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Objective: Leiomyosarcoma (LMS) is the most common subtype of uterine sarcomas. It is a rare and aggressive tumour. The aim of the present study was to assess the performance of fluorine-18-fluorodeoxyglucose (F-FDG) PET/CT imaging in four clinical settings: initial staging, restaging, monitoring of response to therapy and post-therapy surveillance of uterine LMS., Patients and Methods: A bicentric retrospective study was carried out on a group of 21 patients with uterine LMS for whom a total of 52 PET/CT scans were available in initial staging (n=11), restaging (n=11), monitoring of response to therapy (n=17) and post-therapy surveillance (n=13). Clinical (minimum 6 months after PET/CT scan) and/or imaging follow-up and pathology were used as the reference standard., Results: In the initial staging, the sensitivity, specificity and accuracy of PET imaging were 80, 100 and 91%, respectively. In the restaging and monitoring of response to therapy, all these indices were 100%, whereas they were lower in post-therapy surveillance at 75, 100 and 85%, respectively, because of two false-negative results. False-negative lesions were an infracentimetric lung nodule in the initial staging and a peritoneal nodule that had increased in size between two PET/CT scans in post-therapy surveillance. PET-negative lesions were all identified on the computed tomography (CT) part of the PET/CT; thus, the performances of the exam improved to 100% on taking into account the CT component of the PET/CT., Conclusion: PET/CT imaging has a high diagnostic yield in the initial staging and restaging of uterine LMS, but seems less sensitive in post-therapy surveillance. Evaluation of the CT part improves the sensitivity of the PET scan. Thus, PET/CT imaging should be considered in patients presenting with LMS.

The unidentified presence of uterine smooth muscle malignancies poses a tremendous risk in women planning surgery for presumed benign leiomyomas. We sought to investigate whether preoperative FDG PET may be useful to identify leiomyosarcomas (LMS) and smooth muscle tumors of uncertain malignant potential (STUMP). Methods . We investigated patients with rapidly growing uterine masses which were suspected of being malignant on ultrasound or MRI. Among the 21 patients who underwent FDG PET, we identified 7 LMS, 1 STUMP, and 13 leiomyomas. PET-derived parameters and FDG uptake patterns were analyzed retrospectively. Results . The SUV max values of LMS/STUMP (range: 3.7-11.8) were significantly higher than those observed in leiomyomas (range: 2.0-9.4; P =0.003) despite a significant overlap. The metabolic tumor/necrosis ratio was significantly higher in LMS/STUMP than in leiomyomas ( P < 0.001), with no significant intergroup overlaps. All LMS/STUMP revealed a characteristic pattern of FDG uptake, identifying a specific "hollow ball" sign (corresponding to areas of coagulative tumor necrosis). In contrast, this sign was invariably absent in patients with leiomyomas. Conclusion . The characteristic FDG uptake pattern instead of SUV on PET images allows identifying LMS/STUMP in patients with rapidly growing uterine masses, avoiding the deleterious consequences of regular surgery for presumed benign leiomyomas.

Objective: The purpose of this study is to assess the utility of texture analysis of multiple MRI sequences for the differentiation of uterine leiomyomas and leiomyosarcomas., Materials and Methods: Seventeen leiomyosarcomas and 51 leiomyomas undergoing MRI before resection were included. Whole-lesion volumes of interest were placed on T2-weighted images, contrast-enhanced T1-weighted images, and apparent diffusion coefficient (ADC) maps. The diagnostic performance of histogram metrics was assessed., Results: For T2-weighted images, significant differences were observed for mean, skewness, entropy, mean of the bottom 10th percentile (mean 0-10 ), mean of the 10th through 25th percentiles (mean 10-25 ), and mean of the 25th through 50th percentiles (mean 25-50 ) (p ≤ 0.010). For T1-weighted contrast-enhanced images, significant differences were observed for mean 0-10 , mean 10-25 , and mean 25-50 (p ≤ 0.045). For the ADC maps, no metrics showed a significant difference (p ≥ 0.067). Metrics with AUC greater than 0.8 were the mean 0-10 (0.875), mean 10-25 (0.863), mean 25-50 (0.839), and mean (0.802) for T2-weighted imaging. The mean 0-10 , mean 10-25 , and mean 25-50 for T2-weighted imaging all achieved greater AUCs than did the standard mean (p ≤ 0.038). Patients with leiomyosarcoma were significantly older than those with leiomyoma (p < 0.001; AUC = 0.866). At multivariable regression, significant independent predictors of leiomyosarcoma were patient age (p = 0.002) and T2-weighted imaging mean 0-10 (p = 0.004), with a combined AUC of 0.955. Patient age achieved sensitivity of 82.4% and specificity of 92.2%; T2-weighted imaging mean 0-10 achieved sensitivity of 82.4% and specificity of 74.5%., Conclusion: For whole-lesion histogram metrics obtained on various MRI sequences, T2-weighted images provided the highest, and ADC maps the lowest, performance for differentiating uterine leiomyomas and leiomyosarcomas. Metrics reflecting percentiles from the bottom half of the histogram distribution outperformed the standard mean. Models combining the T2-weighted imaging whole-lesion metrics and patient age achieved particularly high diagnostic performance. Although these findings require validation in larger studies, they have implications for facilitating improved treatment selection for these two entities.

