Surgical treatment of primary cardiac tumors in children.

OBJECTIVE: Summarizing the treatment experience of primary cardiac tumors in children. METHODS: The date of 24 children with primary cardiac tumors who underwent surgery in our department from July 2003 to September 2022 was collected and analyzed treatment efficacy. RESULTS: All patients completed the surgery successfully, including 21 cases of complete tumor resection, 2 cases of partial tumor resection, and 1 case of tumor biopsy. The location: 5 cases in the right atrium, 5 cases in the right ventricle, 6 cases in the left atrium, 6 cases in the left ventricle, 1 case in the left, right ventricle and ventricular septum, and 1 case in the ventricular septum. 23 cases were benign: 11 cases of myxoma, 7 cases of fibroma, 3 cases of rhabdomyoma, 1 case of infantile capillary hemangioma, and 1 case of lipoma. There was 1 case of borderline or malignant tumor. 23 patients were discharged successfully, 1 patient died of cardiac failure on the first day after operation. Follow-up was done from 5 months to 19 years and 2 months, 2 cases were lost to follow-up, and 1 case died of cardiac failure in the second year after operation due to severe mitral regurgitation. There was 1 case of tumor biopsy with space-occupying lesion gradually shrinking during follow-up. The prognosis of another 19 children with complete or partial tumor resection was good. There was no recurrence, enlargement, or reoperation of the tumor during the follow-up period. CONCLUSIONS: Primary cardiac tumors in children are mostly benign. Surgery is effective, but the timing of surgery depends on the patient's condition.

Surgical Treatment of Pediatric Refractory Ventricular Tachycardia Originating From a Left Ventricular Rhabdomyoma.

Cardiac tumors are very rare in children, and echocardiography is very important in their detection. The clinical presentation can vary greatly depending on arrhythmia or obstruction. One of the most important factors determining the surgical approach is the clinical process. In this case report, we report the surgical treatment of a rhabdomyoma that caused refractory ventricular tachycardia.

Extracardiac adult-type rhabdomyoma-Report of two cases in dogs.

This report describes the cytologic, histopathologic, and immunohistochemical features of adult-type rhabdomyoma located within the subcutaneous tissue in a 14-year-old female Border Collie (thigh) and a 13-year-old male Mongrel (flank). In both cases, fine-needle aspiration biopsy revealed cluster-forming, epithelial-like polygonal cells with abundant foamy cytoplasm, and moderate to marked anisocytosis and anisokaryosis; therefore, an epithelial tumor was suspected. After surgical excision, tumors underwent histopathologic examination with additional immunohistochemistry. Both tumors were well-demarcated and located within the subcutaneous tissue in the vicinity of the cutaneous muscle. The tumor mass consisted of densely packed round or polygonal cells with distinct vacuolation of the cytoplasm. Tumor cells expressed vimentin, desmin, and NSE and were cytokeratin and α-SMA negative. Based on histologic features and immunophenotyping, adult-type rhabdomyoma was diagnosed in both cases. This study highlights that the cytologic features of rhabdomyoma can be misleading and may suggest an epithelial tumor.

Cutaneous rhabdomyoma in an 82-year-old White man.

This case highlights a primary cutaneous rhabdomyoma presenting as a slowly enlarging subcutaneous nodule on the mentum of an 82-year-old White man with a medical history of two intracranial rhabdomyomas. Although they are rarely syndromic, it is important to note that the most common demographic for presentation of rhabdomyomas includes older males presenting as a subcutaneous nodule on the head, neck, or oral cavity. They are most often seen in isolation but can be multifocal in up to 25% of all cases. Being a rare entity, there is no generally recognized treatment consensus; however, complete surgical excision is recommended to prevent recurrence and morbidity from local tissue destruction.

Management of neonatal cardiac rhabdomyoma obstructing the aortic valve.

Rhabdomyomas associated with tuberous sclerosis are common cardiac tumors in children, and no surgical intervention is needed in most cases. However, when the tumor causes left ventricular outlet tract obstruction (LVOTO), immediate surgical intervention is indicated. Here, we report a newborn who was diagnosed antenatally with multiple cardiac rhabdomyomas, one of which blocked the aortic valve during systole. The tumor was resected early in the postnatal period with excellent outcomes. Until surgery, we maintained ductus arteriosus patency and systemic circulation using prostaglandin E1, which helped to reduce the risk of sudden death due to LVOTO. Postoperative two-dimensional echocardiography at discharge showed that surgery was effective in resolving LVOTO.

Congenital Solitary Left Atrial Mass.

Rhabdomyoma in the absence of tuberous sclerosis presenting as a large solitary atrial mass is an atypical finding. We hereby present images from an infant with a fetally diagnosed large left atrial mass with no evidence of mitral valve dysfunction who subsequently developed persistent arrhythmias requiring surgical resection of the mass in the neonatal period.

Oral Adult Rhabdomyoma.

