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Rationale: Primary leiomyosarcoma of the bone was firstly reported by Evans and Sanerkin in 1965, whereas primary leiomyosarcoma of the vertebra is extremely rare. Because of the rarity of primary vertebral leiomyosarcoma, the diagnosis, treatment, and clinical outcome still remain controversial. Here we report a special case of primary leiomyosarcoma in the thoracic vertebra., Patient Concerns: A 47-year-old female patient was admitted to our institution with the chief complaint of persistent back pain for 4 weeks. She had no symptoms of numbness, weakness, pain, and abnormal sensation in her extremities., Diagnoses: Neurological examination on admission revealed no obvious abnormality. Magnetic resonance imaging showed a bone destruction of the T11 vertebral body and the right pedicle. Therefore, primary vertebral leiomyosarcoma was suspected. Pathological hematoxylin and eosin staining of the resected tumor revealed a diagnosis of polymorphic undifferentiated sarcoma. Furthermore, to identify the subtype of this sarcoma, the immunohistochemical staining of the tumor was performed with each of the various antibodies and the results are epithelial membrane antigen (-), H-caldesmon (-), desmin (+), smooth muscle actin (+), S-100 (-), myogenin (-), pan-keratin (-), and Ki-67 (positive rate: 20%). Finally, the patient was diagnosed as primary vertebral leiomyosarcoma., Interventions: the anterior corpectomy and autogenous iliac bone graft with instrumentation combined with the posterior spinal canal decompression and fusion with the pedicle screw system were performed through an anterior-posterior union approach., Outcomes: Neither clinical symptoms nor signs of tumor recurrence were detected within the follow-up of 6 months. In addition, 11 cases of the primary vertebral leiomyosarcoma reported in the literature were reviewed and summarized., Lessons: Exclusion of metastatic leiomyosarcoma by various imaging modalities and histopathological examinations, especially the immunohistochemical staining with various antibodies against the epithelial and mesenchymal cell markers, are critical for establishing the correct diagnosis of the primary vertebral leiomyosarcoma. Surgical resection, especially the total en bloc spondylectomy, is the main treatment option with a good outcome, albeit with a limited follow-up duration.

Leiomyosarcoma is a rare condition so there are relatively few and small case series and no prospective studies to provide clear guidelines regarding management. We report on a case that presents some particularities that further underline diagnostic and treatment difficulties posed by the affliction of such a rare tumor. This is the case of a 43-year-old woman who had a large tumor arising from the uterus, with a spectacular growth rate over a short period. The patient, with congenital spastic tetraparesis and hydrocephalus, came for belly enlargement with rapid increase in size over the previous two months. Physical exam and ultrasound lead to the conclusion of a large abdominal mass. A computed tomography scan showed a mass arising from the pelvis and a large amount of ascites. An exploratory laparotomy was performed and the histopathology report confirmed the diagnosis of uterine leiomyosarcoma. Leiomyosarcoma poses challenging problems regarding histological grading and, due to its rate of growth, real surgical difficulties. Final diagnosis is done by histopathological examination after surgical excision. Frequently, it is late diagnosed when complete removal of the tumor is often impossible.

Metastasis of a nonvisceral leiomyosarcoma to the heart is rare. We present the case of a man with a history of an upper extremity cancerous lesion that was completely resected with appropriate surveillance monitoring, which then metastasized to the heart 14 years later, presenting as superior vena cava syndrome. Full evaluation found no other metastatic lesions, including no residual sarcoma at the former primary site. We include transthoracic echocardiography and computed tomography images of unusual presentation of the large mass extending from the caudal superior vena cava to the right atrium and into the right ventricle across the tricuspid valve.

Primary leiomyosarcoma of the orbit is extremely rare. Here we report the case of a 32-year-old woman who presented with proptosis and diplopia of the left eye. MRI examination showed a solid mass in the left orbit with invasion to adjacent tissue, and the lesion had intense FDG uptake with SUVmax of 18.7 on F-FDG PET/CT. Primary orbit leiomyosarcoma was diagnosed by surgery-pathology. F-FDG PET/CT has an advantage in excluding secondary or metastatic orbit malignancy.

