Assuntos
Neoplasias Cardíacas , Hemangioma , Propranolol , Rabdomioma , Esclerose Tuberosa , Humanos , Propranolol/administração & dosagem , Propranolol/uso terapêutico , Rabdomioma/complicações , Rabdomioma/tratamento farmacológico , Rabdomioma/diagnóstico , Esclerose Tuberosa/complicações , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/tratamento farmacológico , Neoplasias Cardíacas/diagnóstico , Lactente , Hemangioma/tratamento farmacológico , Hemangioma/complicações , Antagonistas Adrenérgicos beta/uso terapêutico , Antagonistas Adrenérgicos beta/administração & dosagem , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/diagnóstico , Feminino , MasculinoRESUMO
Intracardiac lymphomas are exceedingly rare accounting for only 1% of all primary cardiac tumours. Historically, due to their insidious development and non-specific clinical presentation, the diagnosis has been challenging with most cases being confirmed on post-mortem examination. Our case report details the experience of a previously fit and active woman in her 60s who presented with gradual onset exertional dyspnoea. Through a series of multimodal imaging tools (including echocardiogram, cardiac MRI, CT and positron emission tomography-CT) and biopsy, we confirmed the diagnosis of intracardiac diffuse large B-cell lymphoma. Our patient was managed with chemotherapy and went on to demonstrate excellent radiological response with near-complete resolution of the intracardiac mass. Subjectively, our patient reported significant improvement in exercise tolerance within weeks of commencing treatment.
Assuntos
Neoplasias Cardíacas , Linfoma Difuso de Grandes Células B , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/patologia , Feminino , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/tratamento farmacológico , Pessoa de Meia-Idade , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ecocardiografia , Dispneia/etiologia , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Ciclofosfamida/uso terapêutico , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Diagnóstico Diferencial , Doxorrubicina/uso terapêutico , BiópsiaRESUMO
Primary cardiac lymphomas (PCLs) are a rare clinical entity, in which treatment guidelines remain to be established. Rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (R-EPOCH) has been proposed, given that it involves a continuous infusion of anthracycline, reducing the risk of a cardiotoxicity and therefore the theoretical risk of perforation. However, the literature on this method of treatment is scarce. Herein, we present a unique case of a 75-year-old male, diagnosed with primary cardiac diffuse large B-cell lymphoma (DLBCL) with relatively unusual involvement of the coronary sinus, treated first with one cycle of R-EPOCH, followed by three cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) to reduce said risk. To our knowledge, this is one of two cases, in which a patient with PCL was treated this way.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica , Seio Coronário , Ciclofosfamida , Doxorrubicina , Etoposídeo , Neoplasias Cardíacas , Linfoma Difuso de Grandes Células B , Prednisona , Rituximab , Vincristina , Humanos , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Linfoma Difuso de Grandes Células B/patologia , Masculino , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Vincristina/administração & dosagem , Vincristina/uso terapêutico , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Prednisona/uso terapêutico , Prednisona/administração & dosagem , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Doxorrubicina/uso terapêutico , Doxorrubicina/administração & dosagem , Seio Coronário/diagnóstico por imagem , Neoplasias Cardíacas/tratamento farmacológico , Neoplasias Cardíacas/patologia , Etoposídeo/administração & dosagem , Etoposídeo/uso terapêuticoRESUMO
Pericardial synovial sarcomas (PSS) have a low incidence rate and are highly invasive with a dismal prognosis. Standard treatment includes surgery, radiotherapy and chemotherapy but with limited response. Here, we report the case of a 15-year-old nonsmoking youngster diagnosed with PSS who developed disease relapsed from surgery after 1 month. Next-generation sequencing (NGS) using baseline tissue was performed, and BRCA2 c.968dupT was detected. Then pazopanib (a multitargeted inhibitor) plus nivolumab (an immune checkpoint inhibitor) was administered, with a partial response and progression-free survival of 14 months. BRCA2 c.968dupT has not previously been reported in PSS and its response to targeted combination immunotherapy are not well characterized. Here, we report the efficacy of pazopanib combined with nivolumab in a PSS patient harboring BRCA2 c.968dupT and also provide the clinical evidence of the utility of NGS in exploring actionable mutations for solid tumor. Combination therapy based on immunotherapy may be a potential treatment choice for PSS harboring BRCA2 mutation.