Background: Leiomyosarcoma of the head and neck region is very rare. Primary parotid leiomyosarcoma has only been reported nine times in the medical literature., Methods: A 68-year-old female presented with a left facial mass. Physical examination revealed a firm immobile mass at the level of the left parotid tail. No facial nerve dysfunction or palpable adenopathy was noted at the time of presentation. This patient underwent a superficial parotidectomy with a facial nerve dissection and left selective neck dissection., Results: Pathologic findings revealed a sarcoma of intermediate to high-grade, composed of spindle cells with herringbone pattern, eosinophilic fibrillary cytoplasm, and focal granularity. Immunohistochemistry was positive for vimentin and smooth muscle actin and negative for desmin, S100 and CD34. The findings are consistent with a leiomyosarcoma. Following complete surgical resection, adjuvant radiation therapy was administered., Conclusion: Primary sarcomas of the parotid gland, specifically parotid leiomyosarcomas, are extremely rare. Based on this patient's tumor size, grade and resectability, this case met the criteria for a primary leiomyosarcoma. We present only the tenth case of a primary parotid leiomyosarcoma to be reported in the English literature., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: To retrospectively assess conventional magnetic resonance imaging (MRI) features that differentiate malignant pure mesenchymal uterine tumors (MPMUT); endometrial stromal sarcoma (ESS) and leiomyosarcoma (LMS) from uterine leiomyoma with cystic degeneration (ULCD)., Methods: We retrospectively reviewed magnetic resonance (MR) images of 30 patients with ULCD, 18 with ESS, and 15 with LMS, to assess tumor location, margin, T2 signal intensity (SI), speckled appearance, and peripheral band using univariate and multivariate analyses., Results: ULCD more frequently showed subserosal location (53.3%), well-defined margin (96.7%), and speckled appearance (90.0%) compared with ESS (0%, 33.3%, and 33.3%, respectively) or LMS (20.0%, 33.3%, and 60.0%, respectively). In quantitative T2 SI comparisons, the T2 SI ratio of the main solid tumor portion to gluteus maximus muscle differed significantly among the three groups, with ULCD showing a lower SI ratio (0.62) compared with ESS (2.44) and LMS (1.13). On multivariate analysis, an ill-defined margin (odds ratio [OR]=44.885; p=0.003) and high T2 SI (OR=4.396; p=0.046) were the significant MR differentiators., Conclusion: An ill-defined tumor margin and high T2 SI ratio of the main solid tumor-to-gluteus maximus muscle were useful MRI features in the differentiation of MPMUT from ULCD., Competing Interests: No potential conflict of interest relevant to this article was reported., (Copyright © 2018. Asian Society of Gynecologic Oncology, Korean Society of Gynecologic Oncology.)

Objective: Hyperintense area on T 1  weighted images (T 1 HIA) have been reported as suggestive features of uterine leiomyosarcoma (LMS), but differentiating LMS from leiomyoma (LM) is often difficult. This study aimed to evaluate the differences between uterine LMS and LM demonstrating intratumoral T 1 HIA., Methods: MRI was performed in 509 patients with 1137 uterine smooth muscle tumours [14 LMSs, 5 smooth muscle tumours of uncertain malignant potential , and 1118 LMs] which exceeded 3 cm in diameter. LM with red degeneration and lipoleiomyoma were excluded from the study. We retrospectively reviewed the images and assessed T 1 HIA within tumours. Results T 1 HIAs were observed in 11/14 (78.6%) LMSs, 0/5 (0%) smooth muscle tumours of uncertain malignant potential, and 15/1118 (1.3%) LMs. T 1 HIAs were more homogenous (53 vs 0%, p < 0.01) and more well-demarcated (60 vs 9%, p < 0.05) in LMs than in LMSs. T 2 hypointense rim within T 1 HIA (53 vs 9%, p < 0.05) was more frequently observed in LMs than in LMSs. The occupying rate of T 1 HIA (0.20 ± 0.24 vs 0.42 ± 0.27, p < 0.05) was smaller in LMs than in LMSs. The signal intensity ratio of T 1 HIA (1.83 ± 0.36 vs 1.38 ± 0.23, p < 0.01) was greater in LMs than in LMSs., Conclusion: T 1 HIA within LM showed more homogeneity, better demarcation, smaller occupying rate, and higher signal intensity than T 1 HIA within LMS. Advances in knowledge: The differences in T 1 HIA within tumours may be useful for differentiating between LMS and LM.

Background: Leiomyosarcoma is a rare neoplasm of the head and neck. The purpose of this study was to present our single-institution case series of head and neck leiomyosarcoma and a review of cases in the National Cancer Data Base (NCDB)., Methods: Patients with head and neck leiomyosarcoma at the University of Pennsylvania and in the NCDB were identified. Demographic characteristics, tumor factors, treatment paradigms, and outcomes were evaluated for prognostic significance., Results: Nine patients with head and neck leiomyosarcoma from the institution were identified; a majority had high-grade disease and cutaneous leiomyosarcoma, with a 5-year survival rate of 50%. Two hundred fifty-nine patients with leiomyosarcoma were found in the NCDB; macroscopic positive margins and high-grade disease were associated with poor prognosis (P < .01), and positive surgical margins were related to adjuvant radiation (P < .001)., Conclusion: Head and neck leiomyosarcoma presents at a high grade and is preferentially treated with surgery. Several demographic and tumor-specific factors are associated with outcomes and prognosis., (© 2017 Wiley Periodicals, Inc.)