Soft tissue sarcomas are very rare tumors, representing less than 1% of all cancers. Leiomyosarcomas are a rare group of them representing about 6% of soft tissue sarcomas and they involve smooth muscles. Less than 2% of all leiomyosarcomas involves large blood vessels. Leiomyosarcomas of vein tunica media are very rare (1/100,000 malignant cancers) and only 10% of these originate from the great saphenous vein. In this article, we report a clinical case that occurred in our institution and review all the literature available at now., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Non-gastrointestinal stromal tumor (GIST) soft tissue sarcomas (STSs) are a heterogeneous group of neoplasms whose classification and management continues to evolve with better understanding of their biologic behavior. The 2013 World Health Organization (WHO) has revised their classification based on new immunohistochemical and cytogenetic data. In this article, we will provide a brief overview of the revised WHO classification of soft tissue tumors, discuss in detail the radiology and management of the two most common adult non-GIST STS, namely liposarcoma and leiomyosarcoma, and review some of the emerging histology-driven targeted therapies in non-GIST STS, focusing on the role of the radiologist.

Uterine leiomyosarcoma (U-LMS) is the most frequent malignant gynecologic mesenchymal tumor, often develops distant metastases and has a dismal prognosis. In this study we aim to characterize the body sites and time to metastasis in women with U-LMS. We evaluated 130 U-LMSs with distant metastases including a series of patients diagnosed at 2 tertiary centers, as well as cases published in the literature, found using a PubMed query. Data collected included clinic-pathologic features, time to first metastasis, and survival. Survival analysis was performed using univariable and multivariable Cox regression model. The most frequent metastatic sites were: lung (67.7%), cranial/intracranial (16.2%), skin/soft tissues (15.3%), and bone (13.8%). Other sites included thyroid, salivary gland, heart, liver, pancreas, adrenal gland, bowel, and breast. Metastases were histologically identical to primary tumors. Median time to first metastasis was highly variable (median: 24 mo; range, 1 mo to 26 y). Lung and peritoneum were the earlier metastatic sites; 21.4% of patients with U-LMS limited to the pelvis develop metastasis >5 yr after diagnosis. Lung metastases significantly associated with other distant metastases. Regarding treatment, only resection of metastases significantly influenced postmetastasis survival in multivariable analysis (hazard ratio: 0.49, P=0.015). In conclusion, U-LMS display highly variable sites of distant metastases. Metastases in unusual locations are sometimes the first to be detected, and not uncommonly, single and prone to surgical resection. There is also a wide range of time intervals to first metastasis, highlighting the need of long-term follow-up, high level of suspicion, and appropriate diagnostic confirmation.

Although primary leiomyosarcoma of the kidney is extremely rare, it is the most common sarcoma of the kidney. Leiomyosarcoma with a large pleomorphic component is designated as pleomorphic leiomyosarcoma. The pleomorphic component is usually similar to undifferentiated high-grade pleomorphic sarcoma, although it variably expresses smooth muscle markers on immunohistochemistry. In the few reported cases of pleomorphic leiomyosarcoma of the kidney, cases with the pleomorphic component showing distinct nodularity similar to dedifferentiated leiomyosarcoma have not been described, to the best of our knowledge. Herein, we present a case of a 49-year-old woman with pleomorphic leiomyosarcoma in the kidney showing distinct nodularity of smooth muscle marker-expressing pleomorphic cells within a background of classic leiomyosarcoma. Along with the classification as a pleomorphic leiomyosarcoma, suggesting aggressive clinical behavior, the renal origin itself might also be a predictor of poor prognosis, as shown in a previous study. This case also involved concomitant distant metastases, already present during the initial detection of the renal tumor.

Introduction: The goal of this study was to assess the sensitivity of different preoperative diagnostic tools used in women with uterine leiomyosarcomas., Material and Methods: Retrospective cohort study of all women with verified uterine leiomyosarcoma in Norway in the period 2000 to 2012. Data were collected from the Cancer Registry of Norway and medical records., Results: There were 212 women diagnosed with uterine leiomyosarcoma in the 13-year period. Histopathological examinations by fractional curettage or endometrial biopsies verified malignancy in 55/142 (38.7%). MRI suggested malignancy in 45/55 (81%) of the examinations. CT evaluations indicated suspected malignancy in 64/107 women (59.8%). Biomarkers had low sensitivity for leiomyosarcoma, but suggested more advanced stage disease when high values were detected. Stage IV disease was present in 53.1% versus 25.5% (p = 0.01) of women with CA 125 values above 35 kU/L, compared with women with normal CA 125 values. In 115/212 (54.2%), leiomyosarcoma was only diagnosed postoperatively by histopathological examination of the removed specimen., Conclusions: Preoperative diagnostic modalities appear to have low sensitivity for differentiating leiomyosarcoma from fibroids. In Norway, approximately 54% of uterine leiomyosarcoma are unidentified before surgery. MRI evaluation was the imaging modality with the greatest sensitivity in identifying leiomyosarcoma preoperatively., (© 2016 Nordic Federation of Societies of Obstetrics and Gynecology.)

