Distinguishing cardiac myxomas from cardiac thrombi by a radiomics signature based on cardiovascular contrast-enhanced computed tomography images.

BACKGROUND: Cardiac myxomas (CMs) and thrombi are associated with high morbidity and mortality. These two conditions need totally different treatments. However, they are difficult to distinguish using naked eye. In clinical, misdiagnoses occur now and then. This study aimed to compare the characteristics of CMs and cardiac thrombi and investigate the value of a radiomics signature in distinguishing CMs from cardiac thrombi, based on cardiovascular contrast-enhanced computed tomography (CECT) images. METHODS: A total of 109 patients who had CMs (n = 59) and cardiac thrombi (n = 50) were enrolled in this retrospective study from 2009 to 2019. First, the lesion characteristics of cardiovascular CECT images were documented and compared by two radiologists. Then all patients were randomly allotted to either a primary group or a validation group according to a 7:3 ratio. Univariate analysis and the least absolute shrinkage and selection operator were used to select robust features. The best radiomics signature was constructed and validated using multivariate logistic regression. An independent clinical model was created for comparison. RESULTS: The best radiomics signature was developed using eight selected radiomics. The classification accuracies of the radiomics signature were 90.8% and 90.9%, and the area under the receiver operating characteristic curves were 0.969 and 0.926 in the training and testing cohorts, respectively. Cardiovascular CECT images showed that the two diseases had significant differences in location, surface, Hydrothorax, pericardial effusion and heart enlargement. The naked eye findings were used to create the clinical model. All metrics of the radiomics signature were higher than those of clinical model. CONCLUSIONS: Compared with clinical model, the radiomics signature based on cardiovascular CECT performed better in differentiating CMs and thrombi, suggesting that it could help improving the diagnostic efficiency.

Aggressive Angiomyxoma in an 11-Year-Old Boy - Diagnostic and Therapeutic Dilemmas: An Unusual Case Report and Review of the Literature.

Aggressive angiomyxoma (AAM) is a rare tumor with a high risk of local recurrence. Scrotal AAM mimics common pediatric pathologies including hernia or hydrocele. We present 11-year-old boy who underwent macroscopically radical excision of right scrotal AAM. The patient has been already followed up for 29 months utilizing US every 6 months and MRI every 2 years. Residual scrotal mass has been visualized in MRI 3 months after surgery however no further growth was reported. Long term follow up with reliable local imaging is mandatory.

The Biology of Myxofibrosarcoma: State of the Art and Future Perspectives.

BACKGROUND: Myxofibrosarcoma (MFS) is among the most highly complex sarcoma types. Molecular cytogenetic studies have identified a high level of genomic complexity. SUMMARY: This review provides an update of the current research related to MFS, with particular emphasis on emerging mechanisms of tumorigenesis and their potential therapeutic impact. Many novel possible molecular markers have been identified, not only for prognostication in MFS, but also to serve as possible therapeutic targets, and thereby improve clinical outcomes. However, the molecular pathogenesis of MFS remains incompletely understood. Key Messages: Patients suffering from advanced MFS might benefit from expanded molecular evaluation in order to detect specific expression profiles and identify drug-able targets. Moreover, immunotherapy represents an intriguingly perspective due to the presence of "T-cell inflamed" tumor microenvironment.

Reconstruction of Mandibular Defects With Bone Marrow-Derived Stem Cells in Odontogenic Myxoma.

Odontogenic myxoma is a benign tumor, mostly located in the mandible. It shows locally aggressive behavior and requires surgical removal. Common treatment options for reconstructing the bone defects are immediate or delayed autologous bone graft or free flap. In this article, the authors present the successful reconstruction with autogenous bone graft and autologous human bone marrow mesenchymal stem, followed by distraction osteogenesis, dental implant placement and prosthodontic restoration in the mandibular defect.

Neuroimaging characteristics and long-term prognosis of myxoma-related intracranial diseases.