Assuntos
Neoplasias Cardíacas , Indazóis , Neoplasias do Mediastino , Neoplasias Pleurais , Sarcoma Sinovial , Neoplasias do Timo , Humanos , Adolescente , Sarcoma Sinovial/tratamento farmacológico , Sarcoma Sinovial/genética , Nivolumabe/farmacologia , Nivolumabe/uso terapêutico , Pirimidinas/uso terapêutico , Sulfonamidas/farmacologia , Sulfonamidas/uso terapêutico , Neoplasias do Mediastino/tratamento farmacológico , Neoplasias do Timo/tratamento farmacológico , Neoplasias Cardíacas/tratamento farmacológico , Neoplasias Pleurais/tratamento farmacológico , Proteína BRCA2/genéticaAssuntos
Insuficiência Cardíaca , Neoplasias Cardíacas , Linfoma Difuso de Grandes Células B , Neoplasias do Timo , Humanos , Insuficiência Cardíaca/etiologia , Neoplasias do Timo/complicações , Linfoma Difuso de Grandes Células B/complicações , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/tratamento farmacológicoRESUMO
INTRODUCTION: Fetal intrapericardial teratoma is a rare tumor that can be diagnosed by antenatal ultrasonography early in pregnancy. CASE PRESENTATION: A fetal intrapericardial teratoma was detected on routine ultrasonography in the second trimester of pregnancy. At 31 weeks gestation, a marked increase in tumor size, fetal ascites, and pericardial effusion were observed, indicating that preterm delivery would be inevitable. Corticosteroid prophylaxis (24 mg of betamethasone in two doses of 12 mg 24 h apart) initiated for prophylaxis of respiratory distress syndrome led to a reduction in fetal ascites and pericardial effusion. Betamethasone therapy (4 mg/per day) was continued with the aim to postpone the expected date of delivery. Gestation was extended for more than 2 weeks. At 33 weeks and 5 days gestation, the neonate was delivered by elective cesarean section with ex utero intrapartum treatment and immediately submitted to fetal cardiac surgery. The infant was discharged from the hospital in good health about 4 months later. CONCLUSION: The present report draws attention to improvement in fetal status and extension of gestation achieved with maternal low-dose corticosteroid therapy on antenatal ultrasound finding of fetal ascites and pericardial effusion due to intrapericardial teratoma.
Assuntos
Neoplasias Cardíacas , Derrame Pericárdico , Teratoma , Recém-Nascido , Gravidez , Humanos , Feminino , Derrame Pericárdico/diagnóstico por imagem , Derrame Pericárdico/terapia , Derrame Pericárdico/etiologia , Cesárea , Ascite , Pericárdio/diagnóstico por imagem , Pericárdio/patologia , Pericárdio/cirurgia , Ultrassonografia Pré-Natal/efeitos adversos , Teratoma/diagnóstico por imagem , Teratoma/tratamento farmacológico , Teratoma/cirurgia , Corticosteroides , Betametasona/uso terapêutico , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/tratamento farmacológico , Neoplasias Cardíacas/cirurgiaAssuntos
Neoplasias Cardíacas , Indazóis , Leiomiossarcoma , Pirimidinas , Sulfonamidas , Neoplasias Uterinas , Feminino , Humanos , Leiomiossarcoma/diagnóstico por imagem , Leiomiossarcoma/tratamento farmacológico , Leiomiossarcoma/patologia , Neoplasias Uterinas/diagnóstico por imagem , Neoplasias Uterinas/tratamento farmacológico , Neoplasias Uterinas/patologia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/tratamento farmacológico , Neoplasias Cardíacas/secundário , TomografiaRESUMO
Tuberous sclerosis complex is associated with the occurrence of cardiac rhabdomyomas that may result in life-threatening arrhythmia unresponsive to standard antiarrhythmic therapy. We report the case of an infant with multiple cardiac rhabdomyomas who developed severe refractory supraventricular tachycardia (SVT) that was successfully treated with everolimus. Pharmacological mTOR inhibition rapidly improved arrhythmia within few weeks after treatment initiation and correlated with a reduction in tumor size. Intermediate attempts to discontinue everolimus resulted in rhabdomyoma size rebound and recurrence of arrhythmic episodes, which resolved on resumption of therapy. While everolimus treatment led to successful control of arrhythmia in the first years of life, episodes of SVT reoccurred at the age of 6 years. Electrophysiologic testing confirmed an accessory pathway that was successfully ablated, resulting in freedom of arrhythmic events. In summary we present an in-depth evaluation of the long-term use of everolimus in a child with TSC-associated SVT, including the correlation between drug use and arrhythmia outcome. This case report provides important information on the safety and efficacy of an mTOR inhibitor for the treatment of a potentially life-threatening cardiac disease manifestation in TSC for which the optimal treatment strategy is still not well established.