Chest radiography indicated a well-defined rounded mass at the left lung hilum in a 77-year-old former smoker. Chest computed tomography revealed a longitudinal saccular enlargement of the left pulmonary artery with surrounding soft tissue opacity. Resection of the left lower lobe with segment 1 + 2c was carried out to completely remove the dilated pulmonary artery. The resected specimen revealed obvious dilatation of the interlobar pulmonary artery and its branches. A small yellowish well-demarcated myxoid tumor was contained in this lesion, which was diagnosed as a primary pulmonary artery intramural leiomyosarcoma., (© The Author(s) 2016.)

Uterine leiomyosarcoma is an uncommon pathology, predominantly found in aged population. Patients with metastatic disease have poor survival and therapy mainly consists of palliative systemic chemotherapy. However, more aggressive strategies such as radiofrequency ablation (RFA) may benefit patients with limited secondary disease. RFA is considered a simple and safe modality for treatment of hepatic lesions. The benefits related to RFA include low morbidity, short hospital stay and the possibility to repeat the procedure when necessary due to recurrences. However, minor and major complications related to mechanical and thermal damage may occur, especially in cases of tumors adjacent to extrahepatic organs and those at subcapsular position. This case report shows a successful RFA of two hepatic subcapsular leiomyosarcoma metastases neighbouring the gallbladder, without a safe cleavage plane from it. Combined hydrodissection, percutaneous cholecystostomy and continuous irrigation were performed as effective techniques to prevent thermal injury. Clinical and radiological follow up demonstrates no local complication.

We present the case of a 76-year-old man with a 2-month history of mildly tender swelling in the right calf for which he had an initial excision and then a wide local excision followed by a split skin graft because the initial histopathology confirmed that there was a Grade I leiomyosarcoma of great saphenous vein. A simple lump in the lower limb could be a malignant vascular tumour and should always be considered in the list of differential diagnosis of a lump in the lower limb., Competing Interests: Conflicts of Interest: None declared., (2016 BMJ Publishing Group Ltd.)

Rationale and Objectives: This study aimed to determine whether uterine leiomyoma can be distinguished from uterine leiomyosarcoma on ultrasound (US), computed tomography (CT), and/or magnetic resonance imaging (MRI) without diffusion-weighted imaging., Materials and Methods: Institutional review board approval was obtained and informed consent was waived for this Health Insurance Portability and Accountability Act-compliant retrospective case-control diagnostic accuracy study. All subjects with resected uterine leiomyosarcoma diagnosed over a 17-year period (1998-2014) at a single institution for whom pre-resection US (n = 10), CT (n = 11), or MRI (n = 7) was available were matched by tumor size and imaging modality with 28 subjects with resected uterine leiomyoma. Six blinded radiologists (three attendings, three residents) assigned 5-point Likert scores for the following features: (1) margins, (2) necrosis, (3) hemorrhage, (4) vascularity, (5) calcifications, (6) heterogeneity, and (7) likelihood of malignancy (primary end point). Mean suspicion scores were calculated and receiver operating characteristic curves were generated. The ability of individual morphologic features to predict malignancy was assessed with logistic regression., Results: Mean suspicion scores were 2.5 ± 1.2 (attendings) and 2.4 ± 1.3 (residents) for leiomyoma, and 2.7 ± 1.3 (attendings) and 2.7 ± 1.4 (residents) for leiomyosarcoma. The areas under the receiver operating characteristic curves (range: 0.330-0.685) were not significantly different from chance, either overall (P = .36-.88) or by any modality (P = .28-.96), for any reader. Reader experience had no effect on diagnostic accuracy. No morphologic parameter was significantly predictive of malignancy (P = .10-.97)., Conclusions: Uterine leiomyoma cannot be differentiated accurately from leiomyosarcoma on US, CT, or MRI without diffusion-weighted imaging., (Copyright © 2016 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

We report here on a long-surviving patient with a vascular leiomyosarcoma arising in the right common carotid artery, and some possible reasons for the long-term survival. The patient was a 40-year-old woman who was diagnosed with a right common carotid aneurysm and underwent aneurysmectomy with graft replacement. Two years and nine months after her surgery, she developed a cerebral infarction and left hemiparesis due to graft occlusion, but later achieved functional recovery through physical rehabilitation. She has been alive for 20 years since her surgery., (© The Author(s) 2016.)

