Benign Myxoma Research Articles

P169 ONE–MONTH RELAPSE OF UNDIFFERENTIATED SARCOMA IN THE LEFT ATRIUM: A CASE–REPORT AND LITERATURE REVIEW

Abstract Background Primary cardiac tumors are extremely rare and mostly benign; among the malignant ones the most frequent are sarcomas (primary cardiac sarcomas, PCS), commonly located in the left atrium. Echocardiography usually provides initial information about tumor location and its size, although the correct diagnosis may be hindered by similarities to benign myxomas or thrombotic formations. Therefore a multimodality approach, with second level imaging techniques, is frequently needed. Case–report: We report a case of undifferentiated cardiac sarcoma in a 47–year–old woman who underwent surgical resection. One month after the operation the control transthoracic echocardiography (TTE) revealed disease recurrence, confirmed by Positron emission tomography (PET)–computed tomography (CT). The patient underwent chemotherapy and hypofractionated radiotherapy, with significant reduction of mass size after 2 months. Conclusions PCS is a very rare disease, predominantly affecting adults in the fifth decade of life, with a very poor prognosis. Symptoms depend on the size and location of the tumor and may be nonspecific up to the advanced stages. Diagnosis may be suspected by TTE or CT but histological diagnosis is often essential. There is no standard treatment. Surgery is potentially the best treatment option, followed by radiotherapy, chemotherapy and immunotherapy targeted to the specific histologic subtype of the tumor, but further studies are needed.

Evaluation of tumour associated macrophages in different histopathological types and grades of canine mammary tumours

Canine mammary tumours (CMTs) are the second highest reported tumours in female dogs, following skin tumours. Human breast cancers (HBCs) and CMTs share common clinical and molecular features and hence, CMTs are considered as ideal models to study the different aspects of HBC. The study utilised samples from 25 CMT suspected cases presented to University Veterinary Hospitals in Thrissur district from December 2020 to October 2021. The tumour samples were analysed histopathologically and the lesions were classified. Among the 25 cases, one was identified as ductal hyperplasia, one as a benign myxoma and all the others were found to be malignant neoplasms. Malignant tumours were further categorised into different histotypes.Histological Malignancy Grading (HMG) was also done in 23 malignant CMTs and 21.74 percent were found to be of grade I, 47.83 per cent were grade II and 30.43 per cent were grade III. Majority of the malignant tumours were simple carcinomas which comprised tubulopapillary, ductal, cribriform, solid and comedocarcinomas. Highly aggressive tumours like cribriform, solid, comedo and inflammatory carcinomas belonged to higher grades, either II or III. Infiltration of tumour associated macrophages (TAMs) was studied in different histotypes and grades of CMTs. It was identified that malignant high grade CMTs had greater TAM infiltration and hence, with further validation TAMs could be effectively used in predicting prognosis and also as a therapeutic target.

A Rare Case of Primary Cardiac Undifferentiated Pleomorphic Sarcoma with Brain Metastases

Abstract Introduction/Objective Undifferentiated pleomorphic sarcomas are highly malignant undifferentiated fibroblastic tumors with an aggressive clinical course. Primary cardiac undifferentiated pleomorphic sarcomas are extremely rare (incidence < 0.1%) and most commonly occur in the left atrium. We report a case of a left atrial undifferentiated pleomorphic sarcoma that was thought to be a cardiac myxoma on imaging studies. Methods/Case Report A 22-year-old female without past medical or family history presented to the emergency department for a cerebrovascular accident. During clinical work up, transesophageal echocardiogram revealed a 3 cm mass attached to left atrial septum and a 1.8 cm mass attached to the interatrial septum with associated thrombus formation. The patient underwent surgical excision of the mass. Histological analysis revealed a tumor comprised of spindle and epithelioid cells with abundant nuclear atypia, mitotic figures, and necrosis. Extensive immunohistochemical analysis did not show specific lineage and the tumor was classified as an undifferentiated pleomorphic sarcoma. Two months after initial surgery, the patient presented with additional neurologic deficits and brain lesions. The pathologic findings in the brain biopsy specimen were identical to those in the cardiac specimen and consistent with metastatic undifferentiated pleomorphic sarcoma. This case provides insight into the metastatic behavior of high-grade cardiac sarcomas and their unusual clinical and radiographic presentations, including neurologic manifestations that are unique to the primary cardiac location of the tumor. Results (if a Case Study enter NA) NA Conclusion In conclusion, because the majority of cardiac undifferentiated pleomorphic sarcomas occur in the left atrium, these growths can be mistakenly diagnosed as benign myxoma preoperatively. This case provides insight into the metastatic behavior of high-grade cardiac sarcomas and their unusual clinical and radiographic presentations, including neurologic manifestations that are unique to the primary cardiac location of the tumor.