Assuntos
Neoplasias Cardíacas , Rabdomioma , Esclerose Tuberosa , Lactente , Criança , Humanos , Everolimo/uso terapêutico , Esclerose Tuberosa/complicações , Esclerose Tuberosa/tratamento farmacológico , Rabdomioma/complicações , Rabdomioma/tratamento farmacológico , Rabdomioma/patologia , Arritmias Cardíacas/complicações , Arritmias Cardíacas/tratamento farmacológico , Serina-Treonina Quinases TOR , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/tratamento farmacológico , Neoplasias Cardíacas/patologiaRESUMO
BACKGROUND: Primary cardiac tumors are extremely rare. Cardiac rhabdomyoma is the most common primary cardiac tumor. 50-80% of solitary rhabdomyomas and all multiple rhabdomyomas are associated with tuberous sclerosis complex. Due to spontaneous regression, surgery is necessary only in severe hemodynamic compromise and persistent arrhythmias. Everolimus, a mechanistic target of rapamycin (mTOR) inhibitor, can be used in the treatment of rhabdomyomas seen in tuberous sclerosis complex. We aimed to evaluate the clinical progression of rhabdomyomas followed-up in our center between the years 2014-2019 and evaluate the efficacy and safety of everolimus treatment on tumor regression. METHODS: Clinical features, prenatal diagnosis, clinical findings, tuberous sclerosis complex presence, treatment and follow-up results were evaluated retrospectively. RESULTS: Among 56 children with primary cardiac tumors, 47 were diagnosed as rhabdomyomas, 28/47 patients (59.6%) had prenatal diagnosis, 85.1% were diagnosed before one year of age and 42/47 patients (89.3%) were asymptomatic. Multiple rhabdomyomas were present in 51% and median diameter of tumors was 16mm (4.5 - 52 mm). In 29/47 patients (61.7%) no medical or surgical treatment were necessary while 34% of these had spontaneous regression. Surgery was necessary in 6/47 patients (12.7%). Everolimus was used in 14/47 patients (29.8%). Indications were seizures (2 patients) and cardiac dysfunction (12 patients). Regression in size of rhabdomyomas was achieved in 10/12 patients (83%). Although, in the long-term, the amount of tumor mass shrinkage was not significantly different between patients who received everolimus and untreated patients (p=0.139), the rate of mass reduction was 12.4 times higher in patients who received everolimus. Leukopenia was not detected in any of the patients, but, hyperlipidemia was noted in 3/14 patients (21.4%). CONCLUSIONS: According to our results, everolimus accelerates tumor mass reduction, but not amount of mass regression in the long term. Everolimus may be considered for treatment of rhabdomyomas which cause hemodynamic compromise or life-threatening arrhythmias before surgical intervention.