Retroperitoneal sarcomas are rare malignant neoplasms that are often diagnosed late because of their typical nonspecific symptomatology. Radical surgical resection, if possible given the local anatomical conditions, is the most important treatment modality. However, even if the surgery is feasible and successful, the prognosis of retroperitoneal sarcoma is not good. The most frequent type of recurrence is local recurrence, and radical surgical resection remains the most important treatment modality even in such cases.Our study presents a case report of a patient with a retroperitoneal leiomyosarcoma that was radically (R0) removed. Two years later a local recurrence developed, which was eventually diagnosed by MRI. Again, radical (R0) surgical resection was approached (because of tumor invasion into inferior caval vein, the vein had to be partially resected as well and substituted with a vascular prosthesis).The report describes a relatively typical case of retroperitoneal sarcoma, while pointing out the importance of timely diagnosis (which is definitely not easy to achieve) and especially of radical surgical treatment.Key words: retroperitoneal sarcoma recurrence surgery.

Background: Kidney transplant (KT) recipients have a higher incidence of malignancy than the general population. Smooth muscle tumors (SMT), including leiomyosarcoma, are rare in kidney transplant recipients, and most cases are associated with Epstein-Barr virus (EBV) infection., Case Report: A 57-year-old man received a deceased donor kidney transplant at the age of 53 years, with 5 human leukocyte antigen (HLA) mismatches. Before the transplantation, the patient was IgG positive for EBV viral capsid antigen (VCA), negative for IgM EBV VCA, and also negative for IgG EBV nuclear antigen (EBNA), suggesting a prior EBV infection. He received immunosuppressive induction with basiliximab, and maintenance with tacrolimus, mycophenolate mofetil, and prednisolone. Two years after transplantation, he had an acute cellular rejection episode treated with methylprednisolone. An increased graft size was found 4 years after transplantation. A computed tomographic scan showed 3 solid tumors involving the renal graft with extension to the perinephric fat; no secondary localizations were found. A nephrectomy of the graft was performed. The histologic diagnosis was a high-grade leiomyosarcoma. In situ hybridization for EBV was negative. Nine months after nephrectomy, local recurrence was diagnosed. The surgical approach was unsuccessful, and the patient died after a brief period., Conclusion: Kidney leiomyosarcoma is a very rare clinical condition. Most of these neoplasms that arise in transplanted recipients are associated with EBV in tumor tissue. Only one case of renal graft leiomyosarcoma without EBV RNA in the tumor has been previously reported., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Uterine leiomyosarcoma (ULMS) is an aggressive tumor associated with high rates of progression, recurrence, and mortality. Pazopanib is the only approved molecular targeted drug for advanced soft tissue sarcoma, and it has been proven to prolong progression-free survival relative to placebo. We herein report a case of ULMS with multiple lung metastases treated with pazopanib, which led to sustained disease control for 44 weeks. A 53-year-old woman was referred to our hospital due to massive uterine bleeding from a uterine corpus tumor mass. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed as emergency surgery. The final histopathological diagnosis was uterine leiomyosarcoma, and computed tomography revealed multiple lung metastases. After chemotherapy with 17 cycles of gemcitabine and docetaxel and two cycles of doxorubicin, the lung metastases had increased in size and new lesions had appeared. Pazopanib administration at 800 mg/day was started as third-line therapy. Ten weeks later, the dose of pazopanib was reduced to 600 mg/day because of hepatic impairment and hypertension. However, lung metastases of ULMS were stabilized by pazopanib administration for about 44 weeks without a decline in the patient's quality of life. After 44 weeks of therapy, pazopanib administration was discontinued because of progressive disease and worsening of the patient's respiratory status. Pazopanib is an oral multityrosine kinase inhibitor of vascular endothelial growth factor receptor-1, -2, and -3; platelet-derived growth factor-α and -β; and c-Kit receptor. The role of pazopanib may be clinically significant in the treatment of advanced ULMS.

