Search

Background: The occurrence of cerebral aneurysm in a case of cardiac myxoma is rare with less than 60 cases reported worldwide. The course of management is still debatable given its rarity. We present a case of multiple intracranial aneurysms secondary to atrial myxoma in a young lady with a brief review of the literature. Case presentation A young lady in her late 30s with a history of right middle cerebral artery territory stroke eight years ago presented with gradually progressive symptoms in the form of holocranial headache, inattention and forgetfulness for the last few years. On neuroimaging, she was found to have multi-territorial lacunar infarcts and multiple intracranial artery aneurysms which was confirmed with a digital subtraction angiogram. A cardiac evaluation revealed a left atrial myxoma. The aetiology of subcortical cognitive decline and intracranial aneurysms was attributed to the myxoma with secondary myxomatous embolism. Other secondary causes were ruled out. She is being followed up medically after resection of the myxoma. Conclusion: Intracranial aneurysms are rare complications of cardiac myxoma which may present before, concurrent or many years after diagnosis of the myxoma. Nonspecific neurological complaints occasionally are the ominous signs of intracranial aneurysms which mandate a low threshold for neuroimaging in a case of cardiac myxoma. Given the absence of definitive risk factors and unclear natural history, clinical and radiological follow-ups are critical. Learning Points Intracranial aneurysms are rare complications of cardiac myxoma that may present before, concurrent or many years after diagnosis of the myxoma. Special attention must be given to nonspecific neurological complaints with a low threshold for neuroimaging in those with a prior history of cardiac myxoma. Given the absence of definitive risk factors and unclear natural history, clinical and radiological follow-up including conventional angiography and/or magnetic resonance imaging is critical. [ABSTRACT FROM AUTHOR]

Minimally invasive surgery has emerged as a favorable alternative to conventional surgery for various cardiac conditions. This study aimed to compare the perioperative outcomes and follow-up results of the robotic approach versus the sternotomy approach for left atrial myxoma (LAM) resection. We retrospectively analyzed the perioperative outcomes and follow-up results of 94 patients who underwent left atrial myxoma resection using either the sternotomy approach (n = 64) or the robotic approach (n = 30) at our center between January 2017 and April 2023. Multiple linear regressions were employed to examine the actual impact of the surgical approach on perioperative outcomes while controlling for potential confounding factors. There were no in-hospital deaths or follow-up deaths in the robotic group. Univariate analyses revealed that robotic LAM resection had a longer cardiopulmonary bypass (CPB) time (99.93 ± 22.30 vs. 76.28 ± 24.92, P < 0.001), longer aortic clamping time (57.80 ± 20.27 vs. 47.89 ± 18.10, P = 0.019), reduced postoperative drainage (P < 0.001), shorter mechanical ventilation time (P = 0.005), shorter postoperative bed-stay time (P < 0.001), shorter postoperative hospitalization time (P = 0.040), and higher hospital costs (P = 0.001) compared to the sternotomy group. After adjusting for baseline characteristics in a multiple regression model, a longer CPB time (B = 28.328; CI, 18.609–38.047; P < 0.001), longer aortic clamping time (B = 11.856; CI, 4.069–19.644; P = 0.003), reduced postoperative drainage (B = −200.224; CI, −254.962– –145.486; P < 0.001), shorter mechanical ventilation time (B = −3.429; CI, −6.562– –0.295; P = 0.032), shorter postoperative bed-stay time (B = −2.230; CI, −3.267– –1.193; P < 0.001), shorter postoperative hospitalization time (B = −1.998; CI, −3.747– –0.250; P = 0.026), and higher hospital costs (B = 2096.866, P = 0.002) were found in the robotic group. Furthermore, the robotic group exhibited a faster return to exercise compared to the sternotomy group (Log-Rank χ2 = 34.527, P < 0.001). Both the robotic and sternotomy approaches are viable and safe options for LAM resection. However, despite the higher costs, longer CPB time, and longer aortic clamping time associated with robotic LAM resection, this technique was correlated with reduced postoperative drainage and faster postoperative recovery compared to the sternotomy technique. [ABSTRACT FROM AUTHOR]

AbstractBackgroundCase reportConclusionsCardiac myxoma is a rare, benign tumour that commonly originates in the left atrium and may lead to embolic events. Parenchymal brain metastases represent a rare neurological manifestation. While surgical intervention is commonly used, there is limited information on long-term outcomes after radiotherapy treatment. This report describes a case of successful treatment of haemorrhagic brain metastases with radiotherapy and offers a literature review of long-term results after radiotherapy treatment.A 49-year-old woman presented with multiple haemorrhagic brain lesions and a cardiac mass. Surgical removal of the cardiac mass and the symptomatic brain lesion confirmed metastatic cardiac myxoma. Post-surgery, she experienced fatigued and neurocognitive impairment and was closely monitored. However, the metastases progressed. She subsequently received whole-brain radiotherapy, resulting in complete response. Seven years later, she remains in remission, although with enduring neurocognitive impairment.Whole-brain radiotherapy can provide long-term control of haemorrhagic brain metastases arising from cardiac myxoma. Radiotherapy dose and treatment volume need careful consideration to reduce toxicity. [ABSTRACT FROM AUTHOR]

Background: Deep angiomyxoma (DAM) is a slow-growing benign tumor with high risk of local recurrence after surgical resection. Although DAM in a lower extremity is extremely rare, clinicians must be aware of its possible occurrence. Suspicion can be made based on clinical examination and radiological imaging but final diagnosis is confirmed on histopathological examination and immunohistochemistry. We intend to present an extremely rare case of DAM in the knee, managed successfully with marginal excision. Case Presentation: A 4-year-old male child presented with the complains of a painless, progressively increasing, soft, non-tender, and fluctuant swelling in his right knee. The plain radiograph showed a non-calcified soft-tissue swelling and MRI revealed a multi-loculated cystic lesion with multiple septations. A pre-operative diagnosis of a benign cystic lesion was made. It was managed by marginal excision of the tumor and a histological diagnosis of DAM was made. IHC staining showed positivity for SMA, CD34 and vimentin were focally positive, while desmin and calponin were negative. At 12 months of follow-up, the patient had a normal painless gait and full knee ROM, without any local recurrence. Conclusion: DAM is a rare tumor which is often misdiagnosed. In this report, we present a rare case of benign cystic lesion which turned out to be DAM on HPE of resected specimen. Marginal excision of this lesion revealed good outcomes with no recurrence until 12 months of final follow-up. With this, we conclude that surgical excision should be the gold standard in cases of DAM. [ABSTRACT FROM AUTHOR]