Assuntos
Cardiomiopatias , Neoplasias Cardíacas , Rabdomioma , Esclerose Tuberosa , Criança , Gravidez , Feminino , Humanos , Adulto , Everolimo/efeitos adversos , Rabdomioma/tratamento farmacológico , Rabdomioma/complicações , Rabdomioma/diagnóstico , Esclerose Tuberosa/complicações , Esclerose Tuberosa/tratamento farmacológico , Esclerose Tuberosa/diagnóstico , Estudos Retrospectivos , Neoplasias Cardíacas/tratamento farmacológico , Neoplasias Cardíacas/diagnóstico , Progressão da DoençaRESUMO
Everolimus is a mTOR inhibitor that has been increasingly used in high-risk cardiac rhabdomyomas in recent years. There are questions regarding the optimal dose and duration of therapy with everolimus for cardiac rhabdomyomas. The purpose of this study was to examine retrospectively the dosage-efficacy relationship in seven babies diagnosed with rhabdomyoma treated with different everolimus dose regimens retrospectively. Cardiac rhabdomyoma diagnosis was made in six of seven babies during the prenatal period. Indication of everolimus was an obstruction in six patients and supraventricular tachycardia which is resistant to antiarrhythmic drugs in the remaining one patient. The median age was 8 days (range; 2-105 days) at the time of starting everolimus. It was administered at a dose of 0.25 mg twice a day for two days a week in four patients; 0.1 mg/day in two and 0.4 mg/day in one patient. Serum everolimus level was kept between 5 and 15 ng/ml. All seven cases showed significant regression of cardiac rhabdomyoma within four weeks, and supraventricular tachycardia was controlled in two weeks after everolimus administration.This study demonstrates that everolimus was effective in accelerating regression of the cardiac rhabdomyoma. Dose with 2 × 0,25 mg/day, 2 days a week, seems appropriate. However, lower doses such as 0.1 mg/day are also effective. But dose adjustment should be made according to serum level monitoring.
Assuntos
Antineoplásicos , Neoplasias Cardíacas , Rabdomioma , Taquicardia Supraventricular , Lactente , Recém-Nascido , Gravidez , Feminino , Humanos , Everolimo/uso terapêutico , Antineoplásicos/uso terapêutico , Rabdomioma/diagnóstico por imagem , Rabdomioma/tratamento farmacológico , Estudos Retrospectivos , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/tratamento farmacológico , Taquicardia Supraventricular/tratamento farmacológicoRESUMO
An eight-year-old castrated male, 45 kg labrador retriever presented for evaluation of a two-week history of cough and tachypnoea. Echocardiography revealed an approximately 10 cm heart base mass, which extended to the right atrioventricular junction, causing compression of both the right atrium and ventricle resulting in right-sided congestive heart failure (abdominal effusion). Cytology of the mass was consistent with a neuroendocrine carcinoma. Given the location and description, a chemodectoma or ectopic thyroid carcinoma was suspected. The patient was treated with toceranib phosphate and famotidine. At the follow-up appointment four weeks later, the right heart compression had resolved due to a clinically significant reduction in the size of the mass. The patient was prescribed furosemide and enalapril to treat right-sided congestive heart failure. When considering treatment options for haemodynamically significant heart base masses, treatment with toceranib phosphate may result in rapid clinical benefit.
Assuntos
Doenças do Cão , Insuficiência Cardíaca , Neoplasias Cardíacas , Cães , Masculino , Animais , Doenças do Cão/tratamento farmacológico , Doenças do Cão/patologia , Pirróis/uso terapêutico , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/tratamento farmacológico , Neoplasias Cardíacas/veterinária , Insuficiência Cardíaca/veterinária , Bloqueio Cardíaco/veterináriaAssuntos
Cardiomiopatias , Insuficiência Cardíaca , Neoplasias Cardíacas , Rabdomioma , Esclerose Tuberosa , Everolimo/uso terapêutico , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/tratamento farmacológico , Insuficiência Cardíaca/etiologia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/tratamento farmacológico , Humanos , Rabdomioma/complicações , Rabdomioma/diagnóstico , Rabdomioma/tratamento farmacológicoRESUMO
Primary cardiac lymphoma (PCL) is a rare disease, the definite diagnosis of which is sometimes difficult and mainly relies on endomyocardial biopsy. Primary cardiac T-cell lymphoma (PCTL) is an extremely rare sub-type of PCL. Here, we report on a 47-year-old female with PCTL who presented with fever, syncope, palpitations, and a third-degree atrioventricular block (AVB) on electrocardiogram. Chemotherapy was administered with two courses of methotrexate, cyclophosphamide, liposomal doxorubicin, vincristine, and dexamethasone (MTX-CHOP). As the tumor vanished, AVB changed from third degree to second degree and finally to sinus rhythm. In conclusion, endomyocardial biopsy is valuable in the diagnosis of primary cardiac lymphoma. It is worth noting that alterations in the electrocardiogram may indicate an attack on the heart by PCTL.