Миксома аппендикса в послеродовом периоде в практике гинеколога

Myxoma (mucocele) of the appendix is a cyst of the appendix containing mucus of various consistency (from watery to jelly-like) with chronic inflammation of the appendix with cicatricial narrowing of the base of the appendix, compression and obliteration of its lumen. It is a rare peculiar neoplasm of the appendix, and occurs in 0.02–0.5 % of all appendectomy, common in women of reproductive age. Myxoma is often found as an incidental, asymptomatic finding in 25 % of cases. The diagnosis of myxoma of the appendix is established only intraoperatively. Currently, the most precise method for the diagnosis and treatment of benign myxoma is laparoscopy. This article presents a clinical case of appendix myxoma diagnosed in a patient in the postpartum period, in which the method of laparoscopic salpingectomy and appendectomy was applied to verify the diagnosis and treatment. This clinical case once again proves that there are no certain pathognomonic signs characteristic of appendix myxoma, including in the postpartum period, therefore, careful history taking, in-depth diagnostic examination methods (ultrasound, CT, MRI) and timely use in the diagnosis of laparoscopic methods in patients with appendix myxoma. This will make it possible to competently conduct differential diagnostics, avoid errors, both in diagnosis and in the treatment of these patients, reduce the stay in a hospital bed, and thereby, most importantly, improve the quality of life of a woman

An exceptionally giant left atrial myxoma: a case report and literature review.

BackgroundCardiac myxomas are considered the most common benign heart tumours. The clinical manifestations mainly depend on the size of the tumour. They usually vary from asymptomatic, mild non-specific symptoms, to severe obstructive cardiac and systemic findings. We describe herein a significantly large left atrial myxoma in a patient misdiagnosed with respiratory asthma.Case summaryA 54-year-old lady, was diagnosed previously with asthma, presented with a history of dyspnoea on exertion, palpitations, and mild peripheral oedema. Chest X-ray suggested pulmonary congestion. Due to high suspicion of cardiac issues, transthoracic echocardiography was done revealing giant left atrial mass. Consequently, the mass was approached and excised surgically through the inverted T biatrial incision. Grossly, the mass measured 10 × 8 × 6 cm, and it had a smooth surface and was filled with gelatinous material. The histopathology confirmed benign myxoma without malignant features.DiscussionOur article mainly focuses on the diagnostic challenges of a patient with atrial myxoma. The major discrepancy between the tumour size and the severity of the patient’s symptoms should draw physicians’ attention to consider atrial myxoma over a long list of differentials, in order to take immediate action to reduce the mortality and improve the overall prognosis.

Primary cardiac myxofibrosarcoma with osteoid differentiation mimicking a left atrial myxoma: A rare entity

Cardiac myxofibrosarcoma (MFS) is an uncommon entity. It is among the most challenging conditions to diagnose due to its rarity, high variability, and non-specific findings. These tumors often simulate left atrial myxoma or mitral stenosis at clinical presentation. Although, the definitive diagnosis of cardiac tumors depends on histopathological examination, various imaging techniques are also useful to study tumor characteristics to plan an appropriate treatment strategy. Here we highlight a case of primary cardiac MFS of left atrium (LA) showing areas of transition to undifferentiated pleomorphic sarcoma (UPS) with bone or osteoid formation, which is extremely rare and not well described.<Learning objective: Primary cardiac myxofibrosarcoma (MFS) is a rare and aggressive cardiac tumor. It is often confused with benign myxoma, leading to a delay in initiation of treatment. This delay can often lead to poor clinical outcomes. Our study will guide clinicians in early diagnosis, treatment, and counseling of patients with this rare entity. Echocardiography, together with magnetic resonance imaging, histology, and immunohistochemistry are essential in the diagnosis of MFS.>