Sarcomas are rare but malignant tumors with high risks of local recurrence and distant metastasis. Anti-angiogenic therapy is a potential strategy against un-controlled and not-organized tumor angiogenesis. We aimed to assess the safety and efficacy of apatinib, an oral tyrosine kinase inhibitor targeting vascular endothelial growth factor receptor-2, in patients with advanced sarcoma. Thirty-one patients who received initial apatinib between September 2015 and August 2016 were retrospectively reviewed. Among them, 19 (61.3%) patients were heavily pretreated with two or more lines of cytotoxic chemotherapy. Apatinib was given at a start-dose of 425 mg qd. During therapy, 9 (29.0%) patients required dose interruption and 7 (22.6%) needed dose reduction, and the mean dosage of apatinib was 372.9 ± 68.4 mg/day. In the study cohort, one patient was treated as adjunctive therapy and 6 patients stopped treatment before radiographic response assessment. Thus, 24 patients were eligible for tumor response evaluation. The objective response rate was 33.3% and clinical benefit rate was as high as 75.0%. The progression free survival was 4.25 (95% confidence interval [CI], 2.22-5.11) months, whereas the overall survival was 9.43 (95% CI, 6.64-18.72) months. Compared with other histological subtypes, leiomyosarcoma did not show significant survival benefits. Most of the adverse events (AEs) were at grade 1 or 2. The main grade 3 AEs were hypertension (6.5%), hand foot skin reaction (6.5%), and diarrhea (3.2%). In conclusion, apatinib showed promising efficacy and acceptable safety profile in metastatic or recurrent sarcoma, giving rationale clinical evidence to conduct clinical trials.

Brain metastases are the most commonly seen intracranial lesions in adults. What is more, meningiomas are the most common primary intracranial tumours after gliomas and their imaging characteristics are well known in both CT and MRI scans. However, there are lesions that can mimic meningiomas in imaging studies, including metastases of extracranial tumours, confronting us with a diagnostic and therapeutic challenge. We present the case of a patient with meningeal metastasis of a uterine leiomyosarcoma that was not known at the time of the surgical intervention., (Copyright © 2017 Sociedad Española de Neurocirugía. Publicado por Elsevier España, S.L.U. All rights reserved.)

Background Intraorbital lesions associated with symptomatic trigeminal autonomic cephalalgias (TACs) are rarely reported. We present a case of orbital metastatic leiomyosarcoma, presenting with chronic paroxysmal hemicrania-like headache. Case report A 43-year-old man presented with a severe paroxysmal headache in his left periocular and frontal area for a year. The attacks occurred 10-12 times per day, lasting 10-15 minutes with ipsilateral lacrimation and conjunctival injection. Neurological examination and brain MRI without contrast were unremarkable. Different medications were tried, without beneficial effects. A follow-up contrast-enhanced brain MRI performed one year after the baseline MRI revealed an enhancing mass in the left superior oblique muscle. Orbital metastatic leiomyosarcoma arising from the thigh was revealed. He received gamma knife surgery, which completely resolved the headache. Discussion Intraorbital lesion should be considered a possibility in patients with headache mimicking TACs. Baseline contrast-enhanced MRI is essential, and repeated MRI scans might be needed if clinically indicated.

Rationale: Leiomyosarcoma (LMS) is a common type of soft tissue sarcoma. Primary colonic LMS in general is a very rare entity, accounting for 1% to 2% of gastrointestinal malignancies., Patient Concerns: We report a case of 55-year-old female who presented with a sudden onset of sharp right lower quadrant abdominal pain. Electronic colonoscopy showed a normal lumen. However, an abdominal computed tomography scan revealed a mass of soft tissue attenuation inseparable from the ascending colon which appeared as a gastrointestinal stromal tumor (GIST)., Diagnoses: It is important to diagnose LMS definitively by immunohistochemical profiling of smooth muscle actin, desmin, and CD34., Interventions: She underwent laparotomy and right hemicolectomy, and histology confirmed a colonic LMS. The patient received no oncological treatment after surgery., Outcomes: No recurrence or metastasis was observed at 5 months postoperatively. It is crucial to identify colonic LMS precisely based on immunohistochemistry, and thereby distinguish it from GIST., Lessons: Further investigation on LMS cases so far is required to establish standard treatment strategies.

Primary leiomyosarcoma is a rare tumor in the CNS, with few reported cases. Here, we describe a case of a primary intracranial leiomyosarcoma of the tentorium cerebelli. A 43-year-old woman presented with headache, acute vision loss, and difficulty speaking. MRI revealed a large heterogeneous-enhancing occipital mass, which was subsequently resected and diagnosed as a primary intracranial leiomyosarcoma. The patient went onto adjuvant radiotherapy delivering 60 Gy in 30 fractions. These tumors are exceedingly rare in immunocompetent individuals. We reviewed the 16 cases that have been reported in the literature. Surgical resection was the most common treatment (92%) with 53% receiving adjuvant radiation. There currently is no standard treatment regimen for intracranial leiomyosarcomas. Additional case reports that include descriptive treatment approaches with patient outcomes may help ascertain the best approach to treating these malignancies., (Copyright © 2018 Elsevier B.V. All rights reserved.)

