Adjuvant chemoradiation for high-grade cardiac leiomyosarcoma in a child: Case report and review of literature.

A 13-year-old healthy girl presented with dizziness and palpitations, found to have a left atrial mass. An 8-cm tumor was removed en bloc. Pathology confirmed grade 3 leiomyosarcoma with multifocal positive margins. She received adjuvant ifosfamide and doxorubicin, followed by concurrent proton radiotherapy and ifosfamide. Radiotherapy included 66 Gy (RBE) in 33 fractions to the operative bed. Prospectively graded toxicities included Grade 2 esophagitis and Grade 1 anorexia, dermatitis, and fatigue. She completed six cycles of ifosfamide. Two years post operation, she had no evidence of disease, intermittent palpitations with normal cardiac function, and no other cardiopulmonary or esophageal symptoms.

Pediatric Extraskeletal Ewing Sarcoma Originating in the Heart: A Case Report and Review of the Literature.

Extraosseous Ewing sarcoma of primary cardiac origin is an extremely rare variety among pediatric cardiac neoplasms. We report a case of extraosseous Ewing sarcoma of primary cardiac origin in a 9-year-old girl, treated with debulking surgery, adjuvant chemotherapy, and radiotherapy.

CYP1B1 as a therapeutic target in cardio-oncology.

Cardiovascular complications have been frequently reported in cancer patients and survivors, mainly because of various cardiotoxic cancer treatments. Despite the known cardiovascular toxic effects of these treatments, they are still clinically used because of their effectiveness as anti-cancer agents. In this review, we discuss the growing body of evidence suggesting that inhibition of the cytochrome P450 1B1 enzyme (CYP1B1) can be a promising therapeutic strategy that has the potential to prevent cancer treatment-induced cardiovascular complications without reducing their anti-cancer effects. CYP1B1 is an extrahepatic enzyme that is expressed in cardiovascular tissues and overexpressed in different types of cancers. A growing body of evidence is demonstrating a detrimental role of CYP1B1 in both cardiovascular diseases and cancer, via perturbed metabolism of endogenous compounds, production of carcinogenic metabolites, DNA adduct formation, and generation of reactive oxygen species (ROS). Several chemotherapeutic agents have been shown to induce CYP1B1 in cardiovascular and cancer cells, possibly via activating the Aryl hydrocarbon Receptor (AhR), ROS generation, and inflammatory cytokines. Induction of CYP1B1 is detrimental in many ways. First, it can induce or exacerbate cancer treatment-induced cardiovascular complications. Second, it may lead to significant chemo/radio-resistance, undermining both the safety and effectiveness of cancer treatments. Therefore, numerous preclinical studies demonstrate that inhibition of CYP1B1 protects against chemotherapy-induced cardiotoxicity and prevents chemo- and radio-resistance. Most of these studies have utilized phytochemicals to inhibit CYP1B1. Since phytochemicals have multiple targets, future studies are needed to discern the specific contribution of CYP1B1 to the cardioprotective and chemo/radio-sensitizing effects of these phytochemicals.

Treatment of recurrent isolated right atrial metastatic cavitary mass from breast cancer with radiation therapy: A case report and review of literature.

The heart is a destination for metastases of various types of primary tumors, with lung, breast, melanoma, and mesothelioma being the most common types. Recurrent isolated cardiac metastasis presenting as a cavitary mass is rare. We present a case of recurrent isolated cavitary metastatic mass to the right atrium in a patient with the history of breast cancer who presented with new-onset atrial fibrillation. The patient successfully received radiation therapy with the resolution of the mass confirmed on repeat echocardiography.

Minimal invasive cardiac mass resection, post-thoracic radiotherapy: a safe approach?

Minimal invasive cardiac surgery by right mini-thoracotomy for cardiac mass resection has emerged as an alternative to median sternotomy, for being less associated to postoperative complications and a faster recovery. Thoracic radiotherapy, widely used for cancer treatment, can result in pulmonary adhesions making it impossible to access the heart by thoracotomy. We report a case of a patient submitted to bilateral thoracic radiotherapy, with a cardiac mass in the left atrium, successfully treated by surgical resection, as well the intraoperative procedure done to make the minimally invasive approach possible.