THE TUMOR PLOP WHO CRIED WOLF: A CASE OF METASTATIC CARDIAC ANGIOSARCOMA

SESSION TITLE: Monday Abstract Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Cardiac sarcomas are extremely rare, primary malignant tumors arising from the heart or great vessels. Due to their complex nature and infrequency, there is often a delay in diagnosis and thus, a dismal prognosis. Here, we review the case of a patient who presented with a constellation of symptoms that, over many weeks, led to the final diagnosis of metastatic cardiac angiosarcoma. CASE PRESENTATION: Our patient is a 63 year-old female with history of type 2 diabetes. She was undergoing an outpatient workup for multiple myeloma and was found to have worsening anemia, renal function and hip pain, secondary to a pathologic ischial fracture. On initial presentation, her chief complaint was progressive dyspnea. Physical exam was notable for tachypnea and tachycardia, and she was found to have an elevated D-dimer. CT angiogram of her chest was negative for pulmonary embolism, however, revealed a large left atrial mass with possible extension into the ventricle and bilateral pleural effusions. The multi-lobulated mass measured approximately 3 x 4 cm on echocardiogram. Secondary metastasis to the heart or primary benign myxoma was initially suspected. Further study with cardiac MRI determined that the mass was centered over the ostium of the left inferior pulmonary vein and covered in thrombus, thus favoring the diagnosis of sarcoma. Biopsy of the pathologic hip fracture was also consistent with metastatic sarcoma. Upon further staging, she was noted to have several abdominal soft tissue nodules, scattered lytic bony lesions, and a filling defect within the proximal jejunum. Initial treatment consisted of diuresis, steroids, bisphosphonates, as well as Lovenox for the associated thrombus. Later, she developed sharp abdominal discomfort and was found to have jejunal intussusception. EGD revealed an adjacent duodenal ulcer and biopsy with immunohistochemical studies was consistent with metastatic cardiac angiosarcoma. Given the location and size of the atrial tumor, surgical resection was not an option. The patient was started on a trial of olaratumab, typically used in soft-tissue sarcomas, and doxorubicin with possible future palliative radiation. Over the next six months, after multiple admissions with various complications and worsening pain, she elected to pursue hospice care. She passed away peacefully at home, approximately one year after her initial presentation. DISCUSSION: This case illustrates a complex case of metastatic cardiac angiosarcoma. Given the late presentation with distant metastasis to various sites within the abdomen and bone, it serves as an example of the devastating effects this rare form of cancer can have. CONCLUSIONS: The complex location of the mass and its paroxysmal extension through the mitral valve into the left ventricle likely caused transient mitral regurgitation and pulmonary hypertension, which likely caused her initial dyspnea on presentation. Reference #1: Patel SD, Peterson A, Bartczak A, et al. Primary cardiac angiosarcoma - a review. Med Sci Monit. 2014;20:103-9. Published 2014 Jan 23. https://doi.org/10.12659/MSM.889875 DISCLOSURES: No relevant relationships by Logan Rhea, source=Web Response

Atrial Myxoma with Glandular Differentiation – A Case Report

Benign cardiac myxoma with glandular differentiation is an extremely rare subtype of cardiac tumors which can mimic myxosarcoma grossly and histologically. Primary cardiac tumors are extremely rare and account for less than 0.2% of all tumors. They are benign in 75% of cases and almost 50% of these are myxomas. The differentiation between benign and malignant cardiac tumors is challenging by echocardiography or histologically. We report a case of primary left atrial benign cardiac myxoma mimicking myxosarcoma grossly and histologically by its glandular differentiation. Only 39 such cases reported in the literature.