Aims: To evaluate the use of computed tomography image-guided percutaneous cryoablation for recurrent retroperitoneal soft tissue sarcomas (RPSs)., Results: Adverse events were limited to grades 1 and 2, included fever (n = 19), local pain (n = 11), emesis (n = 10), frostbite (n = 6), and nerve injury (n = 1). Fever was more frequent in the large tumor group (15.8%) than in small tumor group (1.9%) (P = 0.008). Median PFS and OS were 37.0 ± 7.7 months (range, 4-39 months) and 43.0 ± 5.9 months (range, 6-54 months), respectively. PFS and OS were significantly longer in the small tumor group than in the large tumor group (P = 0.011 and P = 0.015, respectively), but the response rate (82.7% vs. 72.8%, P = 0.240) did not differ significantly. On univariate analysis, tumor size, tumor invasion grade, and distant metastasis were significant prognostic factors for PFS and OS. On multivariate analysis, a tumor size ≥10 cm was an independent negative prognostic factor for PFS and OS after cryoablation (HR: 3.98, 95% CI: 1.27-12.50, P = 0.018 and HR: 4.33, 95% CI: 1.41-13.26, P = 0.010, respectively)., Materials and Methods: Data from 72 patients with recurrent RPSs who underwent percutaneous cryoablation were reviewed retrospectively. The prognostic factors for progression-free survival (PFS), overall survival (OS), and efficacy based on mRECIST criteria were analysis. Adverse events were compared according to tumor size (<10 and ≥10 cm)., Conclusion: Minimally invasive percutaneous cryoablation was safe and efficacious for recurrent RPSs., Competing Interests: None of the authors have any conflicts of interest to declare.

Leiomyosarcoma is an aggressive soft tissue sarcoma derived from smooth muscle cells. Of all soft tissue sarcomas, approximately 5-10% are leiomyosarcomas. Vascular leiomyosarcoma constitutes about 2% of all leiomyosarcomas and involves veins 5 times more than arteries. When they arise from a major blood vessel, symptoms of vascular compromise or leg edema may be present. Because they are rare, definite diagnosis is often delayed. We present the case of an 88-year-old man who was admitted to our department with acute limb ischemia stage 4 according to Rutherford's criteria. His personal medical history included arterial hypertension under medication with nonspecific conduction disturbances showed in the electrocardiography. The duplex scan revealed the presence of thrombotic material to the distal superficial femoral and popliteal artery, whereas the presence of popliteal artery aneurysm was excluded. After the initial diagnostic approach, he underwent 2 unsuccessful embolectomy procedures. During the amputation procedure, a 6-cm mass was palpated in the popliteal fossa, and it was excised. The immunohistopathologic study revealed a grade 3 according to the French Federation Nationale des Centers de Lutte Contre le Cancer classification leiomyosarcoma. The patient was discharged 10 days later and referred to an oncologic center. He returned 6 months later with edema of the amputated limb and inguinal lymphadenopathy. Specimen of the inguinal lymph nodes was sent for histopathologic examination, which indicated the recurrence of the disease. Leiomyosarcomas should be taken into consideration in elderly patients presenting with acute limb ischemia., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Due to their high prevalence and related morbidity, uterine myomas constitute a group of gynecological pathologies largely studied in all clinical, diagnostic, and therapeutic aspects. They have been widely evaluated with a large series of imaging techniques. In fact, ultrasound (also saline infusion sonohysterography) and magnetic resonance imaging (MRI) are considered the optimal methods to assess uterine fibroids in terms of number, volume, echostructure, location, relation with endometrial cavity and uterine layers, vascularization, and differential diagnosis with other benign (adenomyosis) and malignant myometrial pathologies. Nevertheless, further studies are required to fill some gaps such as the absence of a common and sharable sonographic terminology and methodology to scan the myometrium, as well as imaging parameters for differentiation of typical myomas from smooth tumors of unknown malignant potential (STUMP) and leiomyosarcomas., (Copyright © 2016. Published by Elsevier Ltd.)

We report a case of leiomyosarcoma of the thoracic spine. Primary leiomyosarcoma is a malignant connective tissue tumor originating from smooth muscle cells. Leiomyosarcoma frequently occurs in the uterus, retroperitoneal space, gastrointestinal tract, and deep soft tissues; primary leiomyosarcoma of the bone is rare. The MR imaging including intravoxel incoherent motion (IVIM) imaging findings of the current case indicated a low diffusion coefficient and high blood flow, which were in concurrence with high cell density on histology and increased vascularity by angiography. Although some benign tumors such as osteoblastoma and giant cell tumor would show similar findings on IVIM imaging, these additional imaging features may narrow the differential diagnosis of spinal tumors.

Myomas represent a large part of benign gynecological pathology, widely spread in fertile female population. First step to diagnose fibroids is ultrasound (US) that can be 2-dimensional (2D), 3-dimensional (3D), Color Doppler (CD) and sonohysterography (SHG). This review develops according to MUSA's sonographic features (Morphological Uterus Sonographic Assessment). One of the main topic of interest for ultrasonographer today is endo/myometrial junctional zone (JZ), because it may be useful to discern a diagnosis of myoma and adenomyosis. Another important aspect of ultrasound is the analysis of vascularization in front of a uterine lesion. Indeed, vascular pattern can be used to make differential diagnosis between myoma-adenomyosis and leiomyosarcomas. Myomas should be described accurately according to sonographic guidelines. Sonographic features correlated with symptoms should guide an appropriate surgical or medical treatment.

