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PubMed2005

Surgically treated primary cardiac tumors in early infancy and childhood.

OBJECTIVE: Primary heart tumors in childhood are rare and mostly benign. Surgical treatment is advocated when symptoms or hemodynamic impairment is present. MATERIALS AND METHODS: Between 1986 and 2003, 8 children (3 males and 5 females, age ranging 5 days to 6.7 years, median 78 days) with a clinical diagnosis of cardiac mass were treated with surgery. Diagnosis was made by prenatal echocardiography in 3 patients and by 2-dimensional Doppler echocardiography in 5 patients. RESULTS: Complete surgical excision of the cardiac mass was feasible in all but 1 patient who underwent orthotopic heart transplantation. Surgical pathology examination revealed myxoma in 2 patients, fibroma in 2 patients, rhabdomyoma in 2 patients (multiple in 1), hamartoma in 1 patient, and teratoma in 1 patient. One patient died of cerebral malignancy 38 months after cardiac transplantation. At a mean follow-up of 69.2 months (range 3-190 months), all the remaining patients are asymptomatic, with good ventricular function on 2-dimensional echocardiography and no signs of residual or recurrent tumor. CONCLUSION: Surgical excision of obstructive cardiac tumors in childhood is safely feasible. Heart transplantation may represent the only therapeutic option when the tumor extensively invades the ventricular walls. Although 2-dimensional echocardiography remains a reliable diagnostic tool, a definite diagnosis of tumor histotype requires a thorough histopathologic characterization.

Age Factors↗
PubMed2004

Spindle cell rhabdomyosarcoma of the tongue in an infant: a case report with emphasis on differential diagnosis of childhood spindle cell lesions.

Malignant tumors are extremely uncommon in infants, specifically in the head and neck region. We present a three-day-old infant with a large, polypoid, soft tissue mass arising from the floor of the mouth. Histologically, this neoplasm consisted of hypercellular and myxoid areas. A mixture of poorly oriented, small, undifferentiated, hyperchromatic, and round to elongate spindle cells was seen. A high degree of striated muscle differentiation was present, along with areas marked by a herringbone pattern, as well as hemangiopericytic vessels and rare mitosis. Immunohistochemical examinations revealed strong nuclear staining for myogenin and diffuse cytoplasmic staining for desmin and muscle-specific actin (HHF-35). The tumor did not stain for S-100. Based on histologic results and immunostains, this lesion was diagnosed as spindle cell rhabdomyosarcoma. This type of lesion involving the tongue is rarely seen in females, neither in association with a herringbone pattern nor with hemangiopericytic vessels. Furthermore, rare benign and malignant spindle lesions, such as cellular fibromatosis, fetal rhabdomyoma, infantile hemangiopericytoma, infantile rhabdomyofibrosarcoma, and infantile fibrosarcoma, should be in the differential diagnosis and excluded.

Actins↗
PubMed1981

Primary pulmonary rhabdomyosarcoma: a case report and review of the literature.

Although several primary pulmonary neoplasms containing striated muscle fibers have in the past been described as rhabdomyoma or rhabdomyosarcoma, it was not until 1939 that McDonald and Heather described the first acceptable case of this neoplasm. Since then 13 such cases have been reported in the world literature. Previously reported cases of the neoplasm are reviewed and the salient features described. The origin of the striated muscle fibers has been much debated. Some believe that they develop from myoblastic differentiation of primitive mesenchymal cells or metaplastic transformation of pleuripotential mesenchymal cells present in the bronchial walls and pulmonary interstitium, whereas others claim that they develop from misplaced striated muscle tissue from the pharyngeal or esophageal region. A case report of the pleomorphic type of primary pulmonary rhabdomyosarcoma with multiple hematogenous metastases is presented as well as a review of the literature with a discussion of the histogenesis.

Aged↗
PubMed1988

Oncocytic cardiomyopathy syndrome.

The case presented is the first report of oncocytic cardiomyopathy associated with congenital heart disease. The clinical presentation was typical of hypoplastic left heart syndrome, and the oncocytic cardiomyopathy was an unexpected finding at autopsy. The associated lesions of oncocytic metaplasia in thyroid and pituitary gland have been previously reported. Maternal serology and viral cultures from the infant were negative, indicating that the oncocytic lesions were not secondary to congenital viral infection. We support the theory that oncocytic cardiomyopathy is a hamartomatous lesion, and propose as a paradigm the association between cardiac rhabdomyoma and tuberous sclerosis. Careful follow-up of surgically "cured" oncocytic cardiomyopathy should uncover oncocytomas in other organs later in life.