Abstract Background Primary cardiac tumors, while rare, present significant clinical challenges due to their diverse pathology and presentation. Lung cancer frequently metastasizes to the heart; however, cases involving primary cardiac tumors of different origins alongside primary lung cancer are exceedingly rare in the literature. Case presentation We report the case of a 53-year-old female who presented with hemoptysis and was subsequently diagnosed with a left atrial myxoma, pulmonary squamous cell carcinoma, and a thymic cyst. This coexistence of multiple non-homologous tumors in a single patient is exceedingly rare. Conclusion This case underscores the complexity of diagnosing and managing patients with multiple distinct tumors. The simultaneous occurrence of a primary cardiac myxoma, pulmonary squamous cell carcinoma, and thymic cyst is unprecedented, providing valuable insights for future clinical practice.

Soft tissue myxomas are benign mesenchymal neoplasms typically found in the intramuscular components of the proximal extremities. However, soft tissue myxomas in the chest wall are rare. We report the case of a 41-year-old woman with soft tissue myxoma who presented with a slowly growing, palpable lump on her left anterior chest wall that has been present for several years. Mammography revealed an oval, circumscribed, and hyperdense mass in the retromammary fat layer of the upper inner quadrant of the left breast. Ultrasonography revealed an oval, circumscribed, and hypoechoic mass with internal echoes and multiple microcystic spaces. A triangular hyperechoic area adjacent to the pole of the mass and peripheral hyperechoic rim were also detected. The patient underwent surgical excision and was diagnosed with soft tissue myxoma with subcutaneous manifestation.

Background: Primary cardiac tumors, while rare, present significant clinical challenges due to their diverse pathology and presentation. Lung cancer frequently metastasizes to the heart; however, cases involving primary cardiac tumors of different origins alongside primary lung cancer are exceedingly rare in the literature. Case presentation: We report the case of a 53-year-old female who presented with hemoptysis and was subsequently diagnosed with a left atrial myxoma, pulmonary squamous cell carcinoma, and a thymic cyst. This coexistence of multiple non-homologous tumors in a single patient is exceedingly rare. Conclusion: This case underscores the complexity of diagnosing and managing patients with multiple distinct tumors. The simultaneous occurrence of a primary cardiac myxoma, pulmonary squamous cell carcinoma, and thymic cyst is unprecedented, providing valuable insights for future clinical practice. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Understand the importance of considering alternative diagnosis in patients presenting with atypical features, specially when they are not responding to the standard treatment. Understand the importance of considering common presentations of rare cases. Underscoring the critical importance of timely recognition and appropriate management of potentially life‐threatening conditions. [ABSTRACT FROM AUTHOR]

Background: Pseudomyxoma peritonei is an infrequent condition with a global annual incidence of only one to two cases per million people. Mucinous neoplasms, widespread intraperitoneal implants, and mucinous ascites characterize it. Currently, most clinicians misdiagnose this condition, which leads to delayed management. Case presentation: A 44-year-old North Indian female presented with a 1.5-month history of an abdominal lump. Physical examination revealed a sizeable abdominopelvic mass at 36 weeks. Contrast-enhanced computed tomography showed a massive multiloculated right ovarian cystic mass measuring 28 × 23 × 13 cm with mild ascites and elevated carcinoembryonic antigen levels (113.75 ng/ml). A provisional diagnosis of ovarian mucinous neoplasm was made, for which the patient underwent laparotomy. Intraoperatively, there were gross mucinous ascites, along with a large, circumscribed, ruptured right ovarian tumor filled with gelatinous material. The appendicular lump was also filled with mucinous material along with the omentum, ascending colon, right lateral aspect of the rectum, splenic surface, and small bowel mesentery. Cytoreductive surgery was performed along with an oncosurgeon, including total abdominal hysterectomy with bilateral salpingoophorectomy, omentectomy, right hemicolectomy, lower anterior resection, ileo-transverse stapled anastomosis with proximal ileal loop diversion stoma, excision of multiple peritoneal gelatinous implants, and peritoneal lavage. Histopathology and immunohistochemistry confirmed the presence of intestinal-type mucinous carcinoma. Postoperatively, the patient was given six cycles of chemotherapy. She tolerated it without any specific morbidity and had an uneventful recovery. Postoperative follow-up at 15 months revealed normal tumor marker levels and abdominal computed tomography findings and no signs suggestive of local recurrence or distal metastases. Conclusions: Pseudomyxoma peritonei is a rare disease that is frequently misdiagnosed in the preoperative phase. Therefore, radiologists and clinicians should maintain a high index of suspicion for accurate diagnosis and multidisciplinary management. [ABSTRACT FROM AUTHOR]

Background: Carney syndrome is an uncommon autosomal disorder closely linked to mutations in the PRKAR1A gene. Skin lesions are the most pronounced feature of Carney syndrome, affecting over 80% of individuals with this condition. This syndrome is characterized by a triad of myxomas, skin pigmentation, and endocrine hyperfunction, featuring multiple endocrine neoplasms with skin and cardiac involvement. Dilated cardiomyopathy, a primary cardiomyopathy, is defined as the dilation and impaired systolic function of the left or both ventricles. Its clinical presentation varies from being asymptomatic to heart failure or sudden cardiac death, making it a leading global cause of heart failure. Currently, Dilated cardiomyopathy has an estimated prevalence of 1/2500–1/250 individuals, predominantly affecting those aged 30–40 years, with a male-to-female ratio of 3:1. This case report describes a heart failure patient with cardiac myxoma caused by Carney syndrome combined with dilated cardiomyopathy. The patient was successfully treated for heart failure by heart transplantation. Case presentation: Herein, we report a case of heart failure due to Carney syndrome that resulted in cardiac myxoma combined with dilated cardiomyopathy. A 35-year-old male was admitted to the hospital three years ago because of sudden chest tightness and shortness of breath. Echocardiography indicated myxoma, and a combination of genetic screening and physical examination confirmed Carney syndrome with cardiac myxoma. Following symptomatic management, he was discharged. Surgical interventions were not considered at the time. However, the patient's chest tightness and shortness of breath symptoms worsened, and he returned to the hospital. A New York Heart Association grade IV heart function was confirmed, and echocardiography indicated the presence of dilated cardiomyopathy accompanied by cardiac myxoma. Ultimately, the patient's heart failure was successfully treated with heart transplantation. Conclusions: Cardiac myxoma caused by Carney syndrome combined with heart failure caused by dilated cardiomyopathy can be resolved by heart transplantation. [ABSTRACT FROM AUTHOR]