Assuntos
Neoplasias Cardíacas , Linfoma de Células T , Linfoma , Biópsia , Feminino , Coração , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/tratamento farmacológico , Humanos , Linfoma de Células T/diagnóstico , Linfoma de Células T/tratamento farmacológico , Pessoa de Meia-IdadeRESUMO
Cardiac rhabdomyoma is the most common cardiac tumour in childhood, with a strong genetic association to tuberous sclerosis complex. Although most of the patients remain asymptomatic, a small proportion present with cardiac complications in the early neonatal period. Timely initiation of treatment can potentially reduce disease morbidity, and mammalian target of rapamycin (M-TOR) inhibitors play an effective role in promoting regression of these tumours. A healthy term newborn was diagnosed with a giant congenital cardiac rhabdomyoma at birth. He developed clinical signs of compromised cardiac function and progressive myocardial ischaemia, with echocardiography showing significant dyskinesia. He was treated with M-TOR inhibitors and clinical response was monitored via serial echocardiography. Remarkable regression of the tumour was visibly demonstrated within 4 months of sirolimus treatment. The infant continues to be reviewed by a multidisciplinary team of physicians and monitored for features of tuberous sclerosis complex.
Assuntos
Cardiopatias , Neoplasias Cardíacas , Rabdomioma , Esclerose Tuberosa , Feminino , Cardiopatias/complicações , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/tratamento farmacológico , Humanos , Lactente , Recém-Nascido , Masculino , Rabdomioma/complicações , Rabdomioma/diagnóstico por imagem , Rabdomioma/tratamento farmacológico , Sirolimo/uso terapêutico , Esclerose Tuberosa/complicações , Esclerose Tuberosa/diagnóstico , Esclerose Tuberosa/tratamento farmacológicoRESUMO
BACKGROUND: Primary malignant tumors of the heart are rare. Although preoperative histological diagnosis is difficult, it has paramount value in therapeutic strategy development and prognostic estimation. Herein, we reported 2 cases of intracardiac tumors. CASES PRESENTATION: Both patients presented to the hospital with heart-related symptoms. Echocardiography showed massive masses in the atrium and positron emission tomography-computed tomography (PET/CT) revealed hypermetabolism and invasiveness. One patient cannot take surgery due to extensive metastasis and poor condition. The other patient was primarily diagnosed with lymphoma, and surgery was not recommended. They successfully underwent intravenous atrial biopsy, and histological samples confirmed intimal sarcoma and diffuse large B cell lymphoma. Based on immunohistochemical and molecular assessments, targeted chemotherapy was administered, resulting in clinical and imaging remission at discharge. CONCLUSIONS: Percutaneous intravenous catheter biopsy as a safe invasive test provides an accurate pathological diagnosis after imaging evaluation, and offers a therapeutic direction. Nonmalignant masses and some chemo-radiosensitive malignant tumors in the atrium could have good prognosis after targeted therapy.