Female↗
PubMed1994

Primary mesenchymal tumors of the urinary bladder. A histological and immunohistochemical study of 30 cases.

The light microscopic and immunohistochemical features of 30 primary mesenchymal neoplasms of the urinary bladder are reported. Half of the cases represented smooth and striated muscle tumors (five leiomyomas, seven leiomyosarcomas including epithelioid and myxoid subtypes, one rhabdomyoma, one embryonal rhabdomyosarcoma and one alveolar rhabdomyosarcoma). One third of the tumors were of fibrohistiocytic origin (one fibrous histiocytoma and eight malignant fibrous histiocytomas including fascicular and storiform, inflammatory and pleomorphic subtypes). In addition, a malignant epithelioid schwannoma, a round cell liposarcoma, two hemangiomas and two mixed mesodermal tumors were observed. The morphology of the vesical mesenchymal tumors was identical to that of their counterparts known to occur in other sites, particularly in the soft tissue. Muscle-specific actin, alpha-1-antichymotrypsin, S-100-protein and neuron-specific enolase proved to be useful and reliable immunomarkers for differential diagnosis of poorly differentiated leio- and rhabdomyosarcomas, malignant fibrous histiocytomas and malignant schwannomas. Since some tumors coexpressed several classes of intermediate filaments, diagnostic immunocytochemistry should only be used considering a larger panel of antibodies and in close correlation with the histological and cytological features of the neoplasms.

Adolescent↗
PubMed1987

Incessant ventricular tachycardia in infants: myocardial hamartomas and surgical cure.

Infants with incessant ventricular tachycardia (occurring greater than 10% of the day) have generally been described in pathologic studies. This report describes 21 patients with incessant ventricular tachycardia present greater than 90% of the day and night; the age at diagnosis ranged from birth to 30 months (mean 10.5 months). The most common clinical presentation was cardiac arrest (11 patients, in 5 after digitalis for presumed supraventricular tachycardia); another 6 patients had congestive heart failure and 4 were asymptomatic. Three patients had coexisting Wolff-Parkinson-White syndrome. The rate of incessant ventricular tachycardia ranged from 167 to 440 (mean 260 beats/min) and the QRS duration from 0.06 to 0.11 second. The most common electrocardiographic (ECG) pattern (10 of 21) was right bundle branch block with left axis deviation, but other right and left bundle branch block patterns were observed. Conventional and investigational antiarrhythmic agents (nine patients received amiodarone) failed to eliminate incessant ventricular tachycardia in all. Electrophysiologic studies localized incessant ventricular tachycardia to the left ventricle in 17 (to the apex in 2, the free wall in 9 and the septum in 6) and to the right ventricular septum in 4. No structural abnormalities were found on the echocardiogram or angiocardiogram. All 21 patients had surgery at an age of 3.5 to 31 months (mean 16). In 15 a tumor was found: 13 myocardial hamartomas (9 discrete, 4 diffuse throughout both ventricles) and 2 rhabdomyomas (1 multiple). Myocarditis was found in one patient (the oldest). In four, only myocardial fibrosis was found; results of one biopsy were normal.(ABSTRACT TRUNCATED AT 250 WORDS)

Anti-Arrhythmia Agents↗
PubMedDate not supplied

Immunocytochemical studies on a fetal brain of tuberous sclerosis.

A female stillborn baby was delivered at 36 weeks' gestation. There were rhabdomyomas in the heart, multi-cystic kidneys, and tubers in the brain. Neuropathologic examination of the brain tissue revealed typical giant cells in the sub-ependymal nodules, cerebral white matter, and cortex. On immunocytochemical staining, the giant cells stained positively with antisera to glial fibrillary acidic protein (GFAP), myelin basic protein (MBP), and neuron-specific enolase (NSE). The giant cells as well as adjacent glial cells with small eccentric nuclei contained both MBP and GFAP. Both cell types combined immunocytochemical characteristics of astrocytes and oligodendrocytes. There were also intermediate-sized cells found among giant cells and germ cells in subependymal nodules. In white matter nodules, similar intermediate-sized cells were found among giant cells and glial cells with small eccentric nuclei. Therefore, giant cells as well as glial cells with small eccentric nuclei may be multi-potential and may result from abnormal patterns of maturation.

Brain↗
PubMed1999

A left atrial myxoma presenting as a cerebrovascular accident.