Due to overlapping clinical and radiological features, the differentiation between osteomyelitis and bone tumor can be challenging. A 48-year-old lady presented with intermittent left foot pain for a few months. Plain radiographs showed an osteolytic lesion affecting the proximal diaphysis of the left fourth metatarsal bone, with thinning and irregularities of the cortex and focal periosteal reaction. Ultrasonography revealed diffuse subcutaneous edema in the dorsum of the left foot, cortical irregularities along the mid-shaft of the left fourth metatarsal bone, and surrounding periosteal collection. Computed tomography showed medullary expansion along the shaft and base of the left fourth metatarsal bone with cortical irregularities and defects suggestive of cloaca, and focal mild periosteal new bone formation. The clinico-radiologic diagnosis was acute osteomyelitis with periosteal collection. During open biopsy, the finding of intramedullary fleshy tissue in the absence of significant inflammatory edema and purulent discharge, and subsequent negative culture result prompted a review of the histologic slides which was initially reported as benign fibroblastic tissue proliferation. Careful analysis of the histomorphology disclosed a spindle cell sarcoma for which ray amputation of the fourth and fifth metatarsal was performed. The final diagnosis was grade 1 leiomyosarcoma and the patient remained well 33 months after the operation., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Primary aortic neoplasms are rare. Aortic sarcoma arising after endovascular aneurysm repair (EVAR) is a scarce subset of primary aortic malignancies, reports of which are infrequent in the published literature. The diagnosis of aortic sarcoma is challenging due to its non-specific clinical presentation, and the prognosis is poor due to delayed diagnosis, rapid proliferation, and propensity for metastasis. Post-EVAR, aortic sarcomas may mimic other more common aortic processes on surveillance imaging. Radiologists are rarely knowledgeable about this rare entity for which multimodality imaging and awareness are invaluable in early diagnosis. A series of three pathologically confirmed cases are presented to display the multimodality imaging features and clinical presentations of aortic sarcoma arising after EVAR.

The combination of high heterogeneity, both intratumoral and intertumoral, with their rarity has made diagnosis, prognosis of high-grade sarcomas difficult. There is an urgent need for more objective molecular biomarkers, to differentiate between the many different subtypes, and to also provide new treatment targets. Mass spectrometry imaging (MSI) has amply demonstrated its ability to identify potential new markers for patient diagnosis, survival, metastasis and response to therapy in cancer research. In this study, we investigated the ability of MALDI-MSI of proteins to distinguish between high-grade osteosarcoma (OS), leiomyosarcoma (LMS), myxofibrosarcoma (MFS) and undifferentiated pleomorphic sarcoma (UPS) (Ntotal = 53). We also investigated if there are individual proteins or protein signatures that are statistically associated with patient survival. Twenty diagnostic protein signals were found characteristic for specific tumors (p ≤ 0.05), amongst them acyl-CoA-binding protein (m/z 11 162), macrophage migration inhibitory factor (m/z 12 350), thioredoxin (m/z 11 608) and galectin-1 (m/z 14 633) were assigned. Another nine protein signals were found to be associated with overall survival (p ≤ 0.05), including proteasome activator complex subunit 1 (m/z 9753), indicative for non-OS patients with poor survival; and two histone H4 variants (m/z 11 314 and 11 355), indicative of poor survival for LMS patients., (© 2016 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.)

Uterine sarcomas are rare cancers characterized by clinical and histological polymorphism. They are malignant tumors of poor prognosis. Inaugural manifestation of acute abdomen with hemo-pneumoperitoneum and spontaneous uterine rupture remains exceptional. The authors report the case of a uterine leiomyosarcoma manifesting itself as an inaugural generalized acute peritonitis in a 43 year old woman. Uterine rupture due to malignant tumor was suspected during urgent preoperative imaging (ultrasound and CT scan) and confirmed histologically.