A cirurgia cardíaca minimamente invasiva por mini-toracotomia direita para ressecção de massas cardíacas surgiu como alternativa à esternotomia mediana convencional, por se encontrar associada a menos complicações no pós-operatório e a uma recuperação mais rápida. A radioterapia torácica, muito utilizada como adjuvante no tratamento do cancro da mama, pode resultar em adesões pulmonares que tornam difícil o acesso ao coração por toracotomia. Reportamos o caso clínico de uma doente submetida a radioterapia torácica bilateral, com posterior diagnóstico de uma massa na aurícula esquerda, submetida a ressecção cirúrgica, assim como o procedimento realizado para tornar a abordagem minimamente invasiva possível.

A clinical example of extreme dose exposure for an implanted cardioverter-defibrillator : Beyond the DEGRO guidelines.

INTRODUCTION: Considering that the number of malignant diseases in patients over 65 years of age is increasing, it often occurs that patients who carry a cardiac implanted electronic device must undergo radiotherapy. Ionizing radiation can disturb the function of the implantable cardioverter-defibrillator (ICD). As a result of this, an update of the DEGRO/DKG guidelines for radiotherapy of this patient group has been published. METHODS: We report the case of a patient with an ICD and T­lymphoblastic lymphoma with cardiac involvement, who received i.a. a total body irradiation with 8 Gy followed by a consolidating radiotherapy of the pericardium with 14 Gy as well as additional radiotherapy courses after consecutive recurrences. For the purposes of the treatment, the antitachyarrhythmia (ATA) therapy was deactivated and temporarily replaced through a life vest. RESULTS: According to the current DEGRO guidelines for irradiation of patients with cardiac implanted electronic devices, a categorization of the patient in the "high-risk" group was made. Furthermore, regular telemetric checks of the ICD device were performed before and after treatment. Despite unavailable declaration of the manufacturer regarding the cumulative tolerable dose and DEGRO recommendation for a cumulative dose <2 Gy, the aftercare was unproblematic and normal values were assessed for all relevant ICD parameters, despite a cumulative dose >10 Gy in the device. CONCLUSION: This case shows that if the cardiac implanted electronic devices are not directly irradiated und the energy used is reduced to 6 MV, irradiation-induced damage is less likely and can possibly be prevented.

Cardiac Metastasis of Nonvisceral Soft-tissue Leiomyosarcoma.

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

Successful en bloc venous resection with reconstruction and subsequent radiotherapy for 2 consecutive recurrences of intravenous leiomyoma--a case report.

BACKGROUND: Intravenous leiomyomas are a rare variant of uterine leiomyoma. Although histologically benign, these tumors are associated with a poor prognosis due to propensity for metastasis, high recurrence rate, difficulty of obtaining complete resection, and frequent extension into and along major veins. CASE PRESENTATION: We describe a 43-year-old patient initially presenting with lower abdominal pain. Clinical examination revealed a large right pelvic mass that was shown by computed tomography (CT) to surround the right external iliac vein, right common iliac vein and distal inferior vena cava. The patient had a history of total abdominal hysterectomy with bilateral ovarian cystectomies for uterine leiomyoma approximately 3 years prior to her presentation. Her past surgical history also included removal of an ovarian endometriosis cyst and right hydrosalpinx. The patient underwent an exploratory laparotomy. Operative findings included complete occlusion of the right iliac vessels and distal vena cava by a large tumor that filled the pelvis and extended to the level of the right kidney. The mass was resected en bloc with the involved veins and synthetic vascular grafts were placed. This highly technical procedure was complicated by hemorrhage requiring a total of 32 units of red blood cells and 2.0 L of plasma. Pathologic examination confirmed intravenous leiomyoma. On Immunohistochemical staining, the tumor cells were positive for CD32, CD34, Vimentin and smooth muscle actin. Eight months after this procedure, the patient again presented with an abdominal mass. She was diagnosed with a pelvic recurrence and noted to have intravascular extension into the left iliac vein and inferior vena cava. For this tumor she underwent radiation treatment with three-dimensional conformal radiation therapy (total dose 4500 cGy). The tumor gradually decreased in size during follow-up and became undetectable by CT. CONCLUSIONS: Surgical excision is the mainstay of treatment of intravenous leiomyoma. Radiation therapy may be an effective alternative in patients with unresectable disease or poor surgical candidates.