PURPOSE: Myxoma-related intracranial diseases were rarely documented in history. The main purpose of our study is to provide a more comprehensive and detailed understanding of the pathogenesis, imaging features, surgical procedures and pathology of such patients through long-term follow-up. METHODS: From March 2012 to July 2018, baseline information that included neuroimaging and neuropathology data from 12 cardiac myxoma patients with neurological symptoms were retrospectively analysed, and the treatment options were discussed. Nine patients underwent long-term postoperative follow-up. RESULTS: Twelve cardiac myxoma patients with neurological symptoms were identified, and among them, 10 patients were postoperative patients who had undergone excision of cardiac myxoma, 5 patients had received craniotomy, and the others had received conservative treatment. Positive neuroimaging findings were found in all patients, including cerebral infarction (12/12, 100%), multiple intracranial aneurysms (8/12, 67%), and extravascular metastasis (6/12, 50%). After a long-term average follow-up of 27 months, an increased number of metastatic lesions or an enlargement of the intracranial aneurysms was found in 4 patients. CONCLUSIONS: Neuroimaging findings of myxoma-related intracranial lesions were diversed and usually presented as multiple cerebral infarction, aneurysm formation, focal intracranial haemorrhage and space-occupying lesions. Progress is over a long period of time after primary tumour resection. It is necessary for patients to be regularly examined within 2 years after cardiac myxoma resection using MRI+CTA/MRA/DSA in order to be ruled out. Stable and effective chemotherapy drugs are urgently needed.

Bilateral acute lower limb ischemia secondary to complete embolization of cardiac myxoma.

Cardiac myxomas are the most common benign cardiac tumors in adults that can present with peripheral embolization. Complete detachments of myxomas are rare and tend to cause aortoiliac embolism. We report a case of a middle-aged woman with bilateral popliteal artery and segmental renal artery embolisms secondary to a completely detached cardiac myxoma. This case highlights cardiac myxomas as an important cause of acute limb ischemia and that it is not excluded by a normal echocardiogram result.

Somatic PRKAR1A mutation in sporadic atrial myxoma with cerebral parenchymal metastases: a case report.

BACKGROUND: Atrial myxomas are generally considered benign neoplasms. The majority of tumors are sporadic and less than 10% are associated with an autosomal dominant condition known as the Carney complex, which is most often caused by germline mutation in the gene PRKAR1A. Whether this gene plays a role in the development of sporadic myxomas has been an area of debate, although recent studies have suggested that some fraction of sporadic tumors also carry mutations in PRKARIA. Extra-cardiac complications of atrial myxoma include dissemination of tumor to the brain; however, the dissemination of viable invasive tumor cells is exceedingly rare. CASE PRESENTATION: We present here a 48-year-old white woman who developed multiple intracranial hemorrhagic lesions secondary to tumor embolism that progressed to 'false' aneurysm formation and invasion through the vascular wall into brain parenchyma 7 months after resection of an atrial myxoma. Whole exome sequencing of her tumor revealed multiple mutations in PRKAR1A not found in her germline deoxyribonucleic acid (DNA), suggesting that the myxoma in this patient was sporadic. CONCLUSIONS: Our patient illustrates that mutations in PRKAR1A may be found in sporadic lesions. Whether the presence of this mutation affects the clinical behavior of sporadic tumors and increases risk for metastasis is not clear. Regardless, the protein kinase A pathway which is regulated by PRKAR1A represents a possible target for treatment in patients with metastatic cardiac myxomas harboring mutations in the PRKARIA gene.

Cerebral Aneurysms due to Cardiac Myxoma.

Cerebral aneurysms due to cardiac myxoma has been sporadically reported; however, the clinical features of this entity has not been fully elucidated. This meta-analysis was conducted to determine the reported clinical features and outcomes of this condition. It revealed a predominance of cardiac myxoma-related multiple fusiform aneurysms of the middle cerebral artery. The cerebral aneurysms developed at a mean of 53 months after cardiac myxoma resection. Left atrial myoxma was the most common causing cerebral aneurysm than a myxoma arising from any other sites of the heart. Most patients with myxoma-related cerebral aneurysms were under follow-up, conservative, or surgical treatments. Patients' outcomes were promising, with 69.4% (34/49) patients being stable. The overall mortality rate was 4.1% (2/49), and long-term survival seemed to be satisfactory. Cardiac myxoma resection cannot avoid the occurrence of cerebral aneurysm; therefore, cerebroimaging monitoring is recommended even after cardiac myxoma resection.

Odontogenic myxomas: what is the ideal treatment?

There is no consensus regarding the ideal treatment for odontogenic myxomas, an odontogenic mesenchymal neoplasm. Various authors have suggested en bloc resection due to a concern regarding inadequate clearance while others have suggested more conservative treatment. We present a case managed by buccal cortical resection and an iliac crest bone graft. The patient had no recurrence for over 7 years.

Diagnosis and treatment of oral focal mucinosis: a case series.