Cardiac myxoma is the most commonly diagnosed benign cardiac tumor. It is usually located in the left atrium and typically arises from the foramen ovale in approximately 75% of the cases, in the right atrium in 23%, and in the ventricles in only 2%. Symptoms depend on its size, mobility, and location. Neurological complications are seen in 20% to 25% of patients. Herein we present a case of right ventricular myxoma with patent foramen ovale which was complicated by stroke. The mass was surgically excised and patent foramen ovale was closed. [ABSTRACT FROM AUTHOR]

Objective: Cardiac tumors are rare pathology, and myxoma is the most frequently encountered. Co-existence of double myxoma or right heart and coronary artery disease is a rare condition. The aim of our case report is to demonstrate a case with successful simultaneous surgical removal of myxoma and CABG in a patient with double myxoma of right heart and severe mutlivessel coronary artery disease, concomitant type 2 diabetes and heart failure. Case presentation: A 68-year-old patient was admitted with typical complaints of severe shortness of breath, palpitations, lack of air, pain in the heart, weakness. From the anamnesis, it was revealed that the above complaints appeared over the past 4 years, he had also diabetes. Patients had NYHA functional class III-IV. Echocardiography revealed a large mobile mass in the cavity of the right atrium and right ventricle, periodically obturating the right atrioventricular orifice. Coronary angiography displayed mutli-vessel coronary artery disease. We performed the simultaneous operation of double myxoma removal and coronary bypass surgery – arterial graft – left internal mammary artery to left anterior descending artery and venous graft from aorta to posterior descending artery under cardiopulmonary bypass. The surgery was successful and without complication The patient was transferred from intensive care unit to ward on the 4th day after surgery and was discharged on the 10th day. Conclusion: Our case confirms the possibility of successful surgical treatment of several severe pathologies of the cardiovascular system as double myxoma removal and coronary bypass surgery, with the right choice of surgical tactics and well-coordinated work of a team of cardiac surgeons, cardiac anesthesiologists, perfusionists, cardiologists, and reanimatologists. [ABSTRACT FROM AUTHOR]

Background: Cardiac hemangiomas are very uncommon benign primary tumors. They are usually located preferentially in the right atrium and their location in the interatrial septum is extremely rare. Case presentation: We report the case of a 41-year-old patient who was admitted due to a stroke. The transthoracic echocardiogram revealed a large mass in the right atrium adhered to the interatrial septum. Suspecting an atrial myxoma, surgical intervention was performed confirming that the mass extended within the thickness of the interatrial septum, protruding into the right atrial cavity. The histologic report confirmed a hemangioma. Conclusions: Cardiac hemangiomas are rare primary tumors and are usually misdiagnosed as other cardiac tumors. Histopathological examination is essential for a definitive diagnosis. [ABSTRACT FROM AUTHOR]

Cellular myxoma is a benign soft tissue tumor frequently associated with GNAS mutation that may morphologically resemble low-grade myxofibrosarcoma. This study aimed to identify the undescribed methylation profile of cellular myxoma and compare it to myxofibrosarcoma. We performed molecular analysis on twenty cellular myxomas and nine myxofibrosarcomas and analyzed the results using the methylation-based DKFZ sarcoma classifier. A total of 90% of the cellular myxomas had GNAS mutations (four loci had not been previously described). Copy number variations were found in all myxofibrosarcomas but in none of the cellular myxomas. In the classifier, none of the cellular myxomas reached the 0.9 threshold. Unsupervised t-SNE analysis demonstrated that cellular myxomas form their own clusters, distinct from myxofibrosarcomas. Our study shows the diagnostic potential and the limitations of molecular analysis in cases where morphology and immunohistochemistry are not sufficient to distinguish cellular myxoma from myxofibrosarcoma, particularly regarding GNAS wild-type tumors. The DKFZ sarcoma classifier only provided a valid prediction for one myxofibrosarcoma case; this limitation could be improved by training the tool with a more considerable number of cases. Additionally, the classifier should be introduced to a broader spectrum of mesenchymal neoplasms, including benign tumors like cellular myxoma, whose distinct methylation pattern we demonstrated. [ABSTRACT FROM AUTHOR]

Background Multiple myxomas are rare and often associated with Carney syndrome. We present a giant left atrial myxoma that passes through a patent foramen ovale (PFO), mimicking biatrial myxoma. Case summary A 46-year-old female was admitted to the hospital with a cardiac neoplasm without symptoms. The physical examination did not reveal any abnormalities in the skin and endocrine system. Transthoracic echocardiography revealed a large, hypoechoic mass attached to the atrial septum, occupying both the left and right atria. This mass appeared as a bilateral atrial mass on echocardiography. Contrast-enhanced echocardiography revealed a slight enhancement in the mass. The patient was scheduled for surgery without any contraindications. During the operation, a large jelly-like mass originating from the left atrial side of the fossa ovale was found, extending to the right atrium through a PFO. The entire mass was successfully removed, and the atrial septum was repaired. Histopathology confirmed the diagnosis of cardiac myxoma. At the 3-month follow-up, no cardiac abnormalities were observed. Discussion Multiple myxomas have been reported in certain cases of Carney syndrome. A single left atrial myxoma that passes through an atrial septal defect or a PFO can sometimes be misdiagnosed as a bilateral atrial myxoma. Echocardiography plays a crucial role in providing diagnostic information by accurately identifying the location of the myxoma pedicle. [ABSTRACT FROM AUTHOR]