Postoperative radiotherapy for high-grade primary cardiac spindle cell sarcoma: a rare entity.

A patient in her early 20s presented with blood-stained sputum and shortness of breath. Initially, she was treated for pneumonia. Later, upon exacerbation of symptoms, further investigations were done which exhibited a left atrial mass causing compression of contralateral atrium. She underwent surgical resection of the mass, which was initially mistaken as myxoma. However, histopathological correlation revealed spindle cell sarcoma with focal myogenic differentiation. This case report highlights the role of Radiation Therapy in adjuvant setting with promising impact on improving local control after R2 resection. Cardiac spindle cell sarcoma, being one of the rarest cardiac tumours reported to date, warrants establishment of a Rare Tumour Multidisciplinary Team for management of such malignancies.

Cardiac angiosarcoma treated with resection and adjuvant radiation therapy.

Primary cardiac angiosarcoma is a rare and aggressive tumor with a remarkably high rate of metastasis. Due to the high rate of metastasis, surgical resection is often limited and survival expectations modest. This report describes the case of a 35-year-old African-American woman with initially non-metastatic primary right atrial angiosarcoma who was treated with surgical resection of the primary tumor, reconstruction of the right atrium, and patch repair of the atrial septum via Gore-Tex patch. She underwent post-operative radiation therapy to the atrial bed with curative intent. However, after eight months of symptom-free survival, she presented with extensive metastases within bone and the right retroperitoneum without local recurrence. Palliative radiation treatment commenced for the metastases in the retroperitoneum and cervical spine.

Eleven cases of cardiac metastases from soft-tissue sarcomas.

OBJECTIVE: Cardiac metastasis is a highly life-threatening condition because it leads to cardiac failure. However, it is difficult to diagnose because its precise clinical features are unknown. Here, we report 11 cases of cardiac metastasis from soft-tissue sarcoma, and discuss its diagnosis and treatment. METHODS: Of 641 patients with soft-tissue sarcoma treated in our institute between 1996 and 2009, we retrospectively reviewed the medical records of 11 patients whose cardiac metastases were diagnosed while they were alive. RESULTS: The most common primary tumor was leiomyosarcoma (n= 5), followed by clear cell sarcoma (n= 2). In all cases, metastases to other organs, including lungs (n= 10), soft tissues (n= 5) and bones (n= 4) were found along with cardiac metastases. Cardiac metastasis was diagnosed by echocardiography in six cases and by computed tomography in four cases. In four patients, cardiac metastasis was not detected by chest computed tomography as follow-up to lung metastases and echocardiography was required to make the diagnosis. Although five patients complained of exertional dyspnea, four were asymptomatic. Seven cases were treated with radiotherapy. No patient had surgery for their cardiac metastasis. The median survival of patients who received radiation therapy was 10.5 months; that of those who did not was 3.5 months. CONCLUSIONS: Cardiac metastasis is often asymptomatic. Echocardiography is better than computed tomography for diagnosing cardiac metastasis, and should be considered in all patients presenting with soft-tissue metastases. Owing to the highly life-threatening nature of cardiac metastases and the possibility of soft-tissue dissemination, treatment with radiation therapy is recommended immediately on diagnosis.

Treatment of a heart base tumor and chylothorax with endovascular stent, stereotactic body radiation therapy, and a tyrosine kinase inhibitor in a dog.