A 14-year-old female presented with common clinical findings for a rare primary intracardiac tumor. Primary cardiac tumors are rare in all age groups, occurring in 0.05% of routine postmortem examinations. Pediatric primary cardiac tumors are likewise uncommon, with the most common being a rhabdomyoma. Atrial myxomas occur infrequently in the pediatric age group. They occur primarily between the third and sixth decade, making them the most common adult primary cardiac tumor. The following case presentation demonstrates a common clinical presentation for an intracardiac mass rarely diagnosed in the pediatric population. This patient's acute neurologic symptoms required prompt recognition of an intracardiac etiology. This recognition proved critical for the acute and long-term medical and surgical management of this patient.

Adolescent↗
PubMedDate not supplied

Surgical pathology of cardiac tumors. Two decades at an urban institution.

BACKGROUND: As better diagnostic techniques and new operative approaches are developed pathologists will be called upon more often for intraoperative consultation to render a pathologic diagnosis and assess adequacy of resection. METHODS: We conducted a retrospective survey of all patients presenting to our institution from 1979 to 1999. The surgical pathology and cardiothoracic surgery databases were used to identify these patients. RESULTS: Of the 29 patients with primary cardiac neoplasms, 15 were male and 14 female. The mean age at surgery was 51.9 years (range, 7 months to 84 years). Twenty-six of 29 patients had a benign pathological diagnosis. The majority (20/26) of the benign tumors were myxomas. Other benign pathologic diagnoses included rhabdomyoma, fibroma, papillary fibroelastoma, localized fibrous tumor and lipoma. Three out of 29 were malignant: 1 fibrosarcoma, 1 malignant mesenchymoma, and 1 rhabdomyosarcoma. Six of the patients presented with thrombo-embolism, 8 with congestive heart failure symptoms, and three with chest discomfort. Five were asymptomatic or the neoplasm was an incidental finding. 2-D echocardiography established the diagnosis in all of the patients except one. Twenty-two of the 29 tumors were located in the atria (LA=15, RA=6, biatrial=1) and 5 in the ventricles (LV=1, RV=2, biventricular=2). Two patients in this series were referred for reresection. A median sternotomy approach was used for tumor extirpation in all patients. Three of the 29 patients have died at a mean follow-up period of 757 days (median, 118 days). There were two late deaths and one hospital (early) death in a reoperation for recurrent malignancy employing cardiac autotransplantation. One additional patient required cardiac reoperation. CONCLUSIONS: Primary cardiac neoplasms are rare and occur less commonly than metastatic disease of the heart. Congestive heart failure symptoms and thrombo-embolism account for close to half of the presenting signs and symptoms. 2-D echocardiography remains the mainstay of detection. Distinguishing between benign and malignant, thrombus and vegetation, and extracardiac structure is usually established by the size, shape, mobility and attachment of the mass. Clinical presentation and transesophageal echocardiographic views are extremely helpful in sharpening the accuracy of the diagnosis. Since surgery is the only reliable therapy pathologists will be called upon for intraoperative consultation. The majority of the neoplasms are benign. Malignant neoplasms are difficult to excise completely and portend a grave prognosis.

Adolescent↗
PubMed1983

Tuberous sclerosis in an infant of 28 weeks gestational age.

A case of tuberous sclerosis diagnosed at autopsy in a neonate at 28 weeks gestation is described. This is believed to be the earliest case yet reported. The CNS lesions consist of cortical tuberosities, heterotopic white matter nodules and subependymal nodules. These nodules consist predominantly of giant astrocytes but a small undifferentiated cell component is present as well. Multiple cardiac rhabdomyomas are also present. An explanation for neuronal hypocellularity within the cortical tuberosities is suggested.

Brain↗
PubMed1994

Loss of heterozygosity on chromosome 16p13.3 in hamartomas from tuberous sclerosis patients.

Tuberous sclerosis (TSC) is an autosomal dominant condition with characteristic skin lesions, mental handicap, seizures and the development of hamartomas in the brain, heart, kidneys and other organs. Linkage studies have shown locus heterogeneity with a TSC gene mapped to chromosome 9q34 and a second, recently identified on 16p13.3. We have analysed DNA markers in eight hamartomas and one tumour from TSC patients and found allele loss on 16p13.3 in three angiomyolipomas, one cardiac rhabdomyoma, one cortical tuber and one giant cell astrocytoma. We suggest that the TSC gene on 16p13.3 functions like a tumour suppressor gene, in accordance with Knudsen's hypothesis.

Alleles↗
PubMed2001

Outcome of prenatally diagnosed solid fetal tumors.