Background Myxomas are uncommon and benign cardiac neoplasms that can present with various cardiac, systemic, embolic, or without symptoms depending on their location and size. Very few cases of large, truly biatrial, or tumours connected via the cardiac atria have been reported throughout the years. Case summary We present an unusual case of an apparently healthy 25-year-old French woman, who presented with dyspnoea at Munich's Octoberfest. Echocardiography and computed tomography identified gigantic masses in left and right atrium, which were connected through an atrial septal defect. They were successfully removed by emergent cardiac surgery. Discussion This case describes an uncommon tumour and highlights the importance of a quick diagnosis and prompt surgery. We describe the management and surgery for atrial myxomas as well as demonstrating pre- and intraoperative pictures. [ABSTRACT FROM AUTHOR]

Copyright of Medizinische Klinik: Intensivmedizin & Notfallmedizin is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Renal embolisms due to cardiac myxomas are extremely rare; the clinical course, treatment, and prognosis of this disease are not established. A 69-year-old Japanese woman who underwent a nephrectomy for renal cell carcinoma 3 years earlier was hospitalized with a right occipital lobe cerebral infarction. Her renal function suddenly worsened 3 days post-admission: her serum creatinine rose from 1.46 mg/dL to 6.57 mg/dL and then to 8.03 mg/dL the next day, and hemodialysis therapy was started. Abdominal computed tomography (CT) scans showed patchy non-contrasted low-density areas in the right kidney, and chest CT scans and transesophageal ultrasonography revealed a left atrial tumor. We diagnosed renal infarction due to a left atrial myxoma. Hemodialysis and anticoagulant therapy (heparin) were continued, followed by the cardiac myxoma's resection. The patient's renal function gradually improved post-surgery, and the hemodialysis was discontinued. Considering our patient and 19 other case reports of renal infarction associated with cardiac myxoma, the treatment for such a renal infarction and the outcomes differ depending on the embolus site. The poor outcome of abdominal aortic embolism requires a prompt embolectomy, whereas a branch renal artery embolism requires anticoagulation therapy to prevent thrombosis formation around the myxoma. [ABSTRACT FROM AUTHOR]

Studies have shown that primary cardiac tumors are exceptionally rare and among them myxomas represents approximately half. Although majority of these benign tumors are located in left atrium, they can also emerge in the right cardiac chambers and also on cardiac valves. In rare instances, they may extend into the great vessels. In this case, we present a teenage girl with a past medical history of successfully cured acute lymphoblastic leukemia, who presented with progressive dyspnea and lower limbs edema. Based on history, laboratory, and echocardiographic evidences, she was initially treated for high-risk thromboembolism but after inefficient response, she underwent surgery, with a large right ventricular outflow tract myxoma being diagnosed.

Ganglion cysts are the most common soft‑tissue tumors observed in the finger. Some tumors could be easily confused clinically with ganglion cysts due to their localization, mobility, shape, and being soft in nature. In this article, two cases of schwannoma and superficial acral myxoma that were clinically and radiologically confused with ganglion cysts are presented. Suspecting this tumor type in the preoperative diagnosis is essential to avoid unnecessary surgical approaches. Masses in close proximity to the nerve or nerve sheath are very important as they require preservation of the nerve during surgery. For these masses, surgeons should be prepared as these cases can have many intraoperative surprises in store. [ABSTRACT FROM AUTHOR]

The article describes the case of a 70-year old female diagnosed with large superficial angiomyxoma of the tongue causing dysphagia. The patient was treated with surgical excision using carbon dioxide laser. The clinical features of superficial angiomyxoma are described and differential diagnoses are also indicated, that include angiomyolipoma, soft tissue myxoma and oral focal mucinosis.

Abstract An 80-year-old female was referred to our institution due to transient right upper limb weakness. Transthoracic and transesophageal echocardiography revealed a tumor in the left atrium. The tumor was attached to the posterior wall of the left atrium near the atrioventricular node. Intraoperative pathological examination revealed that the tumor was a myxoma, and complete resection was successfully performed. However, she experienced persistent complete atrioventricular block postoperatively and required pacemaker implantation.

Myxomas are tumors of soft tissue with fibroblast origin characterized by their remarkable myxoid matrix rich in mucopolysaccharides. Here, we report the clinical and histopathological characteristics of angiomyxoma in a pregnant two-year-old- Holstein heifer. A notable and palpable mass was found in the right teat of the front quarter with no ulcer or infection. In the gross examination, the mass was soft, solitary, well-circumscribed, and unencapsulated grey to white. The mass was removed by local anesthesia and surgery. Histopathology dominantly demonstrated an unencapsulated and well-differentiated proliferation of spindle to stellate-shaped fibroblasts loosely arranged in a myxoid matrix associated with admixed numerous thin-walled blood vessels. Cellularity was low, mitoses were rare, and there was little cytological atypia. No evidence of hemorrhage, edema, necrosis, and acute inflammation were observed on the tissue sections. Immunohistochemical examinations were positive for S100 and vimentin markers. The surgical removal of the tumor mass with sedation and local anesthesia was suggested as the treatment of choice for optimal recovery.

Abstract Background Myxomas are the most common primary cardiac tumor and typically originate in the left atrium. Atrial myxomas may present following complications of obstruction and emboli. If an atrial myxoma goes untreated, complications such as congestive heart failure, embolic stroke, and sudden death can occur. Case presentation A 58-year-old Caucasian male presented following a cardiac arrest. He was taken emergently to the cardiac catheterization lab and received two drug eluting stents. Following the procedure, he was found to have a left atrial mass that was intermittently obstructing the mitral valve on echocardiography. After leaving the cardiac catheterization lab, he was hypotensive and placed on multiple intravenous medications for hemodynamic support as well as an Impella device. Following medical optimization, he underwent one vessel coronary artery bypass graft as well as surgical excision of the left atrial mass, which pathology had shown to be an atrial myxoma. Conclusion This patient’s case of cardiogenic shock following revascularization was complicated by the identification of an atrial myxoma, which, when large enough, can obstruct blood flow through the mitral valve leading to acute mitral dynamic stenosis. This condition results in circulatory collapse due to obstruction of the left ventricle in diastole as the myxoma occludes the mitral valve.