An 8-year-old 28-kg male castrated rough collie was evaluated for persistent chylothorax secondary to right atrial mass. Cardiac ultrasound and computed tomography revealed a right atrial intra- and extraluminal mass with partial obstruction of the cranial vena cava and secondary chylothorax. Vascular stent placement was elected to alleviate cranial vena cava obstruction and secondary chylothorax. An 18 mm × 180 mm self-expanding stent was deployed in the region of the stricture, spanning the cranial vena cava and right atrium. An intrathoracic drainage catheter and subcutaneous port were placed within the right hemithorax, and antiplatelet therapy was initiated. Four weeks later, the dog underwent stereotactic body radiation therapy. Three months following treatment, the dog was diagnosed with supraventricular tachycardia and received antiarrhythmic therapy and antiangiogenic/antiproliferative medication (Palladia™). Subsequent evaluations confirmed the resolution of arrhythmia and pleural effusion. Combined vascular stent placement and stereotactic body radiation therapy for the treatment of a right atrial intraluminal and extraluminal mass leading to cranial vena cava compression and subsequent chylothorax may lead to long-term survival. A good outcome was achieved in this patient due to resolution of pleural effusion, as well as cytoreduction and presumably delayed progression of tumor growth.

MR-guided stereotactic body radiation therapy for primary cardiac sarcomas.

BACKGROUND: Primary cardiac tumors are an extremely rare disease with limited prognosis. The treatment of choice is surgery. Other treatment options include chemotherapy and radiation therapy, which historically represented a palliative approach in patients who were not eligible for surgery. The development of hybrid MR-guided radiation therapy makes it possible to better visualize cardiac lesions and to apply high doses per fraction in sensible organs such as the heart. CASE PRESENTATION: Patients affected by inoperable primary cardiac sarcomas and treated at two different institutions were considered for this analysis and retrospectively analyzed. All patients were treated using a 0.35 T hybrid MR Linac system (MRIdian, ViewRay Inc., Mountain View, CA). In the present study we investigated the feasibility, early outcome and toxicity of MR-guided RT in primary cardiac sarcomas. Four consecutive non-metastasized patients who were treated between 05-09/2020 were analyzed. The cardiac sarcomas were mostly located in the right atrium (50%) and one patient presented with 3 epicardial lesions. All patients received MRgRT as a salvage treatment for recurrent cardiac sarcoma after initial surgery, after a mean interval of 12 months (range 1-29 months). Regarding the treatment characteristics, the mean GTV size was 22.9 cc (range 2.5-56.9 cc) and patients were treated with a mean GTV dose of 38.9 Gy (range 30.1-41.1 Gy) in 5 fractions. Regarding feasibility, all treatments were completed as planned and all patients tolerated the treatment very well and showed only mild grade 1 or 2 symptoms like fatigue, dyspnea or mild chest pain at early follow-up. CONCLUSION: To the best of our knowledge, in this retrospective analysis we present the first and largest series of patients presenting with primary cardiac sarcomas treated with online adaptive MRgRT. However, further studies are needed to evaluate the impact of this new methodology on the outcome of this very rare disease.

Highly Symptomatic Progressing Cardiac Paraganglioma With Intracardiac Extension Treated With 177Lu-DOTATATE: A Case Report.

Introduction: Primary cardiac paragangliomas are rare tumors. Metastatic disease is even rarer. Surgical management is technically challenging, and sometimes even impossible. Available therapeutic modalities for metastatic disease include external beam radiation therapy as well as systemic treatments, namely 131I-MIBG and more recently, peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE. To our knowledge, this is the first case of progressive unresectable cardiac paraganglioma with intracardiac extension treated with dosimetry based personalized PRRT to be reported. This case is of particular interest since it documents for the first time the efficacy, and especially the safety of the 177Lu-DOTATATE PRRT in this precarious context for which therapeutic options are limited. Case Presentation: A 47-year-old man with no medical history consulted for rapidly decreasing exercise tolerance. The investigation demonstrated an unresectable progressing metastatic cardiac paraganglioma with intracardiac extension. The patient was treated with personalized 177Lu-DOTATATE PRRT and showed complete symptomatic and partial anatomical responses, with a progression-free survival of 13 months. Conclusions: PRRT with 177Lu-DOTATATE should be considered for inoperable cardiac paraganglioma. No major hemodynamic complications were experienced. Therapy resulted in safety and substantially improved quality of life.

