dilated ureter is seen below this physiological constriction. MRI is also helpful in demonstrating complications such as pyelonephritis. In the unresolved cases, Computed tomography remains a reliable technique for depicting obstructing urinary tract calculi in pregnant women, but it involves ionizing radiation. Nephrolithiasis during pregnancy requires a collaboration between urologists, obstetricians, and radiologists.

Impact of magnetic resonance in the preoperative staging and the surgical planning for treating small bowel neoplasms.

PURPOSE: The role of MR enteroclysis/enterography (MRE) in the diagnosis of small bowel (SB) tumor has not been fully evaluated. The aims of this study were to assess the capability of MRE correctly identifying the site, stage and histology of such neoplasms. METHODS: MR enteroclysis/enterography was employed in consecutive patients suspected of having an SB tumor following negative upper and lower endoscopies. The SB was subdivided into proximal jejunum, middle SB and distal ileum. The histological examination (HE) of the surgical specimen was the reference standard. RESULTS: One hundred and fifty-eight patients were examined. Thirty-one out of 32 (96.9 %) SB detected by HE were correctly identified by MRE. The concordance rate between MRE and HE was 100 % for localization, and 87.1, 80.6 and 96.8 % for T, N and M stages, respectively. The concordance rate was 62.2 % for histological diagnosis. CONCLUSIONS: The high concordance rates between MRE and HE for the localization of SB tumors and for their staging have a significant impact upon surgical planning, particularly if laparoscopy is being considered. A preoperative histological diagnosis is not sufficiently reliable.

Nebulization of pharmacological solutions with an innovative medical device based on microvaporization.

The currently available nebulization devices have a slow aerosol flow and produce vapor with large microdrops. Improved devices that achieve higher airflow and produce smaller microdrops are needed to improve the clinical care of patients. To address this critical need, we developed a novel system for the molecular vaporization of liquids. This device vaporizes an active pharmacological substance dissolved in water, alcohol, or a mixture of water and alcohol using two energy sources at the same time: high-frequency ultrasound and thermal induction. Application of energy to a solution contained in the device's tank allows, within tens of seconds, for the vaporization of the solution itself, with the generation of a vapor consisting of microdrops of very small diameter (0.2-0.3 µm). In this article, we illustrate the technology used, the main verification tests performed, and the primary fields of application for this device. In particular, the advantages of both the aerosol delivery system and the administration system are highlighted.

MR imaging of the small bowel.

Small-bowel radiology has undergone dramatic changes in the past 2 decades. Despite important recent advances in small-bowel endoscopy, radiologic imaging remains important for patients suspected of having or with established small-bowel disease. Cross-sectional imaging techniques (computed tomography and magnetic resonance [MR] imaging), used to investigate both extraluminal abnormalities and intraluminal changes, have gradually replaced barium contrast examinations, which are, however, still used to examine early mucosal disease. MR imaging techniques clearly highlight endoluminal, mural and extramural enteric details and provide vascular and functional information, thereby enhancing the diagnostic value of these techniques in small-bowel diseases. Two MR imaging based techniques are currently utilized: MR enteroclysis and MR enterography. In enteroclysis, enteric contrast material is administered through a nasoenteric tube, whereas in enterography, large volumes of enteric contrast material are administered orally. MR enteroclysis ensures consistently better luminal distention than does MR enterography in both the jejunum and the ileum and more accurately depicts endoluminal abnormalities and early disease, particularly at the level of the jejunal loops. Moreover, MR enteroclysis provides a high level of accuracy in the diagnosis and exclusion of small-bowel inflammatory and neoplastic diseases and can be used for the first radiologic evaluation, while MR enterography may effectively be used to follow up both Crohn disease patients without jejunal disease and in pediatric patients where nasogastric intubation might be a problem. MR enteroclysis may also reveal subtle transition points or an obstruction in the lower small bowel, which may escape detection when more routine methods, including enterography, are used. MR imaging offers detailed morphologic information and functional data of small-bowel diseases and provides reliable evidence of normalcy, thereby allowing the diagnosis of early or subtle structural abnormalities and guiding treatment and decisions in patient care.

Spontaneous renal artery dissection diagnosed by unenhanced magnetic resonance angiography: case report.

A 47-year-old patient is presented who was admitted to the emergency department with complaints of right-sided flank pain and hypertension. His creatinine and glomerular filtration rate were 2.5 mg/dl and 37 ml/min respectively, so that contrast media administration was contraindicated. The unenhanced magnetic resonance angiography image obtained with 3D in-flow inversion recovery sequence showed right renal artery dissection, without aortic dissection. Selective renal angiography confirmed the diagnosis and a stent was placed in the renal artery. The patient was free of pain after stenting, with normalized laboratory values and blood pressure.

Imaging of primary retroperitoneal neoplasms.

Retroperitoneal soft tissue tumors are frequently incidental findings on imaging tests as Computed tomography (CT) or Magnetic Resonance Imaging (MRI). Retroperitoneal soft tissue tumors are rare and therefore not common in daily radiological practice. Clinician and radiologist'skills to set retroperitoneal soft tissue tumors at presentation is crucial for a correct patient management. So far, several diagnostic algorithms have been proposed to assess retroperitoneal masses, which have not been validated by case histories (2-5). The aim of this article is to evaluate a new classification of retroperitoneal masses using CT and MRI. KEY WORDS: CT, Diagnosis, MRI, Retroperitoneum, Soft tissue sarcoma.

Primary Leiomyosarcoma of the Mediastinum: A Rare and Challenging Diagnosis?

(1) Introduction: Leiomyosarcomas are highly aggressive mesenchymal neoplasm derived from smooth muscle cells which, in the mediastinum, are present in various primary organs; To our knowledge, less than 10 cases of primary mediastinal leiomyosarcoma have been described. Here, we report a compelling case of primary mediastinal leiomyosarcoma. (2) Case presentation: A 79-year-old woman was admitted to the Thoracic Surgery Unit of S. Andrea University Hospital for persisting cough, exertional dyspnea, and sternal pain. After multidisciplinary consultation, a CT-guided core needle biopsy of the mass was performed, resulting in a provisional diagnosis of mesenchymal neoplasm with smooth muscle differentiation without apparent signs of atypia. The patient underwent surgery that revealed a large irregularly shaped mass with a whorled pattern cut surface, showing admixed yellowish areas of necrosis and areas of hemorrhage. Histologic examination showed a smooth muscle neoplasm with atypia and necrosis, and a grade 2 primary mediastinal leiomyosarcoma diagnosis was given. (3) Conclusions: Soft tissue sarcomas represent a challenging diagnostic group of tumors due to their location, morphologic spectrum, and unique molecular background. Our case of primary mediastinal leiomyosarcoma shows how tumor heterogeneity and limited tissue sampling impact diagnosis. Further studies are needed to shed light on the disease by finding an appropriate molecular signature for each leiomyosarcoma subgroup, providing a more precise diagnosis and the correct background for tailored therapy.