Background: Leiomyosarcoma of the inferior vena cava (IVC) is an exceedingly rare smooth muscle sarcoma. Approximately 300 cases have been described in the literature, and further research is needed to understand the disease and guide its management. Surgery remains the only potential curative measure., Methods: A retrospective chart review of patients who underwent surgical resection of IVC leiomyosarcoma at our institution over the past 3 years was performed. The patients were identified using a prospectively maintained database., Results: Three patients with leiomyosarcoma of the infrahepatic IVC underwent radical resection carried out by a team of surgical oncologists and vascular surgeons. There were 2 males (66.7%) and 1 female (33.3%). Mean age at diagnosis was 60.3 years (range 43-78). Mean tumor size was 12.2 cm (range 5.6-22). The mean operative time was 320 min (range 180-421), mean estimated blood loss was 1,300 mL (100-2,000) mL, and average length of stay 8.67 days (6-12). All patients achieved grossly negative margins (R1 or R0 resections) and are alive with a mean overall survival of 21 months (range 12-30). Patient 1 was a 60-year-old man who presented with metachronous skin leiomyosarcomas at 2 different sites. He underwent PET/CT scan that revealed an IVC mass. Resection of the middle segment of the IVC and right kidney was performed with reconstruction with polytetrafluorethylene (PTFE) graft. Patient 2 was a 78-year-old man with an incidentally found a 9-cm IVC tumor. Resection of the tumor was performed, and no reconstruction was needed since the tumor had a completely extraluminal growth pattern. Patient 3 was a 43-year-old woman who presented with abdominal pain. Her work-up showed a 15-cm IVC mass. She underwent resection of the middle segment of the IVC, right nephrectomy, and cholecystectomy with reconstruction of the IVC with PTFE graft., Conclusions: Surgical resection is the mainstay of treatment in patients with leiomyosarcoma of the IVC. A collaborative approach involving surgical oncologists and vascular surgeons ensures adequate resection with functional reconstruction to achieve the best patient outcomes., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Leiomyosarcoma of the vascular origin is a rare malignant tumor. It originates from the smooth muscle cells of the media with intra- or extraluminal growth, and in most cases it arises in the inferior vena cava. The diagnosis is often delayed because the clinical symptoms of this disease are often nonspecific. Accurate diagnosis of inferior vena cava leiomyosarcoma (IVCLMS) needs histologic confirmation. We report a case of IVCLMS histologically confirmed by aspiration biopsy with a catheter during digital subtraction angiography presenting with pulmonary emboli in a 65-year-old man., (© The Author(s) 2016.)

A 69-year-old man who presented with abdominal discomfort was, on examination, found to have a palpable abdominal mass. Contrast-enhanced CT showed a mass arising from the inferior vena cava, which biopsy confirmed to be a leiomyosarcoma. One month after chemoradiotherapy, CT demonstrated a new 15 mm solitary central right liver metastasis. Microwave ablation (MWA) of the metastasis was performed using an Acculis Sulis V system (Angiodynamics, USA) at a power of 140 Watts for 4 min, with no immediate complications. After 1 month, MRI with gadolinium was performed to assess the liver ablation zone. The MRI demonstrated thrombosis of a right inferior hepatic vein branch leading to the ablation zone and extension of the ablation zone 1 cm into the tissue around the thrombosed vessel., (2016 BMJ Publishing Group Ltd.)

Background Context: Recent advances in image guidance and stereotactic body radiotherapy (SBRT) have resulted in unprecedented local control for spinal metastases of all histologies. However, little is known about early imaging biomarkers of local control., Purpose: This study aimed to identify early magnetic resonance imaging (MRI) biomarkers to predict local control after SBRT for patients with sarcoma spine metastases., Study Design/setting: This study used a retrospective case series at a large tertiary cancer center., Patient Sample: From 2011 to 2014, 9 consecutive patients with 12 metastatic sarcoma lesions to the spine were treated with SBRT and underwent evaluation with dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) both pre- and post-SBRT., Outcome Measure: Changes in perfusion metrics, including the wash-in rate constant (Ktrans), plasma volume (Vp), composite multiparametric magnetic resonance imaging (mpMRI) score, bi-dimensional tumor size, and a graded response assessment were performed and correlated to local control., Methods: All measurements were independent and blinded by two neuroradiologists. R2 statistics were performed to document correlation, and two-tailed t tests were used to compare groups. p<.05 was deemed statistically significant., Results: The median time from SBRT until posttreatment MRI was 57 days. Local failure developed in one lesion (8.3%) 10 months after SBRT. The Vp mean, Ktrans mean, Vp max, and Ktrans max were significantly decreased post-SBRT as compared with pre-SBRT (58.7%, 63.2%, 59.0%, and 55.2%; all p-values <.05). Bi-dimensional tumor measurements demonstrated an average increase in size across the cohort, and 50%, 25%, and 25% of the treated lesions demonstrated features of "worsening," "no change," or "improvement," respectively, by both radiologists' graded impressions. There was good inter-reader reliability for both size and subjective disease response scores (R2=0.84). The mpMRI score had 100% accuracy in predicting local control at time of last follow-up. There was no apparent correlation with size changes compared with the mpMRI score change post-SBRT (R2=0.026)., Conclusions: We report the first analysis on the utility of DCE-MRI for metastatic sarcoma spine metastases treated with SBRT. We demonstrate that early assessment at 2 months post-SBRT using size and subjective neuroradiology impressions is insufficient to judge ultimate disease progression, and that a combination of perfusion parameters provides excellent correlation to local control., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Leiomyosarcoma of the uterus is an extremely rare but highly aggressive tumor that accounts for only 1-2 % of uterine malignancies, and is usually associated with a dismal outcome., Case Presentation: The authors present an unusual case of pedunculated subserosal leiomyosarcoma of the uterus mimicking ovarian carcinoma. A 57-year-old postmenopausal woman presented with progressive low abdominal pain and urinary frequency. Pelvic magnetic resonance imaging revealed a large adnexal mass with carcinomatosis peritonei. Laboratory examination revealed an elevated serum CA-125 level (398.4 U/ml). The patient underwent exploratory laparotomy under suspicion of ovarian malignancy. Frozen section indicated malignancy originating from the uterus, and thus, total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic and para-aortic lymph node dissection, and mass excision were performed. Subsequent histopathologic examination resulted in a final diagnosis of leiomyosarcoma of the uterus. The patient's postoperative course was uneventful, and gemcitabine and docetaxel adjuvant chemotherapy was administered., Conclusion: The authors report an unusual case of pedunculated subserosal leiomyosarcoma of the uterus mimicking ovarian carcinoma.