The Use of Peptide Receptor Radionuclide Therapy in Patients With Neuroendocrine Tumor Cardiac Metastases.

Cardiac metastases are an infrequent site of metastasis in neuroendocrine tumors, and the treatment implications in the era of peptide receptor radionuclide therapy (PRRT) are unclear. Potential safety concerns exist regarding cardiac integrity and function in response to PRRT. We describe our institutional experience with 4 patients with well-differentiated, midgut neuroendocrine tumors with cardiac involvement detected on Ga-DOTATATE PET/CT scans who were treated with PRRT.

A retrospective analysis of stereotactic body radiation therapy for canine heart base tumors: 26 cases.

INTRODUCTION: This study retrospectively evaluated outcomes and adverse radiation effects (AREs) associated with stereotactic body radiation therapy (SBRT) for canine heart base tumors (HBTs). A secondary aim was to identify any demographic or echocardiographic factors that might determine which dogs would most benefit from SBRT. ANIMALS: Twenty-six dogs that received SBRT for an imaging-based diagnosis of a HBT were evaluated. METHODS: Twenty-three dogs were treated with three fractions of 10 Gy delivered daily or every other day. The remaining 3 dogs received variable protocols of one to five fractions. Demographic, echocardiographic, and radiographic information, AREs, and treatment responses were collected. Correlations of these data with survival time were evaluated. RESULTS: The median overall survival time was 404 days (95% confidence interval: 239-554 days). The majority of dogs experienced a partial response (25%) or stable disease (60%) for a median duration of 333 days (95% confidence interval: 94-526 days). Three dogs had progressive disease within six months of SBRT. Radiographic pneumonitis was identified in 7 of 23 dogs, and clinical pneumonitis was identified in 4 dogs. No other AREs were noted. The rate of distant metastasis was 13%. On multivariate analysis, it was found that vena caval obstruction, supraventricular and ventricular arrhythmias, clinical signs, and enlarged locoregional lymph nodes at presentation were negatively associated with survival time. CONCLUSIONS: Stereotactic body radiation therapy was delivered with a low rate and degree of normal tissue complications. Asymptomatic dogs with confirmed, progressive growth of a HBT may most likely benefit from SBRT.

Twelve-Year Survival of a Patient With Lymph Node, Pulmonary, Bone, Cardiac and Intraspinal Metastases of a Rectal Neuroendocrine Neoplasm Treated With Peptide Receptor Radionuclide Therapy-The Value of Salvage Peptide Receptor Radionuclide Therapy.

We report here the 12-year survival after the first peptide receptor radionuclide therapy (PRRT) of a patient with metastatic rectal neuroendocrine neoplasms, who received 7 cycles of PRRT with Lu/Y-DOTATATE/DOTATOC in 4 treatment phases. The patient demonstrated excellent response to each cycle of treatment, without any adverse effect even after repeated PRRT cycles. Most recently, immunohistochemistry revealed a G3 neuroendocrine neoplasm and intraspinal metastasis were successfully resected by neurosurgical intervention. This case nicely demonstrates that several "salvage" PRRTs can be given over many years leading to repetitive benefit for the patient and saving patients of possible toxicity of alternative treatments.

Intracardiac chloroma.

Chloromas are not frequently seen in patients with acute myelogenous leukemia and chloromas involving cardiac structures have only been rarely reported in the literature. We report a complete radiographic response to low-dose fractionated radiotherapy in a patient with an intracardiac chloroma.

Bone metastases from primary cardiac sarcoma: case report.

Tumor spread from primary cardiac sarcoma to the bone is very rare and has a poor prognosis. Only six cases have been reported in the literature. We present a 32-year-old female patient with bone metastases from primary cardiac sarcoma.