The objective of this article is to discuss the pathophysiology, diagnosis, differential diagnosis, and therapy of vulvar leiomyoma. We performed a review of all English-language reports of vulvar leiomyoma published in PubMed from 1978 to 2015 using the following search terms: "vulval leiomyoma," "vulvar leiomyoma," "vulval smooth muscle tumor," and "external genitalia smooth muscle tumor." Vulvar leiomyomas, which are rare benign monoclonal tumors, most commonly occur in the fourth and fifth decades of life. The genetics of vulvar leiomyoma remain undefined. Three principal histological patterns have been identified: spindled, epithelioid, and myxoid. Imaging tests such as ultrasound, endoscopic ultrasound, and magnetic resonance imaging are used in diagnosis. Surgical excision is the only curative treatment for vulvar leiomyomas. Establishment of a full differential diagnosis list and correct final diagnosis before surgery are essential for optimal clinical management. Although recurrence of vulvar leiomyoma is extremely rare, long-term follow-up of all cases is advisable.

Objectives: There are no current established pathognomonic diagnostic features for uterine leiomyosarcomas in the pre- or perioperative setting. Recent inadvertent upstaging of this rare malignancy during laparoscopic morcellation of a presumed fibroid has prompted widespread debate among clinicians regarding the safety of current surgical techniques for management of fibroids. This study aims to conduct a systematic review investigating significant diagnostic features in magnetic resonance imaging (MRI) of uterine leiomyosarcomas., Methods: A comprehensive database search was conducted guided by PRISMA recommendations for peer-reviewed publications to November 2017. Parameters available in MRI were compared for reliability and accuracy of diagnosis of leiomyosarcomas. A decision tree algorithm classifier model was constructed to investigate whether T1 and T2 MRI signal intensities are useful indicators., Results: Nine eligible studies were identified for analysis. There appears to be a significant relationship between histopathological type and T1 and T2 intensity signals (p < .05). A decision tree model analyzing T1 and T2 signal intensity readings supports this trend, with a diagnostic specificity of 77.78 percent for uterine leiomyosarcomas. The apparent diffusion coefficient (ADC) values were not observed to have a significant relationship with tumor pathology (p = .18)., Conclusions: Various studies have investigated pre- and perioperative techniques in differentiating uterine leiomyosarcoma from benign fibroids. Given the rarity of the malignancy and lack of pathognomonic diagnostic parameters, there is difficulty in establishing definitive criteria. A decision tree model is proposed to aid diagnosis based on MRI signal intensities.

Background/aim: Primary hepatic leiomyosarcoma is an extremely rare type of liver sarcoma with relatively poor prognosis, with about 50 cases having been reported in the literature. Potential origins of this tumor in the liver are the smooth muscle cells in the round ligament, intrahepatic blood vessels and intrahepatic bile ducts. There is no apparent sex predilection and there is a wide age range. The clinical presentation is not specific and the diagnosis depends on the expression of markers such as smooth muscle actin, desmin and vimentin by tumor cells., Patients and Methods: Herein, we present a case of a bulky primary hepatic leiomyosarcoma in a 68-year-old female patient. The patient underwent resection of the exophytic mass en block with the hepatic segments III and IVB., Conclusion: Surgical resection is the most effective among the treatment options.

A 40's woman had a complaint of abdominal and back pain. Enhanced CT visualized a large retroperitoneal tumor and huge multiple myomas of the uterus. The tumor was 10cm in diameter and located in the anterior of the inferior vena cava, and progressed from the posterior of the duodenum to the abdominal aortic bifurcation. Diffusion-weighted MR image showed the tumor with high signal intensity. Upper gastrointestinal endoscopy revealed a type 2 tumor at the anal side of the Vater. The patient was performed curativly abdominal total hysterectomy and pancreaticoduodenectomy with inferior vena cava resection. Immunohistochemical examination showed that the tumor cells were negative for CD34 and c-kit, and positive for desmin and a-SMA. The tumor was histopathologically diagnosed as leiomyosarcoma originating from the duodenum.

Background: Non-gastrointestinal stromal tumor sarcomas (NGSs) have heterogeneous histology, and this heterogeneity may lead to uncertainty regarding the prognosis of patients with liver metastases from NGS (NGSLM) and decision regarding their surgical management. Furthermore, the role of preoperative chemotherapy in treatment of NGSLM remains poorly defined. We investigated long-term survival and its correlation to response to preoperative chemotherapy in patients with NGSLM., Patients and Method: Patients who underwent liver resection for NGSLM during 1998-2015 were identified. Clinical, histopathologic, and survival data were analyzed. Multivariate analysis was performed using a Cox proportional hazards model., Results: 126 patients [62 (49%) with leiomyosarcoma] were included. Five-year overall survival (OS) and recurrence-free survival (RFS) rates were 49.3 and 14.9%, respectively. Survival did not differ by histologic subtype, primary tumor location, or use of preoperative or postoperative chemotherapy. NGSLM ≥ 10 cm and extrahepatic metastases at NGSLM diagnosis were the only independent risk factors for OS. In the 83 (66%) patients with metachronous NSGLM, disease-free interval > 6 months was associated with improved OS and RFS. Among the 65 patients (52%) who received preoperative chemotherapy, radiologic response according to Choi criteria specifically was associated with improved OS (p = 0.04), but radiologic response according to RECIST 1.1 criteria was not., Conclusions: Resection of NGSLM led to a 5-year OS rate of 49%, independent of histologic subtype and primary tumor location. Choi criteria (which take into account tumor density) are superior to RECIST 1.1 in assessing radiologic response and should be used to assess response to preoperative chemotherapy.