This report describes a case of an adult rhabdomyoma (ARM) occurring in the oral cavity. A 47-year-old man was referred for the diagnosis of a painless, well-circumscribed, submucous nodule located on the floor of the mouth, measuring approximately 6.0 cm in length. Computed tomography revealed a well-defined, solid, and hypodense mass. A benign salivary gland or mesenchymal tumor were the main diagnostic hypotheses. Under local anesthesia, the patient underwent surgical excision. Microscopically, the tumor comprised large polygonal well-defined cells with abundant, eosinophilic granular cytoplasm with cross striations. No atypia or mitosis was observed. The cells were positive for muscle-specific actin, desmin, and sarcomeric alpha-actin. Based on these features, a diagnosis of ARM was established. No recurrence was observed after 48 months. Although rare, ARM should be considered in the differential diagnosis of oral submucosal nodules, especially those located on the floor of the mouth.

Recurrent primary endobronchial fetal rhabdomyoma: a case report and literature review.

Fetal rhabdomyoma is an extremely rare benign rhabdomyoblastic tumor with myotube-like differentiation, mainly arising on mucosal surfaces of the head and neck region of both children and young patients, almost invariably definitively treated with surgical excision. Herein the case of a male adult suffering from a recurrent fetal rhabdomyoma primary involving the bronchial structures is reported, along with a detailed literature review. This is the first fetal rhabdomyoma described to originate in such a localization; furthermore, an 11-year interval period between the first lesion and the recurrent one has never been reported.

Case Report and Review of the Literature of a Rare Entity of a Uterine Fibroid: A Genital Rhabdomyoma.

Extracardiac rhabdomyomas are rare benign tumors. According to histopathologic and clinical characteristics, they are divided into 3 subgroups: adult, fetal, and genital rhabdomyomas. Various adult extracardiac rhabdomyomas have been reported in the head and neck region, whereas genital rhabdomyomas are uncommon. Here, we report on a uterine genital rhabdomyoma in a 32-yr-old woman with secondary sterility. After myomectomy, the histopathologic analysis showed a slow cycling tumor with striated muscle differentiation and without any evidence of malignancy. Immunohistochemical staining proved coexpression of actin, caldesmon, and desmin. To the best of our knowledge, this is the first case of a uterine-based genital rhabdomyoma.

Simultaneous Malignant and Benign Nonepithelial Neoplasms of the Larynx-A Case Report and Literature Review.

Nonepithelial tumors of the larynx are relatively rare neoplasms of the head and neck. The chondrosarcoma, which develops commonly from cricoid and thyroid cartilage, stands for 0.2% of the laryngeal malignant neoplasms. The rhabdomyoma is even more uncommon benign tumor developing from the laryngeal striated muscles. The clinical manifestation and the treatment options depend on the histopathological evaluation, tumor localization, and its size. In presented case, the simultaneous occurrence of benign and malignant tumors of mesenchymal origin in the patient's larynx was provoking hoarseness, globus sensation, and dysphagia. To the best of authors' knowledge, no other case of the concomitance of rhabdomyoma and chondrosarcoma of the larynx have been reported in the literature.

Histiocyte-rich rhabdomyoblastic tumor: a report of two cases and a review of the differential diagnoses.

Histiocyte-rich rhabdomyoblastic tumor is a recently described skeletal muscle neoplasm of uncertain malignant potential, characterized by slow growth, a fibrous capsule containing peripheral lymphoid aggregates, spindle-to-epithelioid cells with a rhabdomyoblastic immunophenotype, and a dense histiocytic infiltrate. It most commonly arises within the muscles of the lower legs and trunk in young-to-middle-aged men, and initial reports suggest indolent behavior. In this paper, we present two additional cases of histiocyte-rich rhabdomyoblastic tumor with similar clinicopathologic features and discuss the differential diagnosis including its overlap with inflammatory leiomyosarcoma.

Multifocal adult rhabdomyoma of the head and neck: case report and systematic review of the literature.

Adult-type rhabdomyoma (ARM) is the most frequent extracardiac subtype of rhabdomyoma. In very rare instances ARM presents as multiple cervical lesions, an entity known as multifocal ARM. The aim of this study was to review the clinical characteristics and the best therapeutic options for multifocal ARM. The case of a 73-year-old patient recently diagnosed with multifocal ARM and managed in our institution is reported, followed by a systematic review of the literature. The review was conducted using the PubMed, Scopus, and Google Scholar databases, according to the PRISMA guidelines. All case reports and case series related to multifocal ARM written in English or French were included. A total of 29 cases were included in the qualitative analysis. Mean age at presentation was 65.0 years and the male to female ratio was 13.5:1. Dysphagia was the most common presenting symptom. ARM was most frequently found in the submandibular spaces. In conclusion, multifocal ARM are benign tumours with a predilection for the head and neck region. They become symptomatic with gradual compression of the adjacent structures. No cases of malignant transformation were reported in the literature. We suggest a 'watchful wait' approach for small asymptomatic tumours and excision of large symptomatic ones.

A rhabdomyoma in the right ventricle presenting as hemodynamics of hypoplastic right heart.