An 80-year-old female was referred to our institution due to transient right upper limb weakness. Transthoracic and transesophageal echocardiography revealed a tumor in the left atrium. The tumor was attached to the posterior wall of the left atrium near the atrioventricular node. Intraoperative pathological examination revealed that the tumor was a myxoma, and complete resection was successfully performed. However, she experienced persistent complete atrioventricular block postoperatively and required pacemaker implantation. [ABSTRACT FROM AUTHOR]

Background: The presence of a primary cardiac tumor in a pediatric patient is a rare echocardiographic finding. Case Report: We report the case of an 11-year-old female patient with multiple peripheral embolisms, due to a gigantic left ventricular tumor, with a unique echocardiographic appearance. The patient was referred to the emergency department due to acute pain and loss of sensitivity in both of her legs. Past medical history was significant for acute lymphoblastic leukemia. Upon physical examination, suspicion of bilateral lower leg ischemia was raised. Doppler arterial ultrasound of both legs confirmed the suspicion mentioned above, as the right lower extremity suffered from partial arterial occlusion of the external iliac artery and total occlusion of the femoral arteries. Meanwhile, in the left lower extremity, the occlusion was localized in the proximal tibio-peroneal artery. Cardiac sonography revealed a massive, mobile, left ventricular intracavitary mass. Aside from its large dimensions (6.3 cm by 3 cm), its aspect was striking as well as it had very mobile and friable edges. Emergency bilateral endarterectomy and excision of the left ventricular tumor were performed alongside systemic anticoagulant therapy, with excellent results, as no tumoral residual masses could be seen in the left ventricle, and the arterial blood flow was restored completely in both lower extremities. The histopathological aspect of the excised masses was that of a myxoma. The patient recovered well after surgery and was discharged on postoperative day 14. Conclusion: Despite only a handful of cases of cardiac myxomas being reported due to their rarity in the pediatric population, clinical presentation with peripheric embolism triggered a high index of suspicion of embolic mechanism in our patient and prompted a rapid assessment and successful management. [ABSTRACT FROM AUTHOR]

Background: Left atrial myxoma during pregnancy is rare. We present three cases in order to aid in the management. Case Presentation: Three cases of left atrial myxoma during pregnancy were presented in this article. Three patients all received multidisciplinary team work and acquired good outcomes. The case 1 had no symptoms and delivered before traditional cardiac surgery. The case 2 and case 3 undergone totally endoscopic minimally invasive cardiac surgery during pregnancy. The case 3 maintained pregnancy to term and gave birth to a healthy baby via vaginal delivery. No relapse of the tumor was observed. Conclusions: The management of left atrial myxoma during pregnancy ought to be individualized and combined with the gestational age. If the diagnosis was made in the first two trimesters of pregnancy, totally endoscopic minimally invasive cardiac surgery during pregnancy would be an optimal choice. The patients can benefit from the multidisciplinary team work. [ABSTRACT FROM AUTHOR]

Simple Summary: Aggressive angiomyxoma is a mesenchymal tumor with localized aggressiveness, affecting the connective tissue of the perineum or the lower pelvis. Prevalence in the population is unknown due to its rarity, making management and counseling difficult. The management of angiomyxoma includes multiple types of treatment, such as radical surgery with tumor-free margins, but the probability of local recurrence is high, despite extensive excision with unscathed mar-gins. Considering the low mitotic activity of angiomyxoma, there is not always a rationale for adjuvant radiotherapy and chemotherapy. Given its exceptionally low incidence, optimal management of the disease remains a subject of on-going debate, and a unanimous consensus on treatment strategies has yet to be reached. Aggressive angiomyxoma (AAM) is a rare, locally aggressive, myxoid mesenchymal neoplasm primarily found in the pelvic and perineal regions of young adult females. It is a slow growing and locally infiltrating tumor. Preoperative diagnosis is difficult due to the rarity of these tumors and absence of characteristic signs and symptoms. The primary management is tumor excision. Incomplete excision is common because of the infiltrating nature of the neoplasm and absence of a definite capsule. Other non- surgical modalities have been employed, such as radiotherapy, embolization, GnRH analogues or other anti-estrogenic agents. Local relapses occur in 30–40% of the cases, and often appear many years (sometimes decades) after the first excision. Occasional distant metastasis has also been reported. A limited number of cases have been reported in the literature, mostly in the form of small case series or isolated case reports. Therefore, the aim of this paper by a team of experts from the MITO rare tumors group is to review clinical findings, pathologic characteristics and outcome of patients affected by this rare condition in order to be able to offer up-to-date guidance on the management of these cases. [ABSTRACT FROM AUTHOR]

Juxta-articular myxoma (JAM) is a rare soft tissue tumour predominantly composed of mucinous tissue and usually found around large joints. We report a 73-year-old woman with a 5-year history of a soft tissue mass in the thenar eminence of the right wrist who presented to our department. An initial diagnosis of a ganglion cyst was made using magnetic resonance imaging (MRI) and the jellied content of the mass by aspiration. Two years after presentation, surgical treatment with tumour resection and carpal tunnel release via the radial approach was performed because the symptoms of carpal tunnel syndrome worsened, and the tumour invaded the carpal tunnel. Histopathological examination revealed a JAM. At the 1-year follow-up, the symptoms of carpal tunnel syndrome had resolved, and no recurrence was confirmed by MRI. Level of Evidence: Level V (Therapeutic) [ABSTRACT FROM AUTHOR]

Background: Odontogenic myxoma is one of the benign mesenchymal odontogenic tumours with aggressive behaviour and showed slow and asymptomatic expansion, the second until the third decade of life is the most targeted age group. With high female preponderance, about the management, surgical management is the only option concerning this odontogenic pathology with a variety of treatment options. This study aims to analyse a series of 37 patients with odontogenic myxoma treated in a single institution. Materials and method: In total, 37 patients with odontogenic myxoma were treated at KTDH in Sudan and were retrospectively reviewed. With the analysis of medical records of all patients diagnosed with odontogenic myxoma and the related variables, data were analysed using the SPSS statistical program (version 23). Results: Most of the cases were females (26 patients 70.27%), and males were about (11 patients 29.73%). In most of the cases maxilla was affected more than the mandible (25 cases, 67.57%) mostly in its posterior segment (32 cases, 86.49). The most age group affected was the group of cases (0–20) which were about 18 cases (48.65%). In total, 35 of the cases noticed swelling (94.59%), while 14 noticed tooth mobility (37.84%) and paraesthesia was a positive finding in just one case (2.70%). In total, 12 patients (32.43%) had a positive history of tooth extraction related to the lesion, and recurrence was positive in (64, 86%). Conclusion: Odontogenic myxoma is locally aggressive. There is no gold standard protocol for surgical treatment so choosing the most suitable and reliable treatment option relay on the operator taking into consideration the characteristic of each case and the recurrence rate with the associated postoperative impairments. [ABSTRACT FROM AUTHOR]