The diagnosis of a uterine myoma size and location can be very precise when a 3D sonograph and knowledge are available. The majority of fibroids are asymptomatic, and expectant management is recommended. In young patients, fibroids cause infertility and in middle-aged women, abnormal uterine bleedings. Laparoscopic myomectomy is the preferred way of surgery for IM and SS fibroids, versus hysteroscopy for SM fibroids. In both cases, the size, number of fibroids and the surgeon's experience determine the limitations of the MIGS. Medical treatments provide only temporary tumor reduction and symptom alleviation. Leiomyosarcoma risk is higher in older women usually carrying fibroids larger than 8 cm. There are no other pathognomonic parameters ruling out a sarcoma. In case of suspected fibroid malignancy, the best treatment option is laparotomy and total hysterectomy. Myomectomy complications can be reduced when MIGS is performed by a surgeon with proper training and experience., (Copyright © 2017. Published by Elsevier Ltd.)

Recently, several authors have reported stenting into the right upper lobe bronchus using bifurcated stents, but little has been reported on stenting into the right middle or lower lobe bronchus. We report a case with metastatic uterine leiomyosarcoma, who was successfully treated by rigid bronchoscopic intervention, including silicone Y-stenting on the carina between the right middle lobe bronchus and the lower lobe bronchus., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Carney triad is a very rare syndrome characterized by the synchronous or metachronous occurrence of gastrointestinal stromal tumors, pulmonary chondroma, and extra-adrenal paraganglioma. We present the case of a 36-year-old woman with complete Carney triad who underwent a Ga-DOTA-TATE PET/CT scan for restaging of metastasizing extra-adrenal paraganglioma and for evaluation of targeted radionuclide therapy potential. On the Ga-DOTA-TATE PET scan, increased tracer accumulation was observed in paraganglioma metastases. This case highlights the usefulness of Ga-DOTA-TATE PET/CT for restaging of metastasizing paraganglioma in Carney triad and the option of targeted radionuclide therapy in this entity.

Rationale: Primary leiomyosarcoma (LMS) of the vein is a rare tumor that arises from the smooth muscle cells of the vessel wall and has an extremely poor prognosis. This tumor can occur in vessels such as the inferior vena cava, great saphenous vein, femoral vein, iliac vein, popliteal vein, and renal vein; the inferior vena cava is the most common site. LMS of the femoral vein can result in edema and pain in the lower extremity; therefore, it is not easy to be differentiated from deep vein thrombosis (DVT). Moreover, virtually no studies have described the ultrasonographic features of LMS of the vein in detail., Patient Concerns: We present a case of a 55-year-old woman with LMS of the left femoral vein that was misdiagnosed as having deep vein thrombosis (DVT) on initial ultrasonographic examination. The patient began to experience edema and pain in her left leg seven months previously. She was diagnosed as having DVT on initial ultrasonographic examination, but the DVT treatment that she had received for 7 months failed to improve the status of her left lower limb., Diagnoses: She subsequently underwent re-examination by means of a multimodal ultrasonographic imaging approach (regular B-mode imaging, color Doppler imaging, pulsed-wave Doppler imaging, contrast-enhanced ultrasonography), which confirmed a diagnosis of LMS., Interventions: This patient was treated successfully with surgery., Outcomes: This case demonstrates that use of multiple ultrasonographic imaging techniques can be helpful to diagnose LMS accurately. Detection of vasculature in a dilated vein filled with a heterogeneous hypoechoic substance on ultrasonography is a sign of a tumor., Lessons: The pitfall of misdiagnosing this tumor as DVT is a useful reminder.

A 61-year-oldman presentedwith a chief complaint of abdominal pain. Enhancedcomputed tomography andmagnetic resonance imaging showeda left adrenal mass with a diameter of 7 cm with heterogeneous enchancement. He was referredto our hospital for further treatment. No endocrinological abnormality was detected. The tumor showed abnormal uptake on fludeoxyglucose positron emission tomography scan. Preoperative diagnosis was left adrenocortical carcinoma (cT2N0M0). Tumor excision was performedandpathological findings on the resectedspecimen revealedleiomyosarcoma of the left adrenal grand. The patient has been followed up for 16 months with no additional treatment. No evidence of local recurrence or metastasis was seen.