Assuntos
Cateterismo Periférico/instrumentação , Átrios do Coração/patologia , Neoplasias Cardíacas/patologia , Linfoma Difuso de Grandes Células B/patologia , Sarcoma/patologia , Instrumentos Cirúrgicos , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , Feminino , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/efeitos dos fármacos , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/tratamento farmacológico , Humanos , Linfoma Difuso de Grandes Células B/diagnóstico por imagem , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Terapia de Alvo Molecular , Estadiamento de Neoplasias , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Sarcoma/diagnóstico por imagem , Sarcoma/tratamento farmacológico , Resultado do TratamentoRESUMO
BACKGROUND: Sirolimus constitutes a safe and effective treatment for cardiac manifestations of tuberous sclerosis complex (TSC) in children but only four cases describing prenatal treatment of rhabdomyomas with mTOR inhibitors have been published. CASE: In this case, sirolimus was initiated at 26 weeks´ gestation in a pregnant woman with TSC with a fetus with a large rabdomyoma conditioning severe arrythmia. There was a significant reduction in the tumor size with ongoing treatment and a partial reversion of the arrythmia. CONCLUSION: m-TOR inhibitors can be considered for severe cases of fetal rhabdomyomas with poor prognosis given its potencial benefits.
Assuntos
Neoplasias Cardíacas , Rabdomioma , Esclerose Tuberosa , Criança , Feminino , Humanos , Gravidez , Arritmias Cardíacas , Feto/patologia , Neoplasias Cardíacas/tratamento farmacológico , Neoplasias Cardíacas/patologia , Rabdomioma/tratamento farmacológico , Rabdomioma/patologia , Sirolimo/uso terapêutico , Esclerose Tuberosa/complicações , Esclerose Tuberosa/tratamento farmacológicoRESUMO
Cardiac tumors are rare and benign masses account for the most part of the diagnosis. When malignant cancer is detected, primary or secondary cardiac lymphoma are quite frequent. Cardiac lymphoma may present as an intra or peri-cardiac mass or, rarely, it may diffusely infiltrate the myocardium. Although often asymptomatic, patients can have non-specific symptoms. Acute presentations with cardiogenic shock, unstable angina, or acute myocardial infarction are also described. Modern imaging techniques can help the clinicians not only in the diagnostic phase but also during administration of chemotherapy. A multidisciplinary counseling and serial multi-parametric assessment (echocardiography, cardiac troponin) seem to be the most effective approach to prevent possible fatal complications (i.e., cardiac rupture). Currently, only chemo- and radiotherapy are available options for treatment, but the prognosis remains poor. This is a case of secondary cardiac lymphoma presenting as a mediastinal mass with large infiltration of the heart and the great vessels with a good improvement after only one cycle of chemotherapy. It demonstrates the importance of an early diagnosis to modify the natural history of the disease.
Assuntos
Neoplasias Cardíacas , Linfoma , Infarto do Miocárdio , Humanos , Miocárdio/patologia , Linfoma/diagnóstico por imagem , Linfoma/tratamento farmacológico , Prognóstico , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/tratamento farmacológico , Neoplasias Cardíacas/patologiaRESUMO
Myocardial metastasis of nasopharyngeal carcinoma (NPC) is rarely reported in the literature. Some autopsy studies found metastases in more than 10% of cases with malignant neoplasm. However, patients are often diagnosed during the postmortem because myocardial metastasis is often asymptomatic, and its Cardiac complications tend to be severe and fatal. Patients with Cardiac metastases are often treated with chemotherapy or surgical intervention, although the prognosis is poor. Immunotherapy with anti-programmed cell death receptor-1 or ligand-1 (PD-1 or PD-L1) inhibitors has recently been reported to be therapeutically significant in multiple cancers, including melanoma, nonsmall cell lung cancer, and NPC, but the treatment of myocardial metastasis of NPC has not been reported. This study described the case of a 50-year-old male patient who presented initially with NPC and received radiotherapy as first-line therapy. For 20 years, he had recurrent Cardiac metastasis of NPC. The pathological examination suggested tPD-L1 expression. Therefore, off-label sintilimab (200 mg every 21 days) was administered. After 10 cycles of treatment, myocardial metastasis shrank and the enlarged mediastinal lymph nodes disappeared. This case report demonstrated that Cardiac metastasis of NPC expressing PD-L1 might have a sustained response to PD-L1 inhibitor-directed therapy.