Background Primary cardiac tumours are rare, accounting for only 0.002–0.03% at autopsy. Cardiac haemangiomas are benign vascular tumours and constitute for 0.28% of all primary cardiac tumours. Cavernous haemangiomas, capillary haemangiomas, and arteriovenous haemangiomas are three distinct types. Cardiac haemangiomas are often misdiagnosed as myxomas and must be differentiated from malignant angiosarcomas. Case summary We present a 44-year-old Mediterranean male patient with a cavernous haemangioma in the inferior vena cava and right atrium, detected on transthoracic echocardiography. The patient experienced palpitations and dyspnoea on exertion. Computed tomography (CT) angiography revealed a 7.5 × 6 × 5 cm mass suspected to be perfused by the distal right coronary artery. A watch-and-wait approach was suggested, leading to a cardiac magnetic resonance imaging (MRI) with contrast 6 months later. T1 mapping exhibited a prolonged relaxation time and isointensity to the myocardium. T2 mapping revealed a homogenous hyperintense mass with heterogenous late enhancement. Surgical excision was performed using a bicaval cannulation technique on cardiopulmonary bypass. Intraoperatively, no connection to the coronaries was noted. At 1 year follow-up, the patient reported restored physical resilience, with no evidence of tumour recurrence. Discussion Clinical symptoms of cardiac cavernous haemangiomas are unspecific and become evident once the tumour grows. To investigate the nature and vascular involvement of the tumour, a contrast-enhanced CT angiography or MRI can be performed. Cardiac haemangiomas are often misdiagnosed and must be differentiated from malignant angiosarcomas. Clear guidelines for the treatment of cardiac haemangiomas in adult patients are lacking. Primary cardiac tumours require thorough investigation, and surgical intervention should be tailored to the individual's case. [ABSTRACT FROM AUTHOR]

Background: Pseudomyxoma peritonei (PMP) is a rare clinical malignant syndrome, and its rarity causes a lack of pathology research. This study aims to quantitatively analyze HE‐stained pathological images (PIs), and develop a new predictive model integrating digital pathological parameters with clinical information. Methods: Ninety‐two PMP patients with complete clinic‐pathological information, were included. QuPath was used for PIs quantitative feature analysis at tissue‐, cell‐, and nucleus‐level. The correlations between overall survival (OS) and general clinicopathological characteristics, and PIs features were analyzed. A nomogram was established based on independent prognostic factors and evaluated. Results: Among the 92 PMP patients, there were 34 (37.0%) females and 58 (63.0%) males, with a median age of 57 (range: 31–76). A total of 449 HE stained images were obtained for QuPath analysis, which extracted 40 pathological parameters at three levels. Kaplan–Meier survival analysis revealed eight clinicopathological characteristics and 20 PIs features significantly associated with OS (p < 0.05). Partial least squares regression was used to screen the multicollinearity features and synthesize four new features. Multivariate survival analysis identified the following five independent prognostic factors: preoperative CA199, completeness of cytoreduction, histopathological type, component one at tissue‐level, and tumor nuclei circularity variance. A nomogram was established with internal validation C‐index 0.795 and calibration plots indicating improved prediction performance. Conclusions: The quantitative analysis of HE‐stained PIs could extract the new prognostic information on PMP. A nomogram established by five independent prognosticators is the first model integrating digital pathological information with clinical data for improved clinical outcome prediction. Pseudomyxoma peritonei (PMP) is a rare clinical malignant syndrome, and its rarity causes the insufficiency of pathology research. This study established a prognosis nomogram prediction model based on new pathological characteristics of PMP through quantitative analysis of pathological images. [ABSTRACT FROM AUTHOR]

Background: Myxomas are the most common primary cardiac tumor and typically originate in the left atrium. Atrial myxomas may present following complications of obstruction and emboli. If an atrial myxoma goes untreated, complications such as congestive heart failure, embolic stroke, and sudden death can occur. Case presentation: A 58-year-old Caucasian male presented following a cardiac arrest. He was taken emergently to the cardiac catheterization lab and received two drug eluting stents. Following the procedure, he was found to have a left atrial mass that was intermittently obstructing the mitral valve on echocardiography. After leaving the cardiac catheterization lab, he was hypotensive and placed on multiple intravenous medications for hemodynamic support as well as an Impella device. Following medical optimization, he underwent one vessel coronary artery bypass graft as well as surgical excision of the left atrial mass, which pathology had shown to be an atrial myxoma. Conclusion: This patient's case of cardiogenic shock following revascularization was complicated by the identification of an atrial myxoma, which, when large enough, can obstruct blood flow through the mitral valve leading to acute mitral dynamic stenosis. This condition results in circulatory collapse due to obstruction of the left ventricle in diastole as the myxoma occludes the mitral valve. [ABSTRACT FROM AUTHOR]