Rationale: Leiomyosarcoma (LMS) is a malignant sarcoma that can occur in different anatomic sites, including the bone, showing similar histological characteristics but heterogeneous clinical behavior and prognosis. Primary bone LMS was first described in 1965. It is a very rare sarcoma, accounting for <0.7% of all primary malignant bone tumors., Patient Concerns: We report the case of a 52-year-old male with primary bone LMS who presented with a solitary osteolytic lesion with focal cortical destruction in the left clavicle, seen on an x-ray and subsequent computed tomography (CT) scan., Diagnosis: The multidisciplinary Osteoncology team of our institute planned a biopsy that revealed the presence of spindle and pleomorphic cells with a positive reaction for smooth muscle actin and desmin at immunohistochemical analysis, without the presence of cartilage or bone matrix. These results were consistent with a high-grade malignant LMS arising from the bone., Interventions: Complete surgical resection of the tumor was performed and a decision was made with the patient not to proceed with adjuvant chemotherapy or radiotherapy., Outcomes: After more than 1 year of surgery, the patient is well, with no evidence of recurrent or metastatic disease. Follow-up is ongoing., Lessons: Little is known about the biology and clinical behavior of bone LMS due to its extreme rarity. A multidisciplinary team in a specialized center is needed for the optimal management of the disease. Surgery with a curative intent is the cornerstone of treatment of localized disease. No data are available about chemotherapy in neoadjuvant, adjuvant, or advanced settings. Further research is needed to identify more effective therapies.

A 74-year-old woman presented with an 8-year history of unilateral swelling of her right lower limb, which was thought to be 'idiopathic' lymphoedema until she noticed a painless swelling in her right groin. Physical examination showed a painless, non-pulsatile, deep-seated mass in her right proximal thigh with significant lymphoedema distally. MRI scan showed a large mass at her right inguinal region, involving the common femoral vein. Histological examination on complete excision revealed leiomyosarcoma. It is important to exclude proximal obstructive lesion before making a diagnosis of unilateral idiopathic lower limb lymphoedema., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Transoesophageal echocardiography (TOE) is regularly used in cardiac and also increasingly in non-cardiac surgeries. It has a leading role in the perioperative management of renal cell carcinoma with tumour extension into inferior vena cava. We report two cases in which TOE was of immense help for patient management. This report highlights the two cases where intraoperative TOE was of immense help to establish diagnosis, to modify surgical plan, to guide the surgeon during clamp placement, to monitor cardiac function, to rule out tumour embolism and to confirm the complete removal of tumour thrombus., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

A Tc-MDP bone scintigraphy was performed in an 88-year-old man with a history of right external oblique muscle leiomyosarcoma, which was resected 8 months ago. The static images showed abnormal tracer uptake in the region of resection bed. SPECT/CT images demonstrated that the increased activity was from a partially calcified soft tissue mass in external oblique muscle. Subsequent pathological examination confirmed recurrent tumor.

A 54-year-old woman presented withtransient back pain. She was diagnosed withleiomyosarcoma of the inferior vena cava (IVC) by computed tomography (CT) and was referred to our hospital. Contrastenhanced CT revealed a mass (38×42 mm) located in the retroperitoneal space along the course of the right ovarian vein. The mass compressed the IVC into a crescent shape. A tumor thrombus was also found in the IVC. 18 F-fluoro-2-deoxy-D-glucose positron emission tomography (PET) revealed high uptake at the caudal side of the tumor. These radiological findings strongly suggested the diagnosis of leiomyosarcoma arising from the right ovarian vein. She underwent tumor resection with right nephrectomy, IVC resection, and IVC patch reconstruction without any notable events after surgery. Histopathological diagnosis was leiomyosarcoma arising from the ovarian vein, not from the IVC. Two months after the surgery, CT revealed multiple pulmonary metastases and a single liver metastasis. The patient was referred to another hospital for further treatment. She was treated with chemotherapy and was alive with disease at 14 months after the surgery.

Objective: One challenging feature of head and neck pathology is that a dizzying array of spindle cell lesions occurs here which ranges all the way from reactive, very aggressive forms to malignant lesions. Leiomyosarcoma is one such malignant tumour of mesenchymal origin exhibiting smooth muscle differentiation; presenting generally nonspecific signs and symptoms. Here we present a case of leiomyosarcoma in a 21 year old female patient associated with single reddish pink swelling present in the posterior right maxillary tuberosity region with moderate facial asymmetry. On histopathological analysis, all the classic features were noted and diagnosis of a spindle cell neoplasm was made without any obscurity. There are many lesions like rhabdomyosarcoma, fibrosarcoma, liposarcoma, multiple peripheral nerve sheath tumour, angiosarcomas, melanomas, kaposi sarcoma, solitary fibrous tumors, etc., which mimic leiomyosarcoma clinically and histopathologically and therefore immunohistochemical staining with specific markers plays a vital role in arriving at a conclusive diagnosis.