BACKGROUND: Oral focal mucinosis, the oral counterpart of cutaneous focal mucinosis, is a rare disease. As it has no characteristic clinical or radiological features, diagnosis is established by histopathological and immunohistological examination. We present three cases of oral focal mucinosis occurring in the retromolar (which is extremely rare) and gingival regions. CASE PRESENTATION: Case 1 involved a 26-year-old Japanese man with radiolucency in the right retromolar region on panoramic radiograph and computed tomography; no obvious protrusion was observed in the region. This finding was clinically diagnosed as a tumor of the retromolar region. Case 2 involved a 60-year-old Japanese woman. A tumor-like mass of tissue was identified on the buccal gingiva at the maxillary right canine and first premolar region. The lesion measured 7 × 6 mm and exhibited elastic hardness and healthy-colored mucosa. The lesion was diagnosed as an epulis. Case 3 involved a 47-year-old Japanese woman. A tumor-like mass of tissue was identified on the buccal gingiva at the maxillary right canine and first premolar region. The lesion measured 10 × 10 mm and exhibited elastic hardness and redness of the surface mucosa. This lesion was also diagnosed as an epulis. Resection was performed in all three cases, and the lesions were histopathologically diagnosed as oral focal mucinosis. Postoperative courses were uneventful and, thus far, there have been no recurrences. CONCLUSIONS: Although it is difficult to diagnose oral focal mucinosis based on clinical symptoms and imaging findings, the disease should be considered a possibility when diagnosing benign oral tumors. We believe that an emphasis on histopathologic study is essential to confirm the clinical suspicion.

Aggressive angiomyxoma of the prostate: A case report.

RATIONALE: Aggressive angiomyxoma (AAM) of the prostate should be considered as a differential diagnosis for prostatic tumor presenting with classical symptoms of benign prostatic hypertrophy. PATIENT CONCERNS: A 55-year-old man experienced persisting symptoms of prostatic enlargement associated with urinary frequency and urgency and nocturia. Computed tomography images showed low density in the enlarged prostate. DIAGNOSES: The diagnosis of AAM of the prostate was confirmed based on histopathological findings. INTERVENTIONS: The patient underwent transurethral resection of the prostate. OUTCOMES: The patient was enrolled into a watchful waiting protocol. His condition was fine without signs of recurrence on magnetic resonance imaging at the 8-month follow-up. LESSONS: AAM of the prostate should be considered a possible cause of urinary difficulty, including retention, although this may be extremely rare. A reliable diagnosis and complete tumor removal enabled optimal treatment and prevention of tumor recurrence.

A rare aetiology of stroke; myxomatous aneurysm caused by atrial myxoma.

Atrial myxoma is a rare cause of stroke. In this report we present the case of a 52-year-old female patient who went to hospital suffering from a headache. Her neurological examination was normal except for a positive Babinski sign on the left. In the superolateral of the right, a Sylvian fissure consistent with a thrombosed aneurysm was detected using computerised tomography (CT). Diffusion MRI showed an acute infarction on the right MCA area. Transthorasic Echocardiography and ECG were normal. A -16×4 mm-sized fusiform perpendicular aneurysm on the M2 segment Sylvian curve of right MCA and a -6×4 mm-sized dissecting aneurysm on P3 segment of the left posterior cerebral artery (PCA) were observed in cerebral angiography. Transesophageal echocardiography (TEE) demonsrated a large mass with a suspected size of 2×2×1.5 cm on the left atrium. The mass was resected and on the eighth day after the operation, she had a temporary vision loss and hyperintensity on the T1 sequence was interpreted as laminary necrosis suspected on Cranial MRI. In follow up, she was stable with 300mg acetylsalicylic acid treatment. The main treatment is surgical resection in stroke caused by atrial myxoma.

Craniofacial disorders.

The complexity of the craniofacial patient mandates the cooperation of a multidisciplinary team that can systematically evaluate each individual and ensure that a protocol-driven pathway is undertaken for the best patient care. Oral and maxillofacial surgeons contribute to surgical care in this setting with specific knowledge of growth and development of the face. This enables optimum timing for early skeletal correction where appropriate, and definitive surgery following the cessation of growth to maximize function and aesthetics. This chapter will describe the major principles in managing patients with specific craniofacial anomalies and provide examples of the outcomes possible.

Distant metastasis in patients with myxofibrosarcoma.