Left Atrial Metastasis Presenting as Right-sided Heart Failure in a Patient with Acute T-cell Lymphoblastic Leukemia

Background: Cardiac metastasis is usually seen with lung carcinomas or malignant melanoma, but rarely with leukemia, and is usually a post-mortem diagnosis. Secondary cardiac tumors arising from the left atrium, where benign myxomas predominate, are uncommon. Case presentation: We report a 22-year old Filipino male, recently diagnosed with acute T-cell lymphoblastic leukemia, presenting with right-sided heart failure symptoms. On work-up, transthoracic echocardiogram showed a mass with mixed echogenicity, irregular borders and broad-base attachment to the anterior and posterior walls of the left atrium. He was treated with nitrates, ivabradine, captopril and digoxin; and subsequently underwent chemotherapy with vincristine, doxurubicin, intrathecal methotrexate and cytarabine. Right-sided heart failure symptoms improved after initiation of chemotherapy. Resolution of the left atrial tumor was documented after completion of induction chemotherapy. Conclusion: Cardiac metastasis should be considered in patients with acute lymphoblastic leukemia presenting with right-sided heart failure. Chemotherapy and supportive cardiac medications should be considered in these cases. Other treatment options should also be investigated.

Gangrenous Cecal Volvulus; A Case Report and Review of Literature

Primary cardiac neoplasms are very rare as compared to metastatic tumors. 70% to 80% of them are benign myxomas. Complications of myxomas include cyst and microabscess formation, embolization, syncope and sudden death. Rarely, cardiac myxomas are associated with primary nodular adrenal cortical disease, mammary fibroadenomas, testicular tumors or pitiutary adenomas with gigantism or acromegaly known as Carney’s syndrome. We present a patient with a left atrial myxoma who underwent reoperation associated with Carney’s syndrome.

Giant Right Atrial Myxoma: The Importance of Transesophageal Echocardiography during Diagnosis, Evaluation, and Resection

ABSTRACT Most cardiac tumors are benign myxomas, and are most commonly found in the left atrium. Such tumors are identified either during symptomatic workup or found incidentally. We present a case in which a patient with recurrent transient ischemic attacks and syncope was found to have a giant right atrial myxoma with subsequent right atrial outflow obstruction. The mass was initially diagnosed on transthoracic echocardiography and its full scope was detailed utilizing transesophageal echocardiography (TEE). With swift intervention, the mass was successfully removed with the help of TEE guidance and the patient made a full recovery. The importance of TEE both preoperatively and intraoperatively during resection of giant cardiac masses is highlighted. How to cite this article Ungerman E, Haft W. Giant Right Atrial Myxoma: The Importance of Transesophageal Echocardiography during Diagnosis, Evaluation, and Resection. J Perioper Echocardiogr 2016;4(2):74-78.

Familial atrial myxoma in three generations: case report

Familial myxomas constitute 7 % of the total myxoma population exhibiting Mendelian autosomal dominant inheritance. We present a rare case of autosomal dominant benign familial myxoma occurring in three generations of the family operated successfully at our institute. A 40-year-old female presented with chest pain and palpitation, was diagnosed with left atrial myxoma, and was operated in 1993. After 2 years of surgery, the patient developed recurrent myxoma, and excision was done in 1995. A 22-year-old son complained of dyspnoea and chest pain. On evaluation, 5.6 × 2.6 cm right atrial myxoma attached to the roof of the right atrium was found, and the patient underwent myxoma excision in 2007. A 3-year-old granddaughter suffered from right hemiplegia. Echocardiography revealed left atrial myxoma 3 cm × 2 cm pedunculated lesion causing diastolic obstruction of mitral inflow. Operated patients in all three generations and other siblings have been genetically screened. On genetic evaluation, all diseased had autosomal dominant single-gene defect or microdeletion. Atrial myxomas presented at earlier age successively and in more aggressive form in these three generations.