Leiomyosarcoma (LMS) is a mesenchymal tumor originating from the smooth muscle cells. LMS of the great vessels accounts for 60% of cases, with inferior vena cava being the most common site. Pulmonary vein LMS is an extremely rare subset that was first reported in 1939. LMS is an aggressive tumor, making surgical resection the treatment of choice. Herein, we present a rare case of pulmonary vein LMS extending into the left atrium, which was resected., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Uterine leiomyosarcomas are very rare and highly aggressive tumors that have a high rate of recurrence and poor prognosis, even when early diagnosed. Due to their relative rarity, there is limited research on optimal management strategies., Case Presentation: A 60-year-old woman with a history of an asymptomatic uterine leiomyoma presented in October 2015 with postmenopausal bleeding and a friable vaginal cyst that bled when palpated. A partial cystectomy was performed, and malignant-like cystic and solid components were identified. Histopathology diagnosed an unclassifiable malignant epithelioid tumor. Subsequent imaging studies identified a malignant uterine tumor, a metabolically active vaginal lesion, and two benign leiomyomas. An anterior pelvic exenteration (colpectomy, hysterectomy, bilateral adnexectomy, total cystectomy, and cutaneous ureteroileostomy ad modum Bricker) were performed by laparotomy in March 2016. Examination of the surgical specimens identified a 75 × 75-mm leiomyoma, an 80 × 30-mm infiltrating mesenchymal uterine lesion with vascular invasion and tumor emboli, and a 60 × 30-mm perivascular vaginal tumor. Immunohistochemistry indicated a phenotypic transition from a uterine leiomyosarcoma to a vaginal epithelioid lesion; marker expression changed from the uterine tumor actin+/desmin+/caldesmon+/CD10- phenotype, through the tumor emboli, to an actin-/desmin-/caldesmon-/CD10+ phenotype in the vaginal lesion. A high-grade uterine mesenchymal tumor and vaginal metastasis were diagnosed. Adjuvant chemotherapy with docetaxel, gemcitabine, and doxorubicin commenced in May 2016 and treatment has been well tolerated., Conclusions: Differentiating leiomyosarcoma from leiomyoma is challenging and few tools other than microscopic evaluation are available. Vaginal compromise in leiomyosarcoma usually results from tumor extension, not hematogenous metastasis. A vaginal metastasis is a very rare initial presentation. We have found only two cases like this described on published literature. The atypical clinical and histological presentation in our case complicated diagnosis and delayed treatment. An early diagnosis and complete surgical clearance gives the best chance of survival, and imaging tools should be applied early in instances of new suspicious malignant lesions.

Purpose: The study aimed to investigate magnetic resonance diffusion-weighted imaging (DWI) in the differentiation of uterine leiomyosarcoma (ULMS) from degenerated leiomyoma (DLM)., Methods: Sixteen patients with ULMSs and 26 patients with DLMs confirmed by surgery and pathology underwent conventional magnetic resonance imaging and DWI. The mean apparent diffusion coefficient (ADC) values of the 2 groups' tumors were measured and compared using an independent-sample t test (b = 0.1000 s/mm [ADC1]; b = 0.800 s/mm [ADC2], respectively). A receiver operating characteristic curve was used to evaluate the diagnostic performance of DWI in the differentiation of ULMS from DLM. Intraobserver and interobserver agreements were evaluated using an intraclass correlation coefficient and Bland-Altman analysis., Results: The mean ADC value in ULMSs (0.81 ± 0.14 × 10mm/s [ADC1], 0.90 ± 0.11 × 10mm/s [ADC2]) was significantly lower than that in DLMs (1.22 ± 0.22 × 10mm/s [ADC1], 1.50 ± 0.22 × 10mm/s [ADC2]) (P < 0.001, <0.001, respectively). The sensitivity, specificity, accuracy, and positive and negative predictive values for characterizing ULMS were 100%, 90%, 93%, and 83% and 100% [ADC1] and 100%, 93%, 96%, and 90% and 100% [ADC2]; respectively. Intraobserver and interobserver reproducibilities were excellent (intraclass correlation coefficient = 0.967-0.988; small variability and 95% limits of agreement)., Conclusions: Diffusion-weighted imaging is helpful in differentiating ULMS from DLM.

Background: Large vessel sarcomas are rare tumours. Leiomyosarcoma of the inferior vena cava is the most common. About 300 cases have been reported in the literature. They tend to be large, and not develop metastasis. The prognosis of these tumours is poor., Clinical Case: An 81 year-old woman who complained of pain in the right flank, with no other symptoms. Abdominal computed tomography showed a large retroperitoneal mass, which affected the inferior vena cava, with signs of thrombosis inside. It also encompassed the right renal vein and the right kidney. Excision of the tumour was performed in block, performing an autologous saphenous vein bypass between left the renal vein and proximal segment of inferior vena cava., Discussion: Leiomyosarcomas of the inferior vena cava are classified according to their relationship with adjacent structures. The clinical signs and symptoms are generally non-specific. Diagnosis is made using computed tomography or magnetic resonance imaging, and biopsy of the retroperitoneal mass. Surgery is the only treatment capable of providing prolonged survival. The surgical management is determined by: the level of involvement, the extension, and the presence or absence of collateral veins. The role of adjuvant therapy is controversial., Conclusions: Inferior vena cava leiomyosarcomas remain a challenge for surgeons. At present, radical resection with negative margins, offers the highest survival rate. The best results are obtained with a multidisciplinary approach by experienced teams in the management of these tumours., (Copyright © 2016 Academia Mexicana de Cirugía A.C. Publicado por Masson Doyma México S.A. All rights reserved.)

Uterine myometrial tumors are predominantly benign conditions that affect one-third of women and represent the main indication for hysterectomy. Preoperative imaging is of utmost importance for characterization and for precise mapping of myometrial tumors to best guide therapeutic strategy. New minimally invasive therapeutic strategies including morcellation, myolysis, uterine artery embolization and image-guided radiofrequency or focused ultrasound ablation have been developed for the treatment of uterine leiomyoma. However, preoperative differentiation between atypical leiomyomas and leiomyosarcomas is critical on imaging as uterine sarcoma requires a specific surgical technique to prevent dissemination. A single, rapidly growing uterine tumor, associated with endometrial thickening and ascites, in post-menopausal women is suspicious of uterine endometrial stromal sarcoma and carcinosarcoma. Suggestive magnetic resonance imaging features have been described, but overlap in imaging appearance between uterine leiomyosarcomas and cellular leiomyomas makes it challenging to ascertain the diagnosis. This review aims to illustrate the imaging features of uterine sarcomas and potential mimickers to make the reader more familiar with this serious condition which needs special consideration.