BACKGROUND/PURPOSE: In the last 10 years, the ability to diagnose fetal tumors in the prenatal period has improved greatly because of technical advances in imaging. Early diagnosis and determination of tumor may affect prognosis. METHODS: The authors retrospectively reviewed the records of 1316 fetuses who underwent sonographic evaluation for congenital defects at University of California-San Francisco over a 6-year period. Of these, 16 had fetal tumors and were followed up at our institution. There were solid or predominantely solid with small cystic component masses in one of 3 locations: cervical, mediastinal, or abdominal. Excluded from our study were those fetuses with either sacrococcygeal teratoma, congenital cystic adenomatoid malformation of the lung, or ovarian cyst, because these defects have been extensively reviewed elsewhere. In addition, masses that were primarily cystic also were excluded. Data collected included diagnosis, gestational age at diagnosis and at delivery, mode of delivery, fetal and neonatal survival, and disease confirmation. RESULTS: Of the 16 fetuses, 4 had mediastinal tumors: 2 with pericardial teratomas (both of whom died in utero) and 2 with cardiac rhabdomyomas (1 died; the other presented tuberous sclerosis and is alive at 2 years of age). Four patients had cervical tumors (3 died; 1 survived and is alive and well), and 8 had abdominal tumors (3 with liver tumors, 4 with a left adrenal mass, and 1 with retroperitoneal teratoma). All eight patients with an abdominal tumor are alive and well. CONCLUSIONS: Fetal tumors are rare, and the prognosis seems to depend on their location and size. Although easier to detect, cervical and mediastinal tumors have a worse prognosis. Abdominal masses are more difficult to detect but have a better prognosis.

Abdominal Neoplasms↗
PubMed1990

Primary cardiac tumors--French studies of 533 cases.

The present study combining the Eisenman French Joint Study and the Broussais Hospital experience is based upon 533 primary cardiac tumors which were operated on in France and by French-speaking teams during a period of about 25 years. The diagnosis of heart tumors was facilitated by the progressive development of new examination techniques. Echocardiography seems to be the simplest, safest, and the most reliable diagnostic method and is used more frequently then angiocardiography. Cardiac tumors occur at every age with a prevalence for females. About 10% of the surgically treated primary cardiac tumors were malignant (53 patients) with a constantly poor prognosis. The mean survival time of this collective after operation was only 1.5 years. 8% of the benign cardiac tumors were non-myxomatous (36), including fibromas, rhabdomyomas, and hamartomas. The mean age of this group was lower (21 years) than in the collectives with myxomas or with malignant cardiac tumors. Another characteristic of non-myxomatous tumors is the involvement of the ventricles by the neoplasm (88%). Complete resection of the tumor was possible in 75%, only 3 operative deaths occurred. Myxomas are the most common tumors of the heart, representing 83% of all primary cardiac tumors (444). Female patients were involved more often than males. The age of the patients ranged widely from 2.5 to 82 years. The typical localization of the myxomas was the left atrium (83%), followed by the right atrium (11%). In 5 cases myxomas were found multilocalized. Very often myxomas were mimicing valvular disease. In more than 30% peripheral embolization provoked the diagnosis. Only in 2% was the tumor totally asymptomatic.(ABSTRACT TRUNCATED AT 250 WORDS)

Female↗
PubMed2002

Spontaneous resolution of obstructive valvular tumors in infants.

BACKGROUND: Myxomas, the most common cardiac tumor in adults, occur in the atria and are prone to embolization. Obstructive valvular tumors in infants occur in pulmonic and aortic valves and generally have a more benign course. METHODS: We conducted a review of our experience in infants with tumors obstructing the right or left ventricular outflow tract, originating from pulmonic or aortic valve tissue. Tissue diagnosis was available from only the index case; 4 additional infants survived with spontaneous regression, and only echocardiographic characterizations were available. RESULTS: Five cases were found with obstructing valvular tumors. In the index case, the obstruction resulted in heart failure, required surgical extirpation, and revealed histology diagnostic of a myxoma. In the subsequent 4 infants with conditions diagnosed with echocardiography, the appearance was suggestive of myxomas (small, compact, ovoid, with no fronds and no embolization). All 4 cases remained mildly to moderately obstructive and then showed spontaneous regression during a period of months to years. CONCLUSION: None of our cases deteriorated clinically, except the first, and were therefore different from other tumors, such as adult atrial myxomas, papillary fibroelastomas, fibromas, and rhabdomyomas. Only the latter are known to spontaneously regress in infancy. The prevalence of valvular tumors in infants may have previously been underestimated, given the absence of symptoms in 4 of our cases and the spontaneous regression of the tumor. An alternate diagnosis for these 4 cases could be a tissue "myxomatous rest" as opposed to a neoplastic myxoma. In either case, surgical intervention may not be necessary.