A Voluminous Right Atrial Myxoma Revealed by Right Side Heart Failure

Mini‐AbstractRight atrial myxoma is rare. Its clinical presentation is polymorphic. The most common presentation is heart failure, but could be pulmonary embolism or tricuspid valve obstruction. We report here, the case of a 60‐year‐old man admitted for severe heart failure. The echocardiogram showed a voluminous and floating right atrial mass, appending to the interatrial septum and protruding into the right ventricle causing severe tricuspid regurgitation. Chest computed tomography scan demonstrated multiple distal pulmonary emboli. Surgical excision and tricuspid annuloplasty were successfully performed. Histological findings were typical of a benign myxoma. At follow‐up, there was a complete regression of heart failure signs.

A primary cardiac osteosarcoma: Case report and review of the literature

Primary cardiac osteosarcoma is a rare and aggressive neoplasm that can be difficult to diagnose. We report a case of a previously healthy 49-year-old woman who presented with dyspnea, atrial flutter, and heart failure. A mass was visualized in her left atrium by echocardiography and cardiac computed tomography, and the diagnosis of cardiac myxoma was raised. The patient subsequently underwent surgical resection of the mass and atrial reconstruction. Surprisingly, histological and immunohistological analyses revealed the mass to be an osteosarcoma. The patient received chemotherapy and radiotherapy. Eight months later, she has shown evidence of local recurrence. We briefly discuss primary osteosarcomas in the cardiac cavity and their management.<Learning objective: Primary cardiac tumors are very rare and most likely benign. Malignant tumors constitute less than 25% of primary cardiac neoplasms. However, both primary sarcomas and benign tumors are often found in the left atrium. As a consequence of their location and similar clinical presentation, primary cardiac sarcomas can be easily confused with a benign myxoma, therefore abnormal imaging features (immobility of the mass, neovascularity, multicentricity, calcification and invasion into the heart structures) should raise suspicion for a cardiac sarcoma.>

Giant left atrial myxoma causing severe pulmonary hypertension.

This report describes a case of giant left atrial myxoma in a 43-year-old woman. She presented with general malaise and dyspnea. Electrocardiogram showed severe right-axis deviation. Transthoracic echocardiography demonstrated a giant mobile mass with size of 6.5×4.5cm in the left atrium. This mass protruded into the left ventricle in diastole and disturbed transmitral left ventricular filling flow. Bilateral atrium and right ventricle were dilated. Severe tricuspid regurgitation was demonstrated with peak pressure gradient of 91mmHg. This mass was surgically excised and was diagnosed as benign atrial myxoma by histological analysis. Pulmonary hypertension was improved after surgery.

Fibroelastomas of the left ventricular outflow tract

Primary cardiac tumours are uncommon and most of them are benign myxomas. Only one-tenth of the tumours are fibroelastomas of the cardiac valves. We report two cases with a longstanding history of valvular heart disease where a diagnosis of fibroelastoma of the left ventricular outflow tract was confirmed by histology.

A rare diagnosis of multiple hemorrhagic metastases in brain

A 30-year-old female presented with an episode of generalized tonic-clonic convulsions. The imaging revealed multiple hemorrhagic lesions in the brain. There was no significant past history except for a cardiac surgery two years ago, for a benign atrial myxoma. The diagnosis of probable metastases was thought of and a thorough workup was planned, to determine the primary. Surprisingly, no primary lesion was detected, and therefore, as a histological diagnosis was required, she underwent navigation-guided excisional biopsy of one of the lesions. The histopathology proved that it was a myxomatous lesion. Thus, a rare diagnosis of atrial myxoma presenting as hemorrhagic metastases of the brain was formed. Atrial myxoma is a benign tumor of the heart. It can embolize distally and can present with known embolic/ischemic manifestations. However, presentation as hemorrhagic mass lesions, involving multiple areas of the brain, after many years of complete surgical excision, is quite rare and is rarely considered as a differential diagnosis. Hence, such a possibility should be kept in mind and careful primary cardiac intervention, with preventive measures to minimize the chances of distal embolization should be undertaken. As only few such cases have been reported worldwide, no treatment protocol has been devised at present, and a close follow-up of these patients is warranted.