Tumors arising from or secondarily involving the vena cava (VC) are broad-spectrum diseases, ranging from benign to malignant. The benign tumors include myxoma, leiomyomatosis and leiomyoma. The primary malignant tumors of the VC are rare, and of these rare entities, leiomyosarcoma is the most frequently encountered. The direct extension of a tumor arising from an adjacent organ such as hepatocellular carcinoma or renal cell carcinoma is more common than primary malignant tumor in the inferior VC. In this article, the authors review the spectrum of VC pathologic processes and the relevant findings from magnetic resonance imaging, ultrasonography, and computed tomography., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: Uterine leiomyosarcoma (LMS) is a rare and aggressive disease with poor outcome. Due to its rarity and conflict of data, investigation on finding prognostic factor is challenging. The aim of the study was to investigate the prognostic significance of preoperative ¹⁸F-fluorodeoxyglucose (¹⁸F-FDG) positron emission tomography/computed tomography (PET/CT) in uterine LMS., Methods: This was a retrospective observational cohort study in 3 tertiary referral hospitals. We retrospectively evaluated data from patients with pathologically proven uterine LMS who underwent preoperative ¹⁸F-FDG PET/CT scans at 3 institutions. The prognostic implication of PET/CT parameters and other clinico-pathological parameters on disease-free survival (DFS) and overall survival (OS) was evaluated., Results: Clinico-patholgical data were reviewed for 19 eligible patients. In the group overall, median DFS and OS were 12 and 20 months, respectively. As for the recurrence, large tumor size, and high tumor maximum standardized uptake value (SUVmax) were demonstrated as risk factors of recurrence. As for the OS, high tumor SUVmax was demonstrated as the unique risk factor. There were significant differences in tumor size, mitotic count, SUVmax, and DFS between patients with and without recurrence. Also, there were significant differences in tumor size, SUVmax, DFS, and OS between 2 subgroups stratified by cut-off SUVmax., Conclusion: SUVmax at preoperative ¹⁸F-FDG PET/CT was associated with worse outcome in patients with uterine LMS. In the preoperative setting, SUVmax can be a valuable non-invasive prognostic marker. Additionally, SUVmax can help identify highly aggressive uterine LMS and may help in adjusting standard treatment toward an individualized, risk-adapted treatment., Competing Interests: No potential conflict of interest relevant to this article was reported., (Copyright © 2017. Asian Society of Gynecologic Oncology, Korean Society of Gynecologic Oncology)

Objective: To study the CT features for solitary fibrous tumor (SFT) in the abdomen and pelvis and to improve the diagnostic accuracy.
 Methods: Fourteen patients with SFT were collected in our hospital from January, 2011 to December, 2015. Characteristic of images were analyzed and compared for 10 SFT, which located outside the abdominal organs with extragastrointestinal stromal tumors (EGIST), leiomyosarcoma, and schwannoma.
 Results: Necrosis and cystic formation were frequently present in SFT in the abdomen and pelvis. CE-CT showed serpentine vessels along the periphery, while pattern of enhancement was map-like inhomogeneous progressive. Comparing with the EGIST or schwannoma, the difference of CT value in non-contrast and the arterial phase were statistically significant (P<0.05). The numbers of peritumoral circuity vessel were significantly different between SFT and EGIST (χ²=18.27, P<0.008) or between SFT and schwannoma (χ²=19.25, P<0.008). Comparing with the leiomyosarcoma or schwannoma, SFT located outside the abdominal organs. We found that tumor necrosis rate was significantly different between SFT and leiomyoscarcoma (χ²=8.00, P<0.008).
 Conclusion: SFT in the abdomen and pelvis show certain CT characteristics. The CT value in non-contrast and at the arterial phase, tumor necrosis rate, and serpentine vessels along the periphery were pivotal in differentiating SFT from leiomyosarcoma, EGIST and schwannoma.

Competing Interests: Competing interests: None declared.

Primary hepatic leiomyosarcoma generally occurs only in adult patients or pediatric patients with immunodeficiency. We report here the FDG PET/CT findings of primary hepatic leiomyosarcoma in a 10-year-old immunocompetent girl.

About one third of focal thyroid uptakes in a fluorodeoxyglucose (FDG) positron emission tomography/computerized tomography (PET/CT) study are malignant, the most frequent histological type being papillary carcinoma. Metastases to the thyroid account for approximately 7.5% of thyroid malignancies and come mainly from kidney, lung, head and neck, and breast cancers. We report the case of a 64-year-old woman presenting a fast growing thyroid nodule whose primitive or metastatic origin was not obvious, for which 18 F-FDG PET/CT helped in the diagnostic process and in the later management of the patient. Histopathologic findings finally revealed a metastasis of uterine leiomyosarcoma., (Copyright © 2016 Elsevier España, S.L.U. y SEMNIM. All rights reserved.)