PURPOSE: A clinical feature of myxofibrosarcoma is local recurrence, but knowledge about distant metastasis is sparse. We evaluated the tendency of clinical and histological features of metastasis in myxofibrosarcoma patients. METHODS: Fifty-eight patients with myxofibrosarcoma were treated in our hospitals, and a total of 16 consecutive patients with distant metastases were included in this retrospective study (9 males and 7 females, with a mean age of 77 years). Because there was no patient complicated by both lung and lymph node metastases, we compared the age, sex, tumor size and location, French Federation of Cancer Centers Sarcoma Group (FNCLCC) grade, American Joint Committee on Cancer (AJCC) stage, and times of the first metastasis from the initial examination between the lung and lymph node groups. In addition, we examined factors affecting the prognosis. RESULTS: The median follow-up period was 42.9 months (range 8-142). Eleven of 16 patients developed pulmonary metastases. The sites of extra pulmonary metastases were the lymph nodes in 5 patients, bone in 1, subcutaneous in 1, intramuscular in 1, and peritoneum in 1. The median time for patients to develop distant metastases was 17.4 months (range 0-59). The time until the onset of the first metastasis in the lung metastasis group was significantly shorter than in the lymph node group (p < 0.05). Also, the survival rate in the lymph node metastasis group was better than in the lung metastasis group (p < 0.05). CONCLUSIONS: Not only lung metastasis but also lymph node metastasis occurs frequently in myxofibrosarcoma patients. Myxofibrosarcoma with lung metastasis is more aggressive than the type with lymph node metastasis.

Cardiac myxoma: clinical characteristics, surgical intervention, intra-operative challenges and outcome.

OBJECTIVE: The objectives of this retrospective study were to characterize the clinical presentation, diagnostic findings, surgical approaches, intra-operative challenges and complications following the surgical treatment of cardiac myxoma in two of the largest referral hospitals in Jordan. METHODS: Medical records of all patients presented to the cardiology department during the period between 1984 and 2016 were reviewed. Criteria for inclusion in the study were: (1) patients who were presented for cardiac evaluation due to symptoms suggestive of a primary cardiac problem, (2) completed medical records, including results of echocardiography suggestive of intra-cardiac occupying mass, (3) the surgical operation was undertaken and intra-operative data was available, (4) a histological diagnosis of myxoma was available and (5) the discharge status and follow-up data were available for at least 2 years after initial surgery. RESULTS: A total of 27 patients fulfilled the criteria for inclusion in the study. The average age was 42 years. Thirteen of the patients were females and 14 patients were males. The most common clinical presentations were dyspnea (29.6%) and murmurs (22.2%). Non-specific signs, such as weight loss, fever, fatigue, arthralgia and anemia, were reported in 10 (37%) patients. Signs related to systemic embolization were reported in 9 (33.3%) of the patients, involving the upper and lower extremities (55.6%), brain ischemia and vision loss (54.4%). Tumors in all patients were successfully resected under cardiopulmonary bypass support. The tumor was pedunculated in 17 (62.9%) of the patients and the size of the tumors varied from 1 to 7 centimeters in diameter. The tumor was located in the left atrium in 21 patients (77.7%), in the right atrium in 4 patients (14.8%), in the right ventricular side of the septum in 1 patient (3.7%) and involving the tricuspid valve in 1 patient (3.7%). The left atrial approach was used in 3 patients, a right atrial approach in 4 patients and a bi-atrial approach in 20 patients. The most common concomitant cardiac pathology was coronary artery disease in 3 patients and an atrial septal defect in one patient. The most common intra-operative challenges were inter-atrial communication (2 patients), large defect in the tricuspid valve leaflet (1 patient) and involvement of the inter-ventricular septum (1 patient). In the post-operative period, 1 patient suffered a stroke. In the post-operative period, the most common complications were stroke (1 patient) and recurrence of the tumor (2 patients). CONCLUSION: The success rate after surgical removal of cardiac myxoma in this study was substantial and complications were rare.

Unusual aspects of cardiac myxoma.

A cardiac myxoma may manifest as miscellaneous and uncharacteristic presentations. These unusual aspects of cardiac myxomas can be rare clinical presentations, special patient populations, unusual locations, and special pathology, which may lead to a delayed diagnosis, improper checkups, and subsequent untimely treatment, eventually resulting in unexpected poor prognosis. Therefore, the diagnosis of cardiac myxomas can be challenging because of these unusual aspects. In order to get a better understanding of a cardiac myxoma and to facilitate an early diagnosis and proper treatment, the unusual aspects of cardiac myxomas are described here.