Rhabdomyomas are the most common paediatric cardiac tumours. The natural history of these tumours is mostly benign, and the tumour usually regresses spontaneously. Although surgical resection of these tumours is one of the considerations in patients with ventricular outflow obstruction, a palliation with Blalock-Taussig shunt is an alternative approach with the hope of regression of the tumour over time. We report a case of prenatally diagnosed rhabdomyomas in the right ventricle and its outflow presenting as hemodynamic simulating hypoplastic right ventricle in a newborn. She required prostaglandin and Blalock-Taussig shunts palliation for pulmonary flow and subsequent regression of tumours.

A case of surgically resected cardiac rhabdomyoma with progressive left ventricular outflow tract obstruction.

Cardiac rhabdomyoma is the most prevalent cardiac tumors in the pediatric population, in close association with tuberous sclerosis complex. It is usually detected antenatally or postnatally by echocardiography. Clinical presentations depend greatly on the size and position of the tumor mass. Interestingly, rhabdomyoma has a propensity to regress spontaneously and is not usually operated upon, unless the patient becomes hemodynamically compromised. Herein, we report an unusual case of surgically treated cardiac rhabdomyoma in a baby boy presented at birth with a progressive enlarging intraventricular mass, complicated with left ventricular outflow tract obstruction 7 weeks later. Histopathological examination of the intracardiac mass revealed sheets of tumor cells with spider-like morphology (known as "spider cells"), confirmed the diagnosis of rhabdomyoma. Close disease monitoring of patient's hemodynamic status in a newly diagnosed cardiac rhabdomyoma is inevitable as the tumor, although rare, may progress.

Oropharyngeal rhabdomyoma: the cause of severe sleep apnoea?

This case report presents an elderly male patient who on diagnosis with a large oropharyngeal tumour had no specific symptomatology apart from severe obstructive sleep apnoea. Histopathology revealed the tumour to be an adult rhabdomyoma, a rare but benign tumour arising from striated muscle cells. The tumour obstructed most of the oropharyngeal space and almost occluded the patient's airway when lying in a supine position. The patient was deemed operable, and the tumour was excised in toto using a transoral robotic surgery system. On follow-up, the patient had a severe reduction of apnoeas/hypopnoeas and felt subjectively 'reborn'. This is to our knowledge the first case where an adult rhabdomyoma is removed using a robot-assisted approach, thus presenting a new and viable option when considering removal of benign tumours of the pharynx leading to a very minor degree of morbidity for the patients.

Clinical Treatment of Pediatric Primary Cardiac Tumors: A Single-Institute 12-Year Experience.

BACKGROUND: Primary cardiac tumors in children are extremely rare. This study aimed to analyze clinical treatment and follow-up of pediatric primary cardiac tumors. PATIENTS AND METHODS: We performed a retrospective analysis by searching the medical records of 75 patients diagnosed with pediatric primary cardiac tumors from June 2005 to August 2017 in our institution. We followed operative patients every half year in the first postoperative year and then at least every year. If the patients had no serious symptoms or hemodynamic changes, they received nonoperative management and were followed regularly every year. RESULTS: Nineteen patients underwent surgery at our department for serious symptoms and critical hemodynamic changes. Four patients had postoperative complications. Two died of low cardiac output syndrome and arrhythmia after surgery. One patient with myxomas had tumor recurrence and one had been found of another rhabdomyoma after surgery. The other 14 patients recovered well. Fifty-six patients had nonoperative management. Four were lost in follow-up. Two patients with malignant tumors died of unknown causes after discharge. The remaining patients had no severe symptoms or tumor growth during follow-up. CONCLUSIONS: Clinical treatment of pediatric primary cardiac tumors should be performed individually. Most pediatric primary cardiac tumors are benign, and spontaneous regression is possible, especially for rhabdomyomas. The principle purpose of surgical treatment is to restore normal hemodynamics and protect important structures and cardiac tissue.

A Mass Causing Right Ventricular Outflow Obstruction - A Dreadful Complication.

The most common cardiac tumour in the pediatric age group is rhabdomyoma. These are usually located in the ventricles, either in the ventricular septum or free wall. Cardiac tumours in early infancy may lead to severely compromised blood flow due to inflow or outflow tract obstruction. The diagnosis of cardiac rhabdomyoma can be established by transthoracic echocardiography (TTE). Rhabdomyomas have a natural history of spontaneous regression; surgical intervention is reserved for patients with symptoms of severe obstruction or hemodynamic instability. In this study, a case of two-year old child who presented with failure to thrive and underwent excision of pedunculated mass from the right ventricular outflow tract was reported.

A mass causing right ventricular outflow obstruction - a dreadful complication

Abstract The most common cardiac tumour in the pediatric age group is rhabdomyoma. These are usually located in the ventricles, either in the ventricular septum or free wall. Cardiac tumours in early infancy may lead to severely compromised blood flow due to inflow or outflow tract obstruction. The diagnosis of cardiac rhabdomyoma can be established by transthoracic echocardiography (TTE). Rhabdomyomas have a natural history of spontaneous regression; surgical intervention is reserved for patients with symptoms of severe obstruction or hemodynamic instability. In this study, a case of two-year old child who presented with failure to thrive and underwent excision of pedunculated mass from the right ventricular outflow tract was reported.