Stroke presentation secondary to a cardiac myxoma thromboembolism is rare in the pediatric population. Because of such rarity, the reported cases in the literature are primarily case reports. Additionally, general pediatric stroke management lacks evidence-based guidelines because of its low incidence and lack of clinical trials. In pediatric strokes identified from a cardiac myxoma, the incidence favors boys with the classical presentation of unilateral weakness and aphasia. We present a pediatric patient who presented with strokelike symptoms secondary to an intracranial embolus from a previously undiagnosed cardiac myxoma. We performed a systematic review by searching PubMed, Google Scholar, Web of Science, and Embase databases for cases of pediatric myxoma causing stroke (n = 2431) and identified 19 reported uses of surgical management in treating pediatric patients who present with stroke symptoms secondary to a cardiac myxoma thromboembolism. The most common imaging modality was magnetic resonance imaging in 42% of cases, computed tomography in 36.8%, followed by computed tomography angiography in 31.6% of cases. Of these 19 children treated with procedures, 36.8% of pediatric patients aged between 4 and 14 years underwent neurosurgery (n = 7). We describe an urgent mechanical thrombectomy and share preoperative and postoperative images and pathology slides confirming a stroke from myxoma origin. We provide added insight in the safe use of mechanical thrombectomy as treatment for pediatric strokes secondary to a thromboembolism. [ABSTRACT FROM AUTHOR]

Myxoma virus (MYXV) is a Leporipoxvirus (genus) belonging to the family Poxviridae; it is characterised by a genome of approximately 161 kb dsDNA encoding for several proteins that play an essential role in both host spectrum determination and immunomodulation. The healthy reservoir of the virus is Sylvilagus spp. At the same time, in wild and domestic European rabbits (Oryctolagus cuniculus), MYXV is the etiologic agent of myxomatosis, a disease with an extremely high mortality rate. In 2014, an interspecies jump of MYXV was reported in Lepus europaeus in the UK. In 2018, myxomatosis induced by a new recombinant strain called MYXV-To was identified during a large outbreak in Iberian hares (Lepus granatensis) in Spain. Here, we describe the case of myxomatosis in another hare species: an adult male Italian hare (Lepus corsicanus) found dead in 2018 in Sicily with lesions suggestive of myxomatosis and treponema infection. Laboratory tests, e.g., end-point PCR and negative staining electron microscopy, confirmed the presence of both pathogens. MYXV was then isolated from tissue samples in permissive cells and sequenced using NGS technology. Main genomic differences concerning known MYXV strains are discussed. [ABSTRACT FROM AUTHOR]

Background: Pseudomyxoma peritonei (PMP) is a clinical entity of subtle onset abdominal pain, ascites, and distention associated with characteristic imaging. In most cases, laparoscopic exploration will give the definitive diagnosis and histopathologic verification. However, usually there are difficulties in the diagnosis of this disease. Case Report: Herein, we present a case of a 51-year-old female who developed ascites over 5 months. An investigational laparotomy established the diagnosis of PMP, after the discovery of a mucinous, grey-brown tumor that was CK20 positive and CK7 negative. Subsequently, chemotherapy with oxaliplatin combined with 5-FU (FOLFOX4 regimen), was initiated and the patient survived for 30 months. We also present a comprehensive review of the English literature concerning the different symptoms and radiological findings of this rare entity. According to the literature review, 35 cases of PMP with different clinical and radiological findings have been described. In the majority of the cases, ultrasound, computed tomography or magnetic resonance imaging was orientating towards a proper diagnosis before a diagnostic laparotomy. Conclusion: The combination of a clinical picture with the characteristic imaging findings enables a prompt diagnosis of PMP, making prognosis more favorable. [ABSTRACT FROM AUTHOR]

Background: We report a rare case of a giant right atrial myxoma after catheter ablation. Case presentation: A 74-year-old man presented with a fever of unknown origin three years after laser catheter ablation. Multimodal imaging revealed a giant tumor located in the right atrium, which was suspected to be malignant. Surgical resection was performed, and pathological examination revealed that the tumor was a myxoma. Conclusions: Several cardiac myxoma cases after catheter ablation have been reported, suggesting a potential association between myxoma development and catheter ablation-related tissue injury. [ABSTRACT FROM AUTHOR]

Plasma concentrations of a pleiotropic cytokine, interleukin (IL)-6, are increased in patients with cardiac myxoma. We investigated the regulation of IL-6 in cardiac myxoma. Immunohistochemical staining and reverse transcription-polymerase chain reaction (RT-PCR) revealed that IL-6 and its receptors, IL-6 receptor (IL-6R) and gp130, co-existed in the myxoma cells. Myxoma cells were cultured, and an antibody array assay showed that a conditioned medium derived from the cultured myxoma cells contained increased amounts of IL-6. Signal transducer and activator of transcription (STAT) 3 and Akt were constitutively phosphorylated in the myxoma cells. An enzyme-linked immunosorbent assay (ELISA) showed that the myxoma cells spontaneously secreted IL-6 into the culture medium. Real-time PCR revealed that stimulation with IL-6 + soluble IL-6R (sIL6R) significantly increased IL-6 mRNA in the myxoma cells. Pharmacological inhibitors of STAT3 and Akt inhibited the IL-6 + sIL-6R-induced gene expression of IL-6 and the spontaneous secretion of IL-6. In addition, IL-6 + sIL-6R-induced translocation of phosphorylated STAT3 to the nucleus was also blocked by STAT3 inhibitors. This study has demonstrated that IL-6 increases its own production via STAT3 and Akt pathways in cardiac myxoma cells. Autocrine regulation of IL-6 may play an important role in the pathophysiology of patients with cardiac myxoma. [ABSTRACT FROM AUTHOR]

Heart masses, including tumors (primary and secondary) and pseudotumor (cysts and thrombus), are rare entities, but of increasing interest in cardiac imaging areas. The clinical manifestations are related to the intracardiac effect of mass, embolization, and systemic symptoms in the case of tumors; however, some of them are detected incidentally. Nowadays, imaging techniques and the advancement of their tools perform the morphological, functional, and tissue characterization of the masses, and additionally know the anatomical relationships, which are crucial factors for the treatment and surgical planning. [ABSTRACT FROM AUTHOR]

Background: Cardiac myxomas are benign tumours that can occur in any heart chamber or valve. They are extremely rare in dogs. We present a novel case involving a cardiac myxoma in the left ventricular outflow tract (LVOT) and a ventricular septal defect (VSD) in a small dog. Case Description: A female miniature dachshund (age, 7 months; weight, 2.88 kg) presented with growth insufficiency, lethargy, and a cardiac murmur. Echocardiography revealed a small polypoid mass in the LVOT and a membranous VSD. Simultaneous surgeries were performed to resect the mass (aortotomy) and close the VSD (right atriotomy) using low-flow cardiopulmonary bypass with surface-cooling hypothermia and retrograde cardioplegia. The tumour was histopathologically identified as a myxoma. The dog survived with no cardiac complications for 11 years after surgery. Conclusion: To our knowledge, this is the first report of ante-mortem diagnosis and simultaneous surgical repair of a cardiac myxoma obstructing the LVOT and a VSD in a small-breed dog. In addition to describing this complicated case, this report presents what we believe is the first reported use of retrograde cardioplegia during open-heart surgery in a small-breed dog. [ABSTRACT FROM AUTHOR]