The authors present a review of pathogenesis, diagnosis and various approaches to treatment for an extremely rare tumor - primary hepatic leiomyosarcoma. This tumor is dif- ficult to be diagnosed by imaging examinations. The prevalent method of treatment is a radical resection (RO surgery). Ac- cording to literature the median survival of patients with LMSL is about one year with DFS after treatment about 10 months. Also the authors describe a case report of the successful cure of middle-aged female with primary hepatic leiomyosarcoma and Hodgkin's lymphoma in the anamnesis. She has undergone 6 cycles of neoadjuvant chemotherapy with Ifosfamide and Doxorubicin. The tumour response was estimated as partial. Then patient has undergone radical surgery in the volume of extended left hemihepatectomy with LND. Follow-up exami- nation 38 months after treatment revealed no progression of the disease.

Stereotactic radiosurgery and percutaneous radiofrequency ablation are emerging therapies for pain palliation and local control of spinal metastases. However, the post-treatment imaging findings are not well characterized and the risk of long-term complications is unknown. We present the case of a 46-year-old woman with delayed vertebral body collapse after stereotactic radiosurgery and radiofrequency ablation of a painful lumbar metastasis. Histopathologic-MRI correlation confirmed osteonecrosis as the underlying etiology and demonstrated that treatment-induced vascular fibrosis and tumor progression can have identical imaging appearances., (© The Author(s) 2015.)

We present the case of 51-year-old woman with a primary breast leiomyosarcoma. On the postoperative screening after breast cancer surgery, a 4-mm-sized mass assessed as BI-RADS category 3 was detected on her contralateral breast. We recommended follow-up and observation. However, the lesion increased in size and became palpable. It was excised and diagnosed as primary leiomyosarcoma. We review the literature on imaging findings and management of breast leiomyosarcoma, and discuss the differential diagnoses in breast imaging., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Both benign and malignant tumors and tumorlike conditions can arise from the nonepididymal extratesticular tissues. Benign tumors are far more common than malignant tumors, with lipoma being the most common lesion found at this site. Several imaging features can help narrow the differential diagnosis of these lesions, including the presence of fat and imaging features suggestive of fibrous tissue. Lesions that contain fat represent lipoma, liposarcoma, or angiomyofibroblastoma (AMF)-like tumor. If enhancing soft tissue is present in conjunction with fat, the differential diagnosis narrows further to just liposarcoma and AMF-like tumor. Lesions that display magnetic resonance imaging characteristics compatible with fibrous tissue are likely to be fibrous pseudotumor. However, many of the lesions in this location have overlapping imaging findings, and surgical excision is most often necessary for accurate diagnosis. The ability to narrow the differential diagnosis with imaging, however, is helpful for the clinician for both treatment planning and patient counseling., ((©)RSNA, 2015.)

Laryngeal leiomyosarcoma is a rare mesenchymal malignancy of the head and neck region. The author presents a case of an atypical laryngeal leiomyosarcoma that featured a large pedunculated lesion without mucosal invasion. This feature has not been previously described in the English-language literature. The diagnosis, treatment, and outcome are also discussed.