Aortic Valve↗
PubMed2003

Congenital and hereditary tumours in domestic animals. 2. pigs. A review.

The literature on congenital and hereditary tumours in pigs was reviewed. One hitherto unreported own case was added. Sporadic cases of congenital tumours included several types found in newborn piglets. Embryonic tumours (nephroblastoma, rhabdomyosarcoma) occurred either in newborn or in juvenile animals. Cardiac rhabdomyomas were provisionally classified as hamartomas. The hereditary tumours, melanomas and lymphomas, were reproducible by experimental matings. These tumours are particularly interesting as models to elucidate genetic and immunologic mechanisms of tumour diseases. Striking is the high degree of regression in porcine melanomas and the associated melanosis.

Animals↗
PubMedDate not supplied

Cardiovascular causes for sudden infant death.

Although sudden infant death syndrome (SIDS) is a cause for sudden infant death, other causes should be ruled out before diagnosing SIDS. Cardiac causes for sudden infant death include viral myocarditis, congential heart disease particularly congential aortic stenosis, endocardial fibroelastosis, and anomalous origin of the left coronary artery from the pulmonary artery. Other cardiac conditions that may result in sudden death include rhabdomyomas of the heart in tuberous sclerosis and conduction system disorders. The most frequent conduction system disorders resulting in sudden death include histiocytoid cardiomyopathy, congential heart block that may be associated with maternal lupus erythematosus, arrhythmogenic right ventricular dysplasia, noncompaction of the left ventricle, and long QT syndromes.

Arrhythmias, Cardiac↗
PubMed1991

Comparative immunohistochemical staining for desmin and muscle-specific actin. A study of 576 cases.

Muscle-specific actin (MSA) and desmin are considered to be sensitive and specific markers for muscle differentiation. The authors compared staining patterns for these markers in 576 samples of normal, reactive, and neoplastic tissues. The standard avidin-biotin-peroxidase complex technique was performed with the use of two commercial antibodies against MSA (HHF35; Enzo Biochemical, Inc., New York, NY) and desmin (DER11; DAKO Corporation, Santa Barbara, CA), respectively, on consecutive paraffin-embedded tissue sections from these cases. Both MSA and desmin were found in all 80 normal muscle samples. Although MSA appeared diffusely in all vascular smooth muscle samples, desmin was demonstrated focally in vascular smooth muscle cells in 100 of 196 samples. MSA but not desmin always was found in myoepithelial cells (25 samples), pericytes (286 samples), and decidual cells (7 samples). Among 76 cases of myofibroblast-containing lesions, 14 and 54 were found to have desmin and MSA, respectively. MSA and desmin were found in 4 of 4 cardiac rhabdomyomas, 34 of 34 rhabdomyosarcomas, and 5 of 6 leiomyomas. Among 22 leiomyosarcomas, 7 displayed either MSA or desmin and 7 showed both markers. In general, more tumor cells showed staining for MSA than desmin, but the reverse was true in some cases. Tissue fixed in Zenker's solution seemed to show a significant decrease in MSA immunoreactivity, but no significant change for desmin staining was observed. None of the 154 normal tissues and 22 benign nonmyogenic tumors expressed MSA or desmin. Among 133 malignant nonmyogenic tumors, positive staining for both desmin and MSA was found in 3 of 8 cases of glioblastoma multiforme, 1 of 10 malignant schwannomas, and 1 of 14 malignant fibrous histiocytomas; staining for only MSA was found in 3 of 14 malignant fibrous histiocytomas, 1 of 10 malignant schwannomas, 6 of 6 fibromatoses, 1 of 1 mammary myofibroblastoma, and 1 of 7 malignant mesotheliomas; and staining for desmin only was seen in 1 of 7 malignant mesotheliomas.(ABSTRACT TRUNCATED AT 400 WORDS)

Actins↗
PubMed1999

Sudden death after a cold drink: case report.

We report a case of sudden cardiac death in a 12-year-old boy after rapid ingestion of a frozen slurry drink. The cause of death was determined to be a cardiac arrhythmia secondary to a previously undiagnosed cardiac rhabdomyoma with associated myocardial scarring. Ingestion of cold liquids has been associated with syncope, but not sudden cardiac death. In this case, bradycardia induced by cold-induced vasovagal reflex may have precipitated the terminal arrhythmia. Ingestion of cold liquids should be considered a potential trigger for fatal cardiac arrhythmias in patients with underlying heart disease.

Arrhythmias, Cardiac↗