Background: Cardiac myxoma (CM) is the most common (58%–80%) type of primary cardiac tumours. Currently, there is a need to develop medical therapies, especially for patients not physically suitable for surgeries. However, the mechanisms that shape the tumour microenvironment (TME) in CM remain largely unknown, which impedes the development of targeted therapies. Here, we aimed to dissect the TME in CM at single‐cell and spatial resolution. Methods: We performed single‐cell transcriptomic sequencing and Visium CytAssist spatial transcriptomic (ST) assays on tumour samples from patients with CM. A comprehensive analysis was performed, including unsupervised clustering, RNA velocity, clonal substructure inference of tumour cells and cell–cell communication. Results: Unsupervised clustering of 34 759 cells identified 12 clusters, which were assigned to endothelial cells (ECs), mesenchymal stroma cells (MSCs), and tumour‐infiltrating immune cells. Myxoma tumour cells were found to encompass two closely related phenotypic states, namely, EC‐like tumour cells (ETCs) and MSC‐like tumour cells (MTCs). According to RNA velocity, our findings suggest that ETCs may be directly differentiated from MTCs. The immune microenvironment of CM was found to contain multiple factors that promote immune suppression and evasion, underscoring the potential of using immunotherapies as a treatment option. Hyperactive signals sent primarily by tumour cells were identified, such as MDK, HGF, chemerin, and GDF15 signalling. Finally, the ST assay uncovered spatial features of the subclusters, proximal cell–cell communication, and clonal evolution of myxoma tumour cells. Conclusions: Our study presents the first comprehensive characterisation of the TME in CM at both single‐cell and spatial resolution. Our study provides novel insight into the differentiation of myxoma tumour cells and advance our understanding of the TME in CM. Given the rarity of cardiac tumours, our study provides invaluable datasets and promotes the development of medical therapies for CM. [ABSTRACT FROM AUTHOR]

Cardiac masses are a significant cause of patient morbidity and mortality by virtue of their symptoms and surgical removal. Preoperative diagnosis of a cardiac mass is usually based on clinical correlation and transthoracic echocardiography findings. Myxomas are the most common benign cardiac tumors, commonly occurring in the left atrium attached to the interatrial septum near the fossa ovalis. Although, at times atypical location and unusual morphology may pose a diagnostic dilemma with 2D echocardiography. 3D echocardiography with its multifaceted advantages, including multiplanar cropping abilities and superior imaging quality can help distinguish between a clot and a myxoma. [ABSTRACT FROM AUTHOR]

Introduction To present, diagnose, and treat a rare case of peripheral odontogenic myxoma occurring in the gingiva of the mandibular molar region. Odontogenic myxomas (OM) are non-cancerous tumors of ectomesenchymal origin, often associated with the tooth-bearing apparatus in both the maxillary and mandibular jaws. The common site of occurrence is the posterior mandible, with a peak incidence in the second to fourth decades of life with female predilection. Peripheral odontogenic myxoma (POM) is usually infrequent, restricted to the soft tissues without involvement of bone, and so is termed an extraosseous counterpart. They grow in an unhurried manner and are considered less aggressive in nature. This case report showcases a rare clinical presentation of peripheral odontogenic myxoma occurring in the gingiva of the mandibular molar region of a 52-year-old male patient. Conclusion Since most of the odontogenic and non-odontogenic neoplasms share similar clinical features, it is very tough to discriminate POM from other tumors. Histopathological analysis assists and lends a helping hand to properly diagnose and treat this benign tumor. Odontogenic myxomas are rare, but they are not uncommon. Though POM is less aggressive in nature, it clinically presents itself as a painless, slow-growing gingival mass that usually interferes with mastication, and minor injuries or trauma during chewing and swallowing could probably lead to ulceration and progressive pain. [ABSTRACT FROM AUTHOR]

Key Clinical Message Understand the importance of considering alternative diagnosis in patients presenting with atypical features, specially when they are not responding to the standard treatment. Understand the importance of considering common presentations of rare cases. Underscoring the critical importance of timely recognition and appropriate management of potentially life‐threatening conditions.

BackgroundMalignant mesothelioma (MM) is a rare and aggressive tumor that is found in the pleura and peritoneum. A few cases of MM in the pericardium and tunica vaginalis testis have been reported. Moreover, primary occurrence in the atrium is extremely rare. The visual appearance of this tumor is similar to that of a common atrial myxoma, which makes it challenging for clinicians and radiologists to diagnose and treat this disease.Case demonstrationAn 18-year-old woman presented with symptoms of chest pain, shortness of breath, cough, and expectoration for 7 days. Echocardiography was performed on the patient, which revealed an atrial mass. Myxoma was one of the differential diagnoses. The tumor was an elliptical mass with tips, and the cut surface was jelly-like, similar to myxoma. After surgery, a pathologic examination of the biopsied tumor confirmed epithelial-type MM. During postoperative follow-up, no recurrence of the tumor was observed.ConclusionsMM originating in the atrium is considered to be extremely rare. Consequently, clinicians can easily misdiagnose atrial MM as a myxoma. Moreover, to confirm the diagnosis, histopathologic biopsy, histomorphological characterization, immunohistochemistry, and molecular genetic testing are required. Therefore, clinical diagnosis and treatment of MM are challenging.

A myxoma is a benign intramuscular gelatinous tumor that is rarely known to arise from the scrotum. It can be confused both clinically and radiologically with other more common scrotal wall lesions such as a sebaceous cyst or an abscess. We report a case of a 54-year-old patient who presented with a scrotal wall swelling that was initially suspected to be an infected cyst or cold abscess on imaging. The final diagnosis of a myxoma was made after surgical excision and histopathological examination.