Clinical Experience of the Surgical Treatment of Cardiac Tumor

Background: Primary cardiac tumors are extremely rare. The most common type are benign myxomas, and these are almost completely curable with early surgery. Malignant tumors, however, such as sarcomas, are difficult to remove surgically, and their prognosis is known to be poor. In this study, data on patients who had undergone surgical treatment of cardiac tumor in the authors’ hospital were collected and analyzed. Material and Method: The subjects included 28 patients who had undergone surgical treatment of cardiac tumor from August 1993 to December 2008. Their medical records were reviewed and retrospectively analyzed. Result: The patients were aged from 20 to 76 years (mean age: 54.2±15.6), and 11 were male (39%) and 17 female (61%). Fifteen of them (54%) underwent emergency surgery to improve heart failure symptoms. The most common preoperative symptom was dyspnea (15 cases, 54%). Preoperative echocardiography was performed on all the patients. The average size of the tumor as measured during the operation was 7.0±6.9 ㎝ (the average length of the long axis was 2~40 ㎝), and the sites of tumor attachment were the interatrial septum (18 cases, 64%), the left atrium (9 cases, 32%), the mitral valve annulus (2 cases, 7%), and the left ventricle (2 cases, 7%). The operation was performed with an incision through both atria in all the patients, and a complete excision was made in 25 cases (89%). According to the biopsy results, there were 4 cases of sarcoma (14%), 1 case of lipoma (4%), and 23 cases of myxoma (82%). The three cases in which the tumors were not completely excised were sarcomas. No operative deaths occurred after the operations. Outpatient follow-up was possible for 24 cases (86%), with a mean follow-up period of 46.8±42.7 months. Late death occurred in 3 of the 24 patients; each of these patients had sarcomas. Of these patients, the first had undergone two repeat surgeries, the second had metastatic sites removed, and the last had only chemotherapy. The average recurrence time was 12.7±10.8 months, and the average metastasis time was 20.5±16.8 months. Conclusion: Most cardiac tumors are benign myxomas. In principle, they should be surgically treated because they can create risks such as embolism, and can be radically treated when surgically removed. In most cases, however, malignant sarcomas are already considerably advanced with severe infiltration into the neighboring tissues at the time of diagnosis. The surgical removal of malignant sarcomas is known to be difficult because of the advanced stage and degree of infiltration. We suggest that excision of the removable portion of the tumor sites to alleviate symptoms such as heart failure can improve quality of life.

Tumors of the Heart

Cardiac Tumors are very rare but have devastating consequences given that they involve such an important organ. The majority of tumors are benign myxomas, which can present in very subtle ways causing a subsequent delay in diagnosis. Routine echocardiography is advised for anybody who is feeling generally unwell, since myxomas can cause pyrexia of unknown origin. The use of cardiac imaging has increased the early diagnosis of these tumors. A total of 25% of cardiac tumors are malignant sarcomas and these have a highly aggressive behavior. Early detection of these tumors while they are resectable offers a possibility for cure in the future.

Large asymptomatic Left Atrial Myxoma with ossification: case report

BackgroundAtrial myxomas are the most common primary cardiac tumors. They are usually small or moderate in size by the time of the diagnosis, exhibiting non specific cardiac or systemic symptoms, and are most frequently soft and friable without microscopic signs of ossification. We describe herein an extremely rare case of an asymptomatic giant left atrial myxoma with angiographic neovascularization and ossification.Case presentationAn asymptomatic 58-year-old male with a giant left atrial tumor, was transferred to our Unit for surgical treatment. The tumor was an incidental finding during a work-up for hemoptysis due to bronchectasis. The coronary angiogram showed tumor vessels originating from the RCA. The tumor macroscopically did not resemble a myxoma, considering its dimensions (12 × 10 cm) and its solid substance. The mass was excised together with the interatrial septum and the right lateral LA wall close to the right pulmonary veins orifices. The defect was closed with Dacron patches in order to prevent malformation of both atria. The pathology study revealed a benign myxoma with excessive osteoid (mature bone) content.ConclusionWe consider our case as extremely rare because of the asymptomatic course despite the large size of the tumor, the blood supply from the right coronary artery and the bone formation.