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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary pulmonary tumors are infrequent in childhood, therefore an accurate diagnosis and treatment is often delayed. We review the English language literature and report the clinical and pathological features of eight tumors arising in the lungs of pre-adolescent children, accessioned between 1960 and 1991 in the pathology department of a children's hospital in South Africa. The ratio of pulmonary primary tumors to secondary neoplasms and to non-neoplastic lesions of the lung examined during this period was 1:5:60. Over the last 31 years we received three plasma cell granulomas, two pleuro-pulmonary blastomas, a mucoepidermoid carcinoma, an endobronchial fibrosarcoma, and a hemangioma. All patients presented with cough unresponsive to medical treatment. The incidence and spread of primary lung tumors in children was similar to that reported from other centers. Plasma cell granuloma is the most common primary tumor in the lungs of children. Aggressive behavior is most frequently encountered with pleuro-pulmonary blastoma and rhabdomyosarcoma, and because of their association with cystic lesions careful examination of lungs is required in such cases. Most other malignant neoplasms, such as muco-epidermoid carcinoma and primary fibrosarcoma, are usually of a low grade of malignancy. A decreasing incidence of bronchogenic carcinoma seems to be reported during the first two decades of life.
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PMID:Primary pulmonary tumors in childhood: a review of 31 years' experience and the literature. 133 97

The consultants agreed that the differential diagnosis should focus on congenital masses, including an encephalocele, glioma, dermoid, hamartoma, hemangioma, rhabdomyosarcoma, neurofibroma, and nasolacrimal duct cyst. There was some disagreement as to which is the best way to evaluate the mass, ranging from an MRI (Dr. Reilly), to CT scan (Dr. Cotton), to both MRI and CT (Dr. Koopman). Blood tests to evaluate pituitary function could be indicated if there was a sphenoid defect (Dr. Reilly). None of the experts would biopsy this lesion. All would proceed with a definitive resection. One surgeon would defer surgery for several months and then perform the resection via a biocoronal craniotomy (Dr. Reilly). A combined anterior craniotomy and external ethmoidectomy would be planned by another (Dr. Koopman). The third consultant would combine an anterior craniotomy with a mid-face degloving, external rhinoplasty, or lateral rhinotomy approach (Dr. Cotton). Routine perioperative antibiotics would only be used by two of the surgeons (Drs. Reilly and Koopman). If a CSF leak were encountered there are several options. A small lesion could be allowed to close on its own (Dr. Reilly). If the leak occurred while the bicoronal incision was still open or if the leak were large, it could be repaired from above (Drs. Reilly and Koopman). One surgeon would proceed with a repair from above even if the leak were encountered during the intranasal approach (Dr. Cotton). Only one surgeon would restrict postoperative activity with intubation and sedation or paralysis (Dr. Koopman). Regarding follow-up, no one was concerned about the final pathology report.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Nasal mass in a pediatric patient. 139 78

Twenty-six infants and children with orbital and ocular pathology were examined with ultrasound (US) utilizing real-time imaging and Duplex Pulsed Doppler evaluation. Twenty-two of these patients underwent concurrent orbital computed tomography (CT) and two had magnetic resonance imaging (MRI). Orbital and periorbital lesions included hemangioma, dermoid, lymphangioma, rhabdomyosarcoma, encephalocoele and abscess. Ocular lesions included infection, trauma, retinal detachment, retinoblastoma, Coat's disease, and persistent hyperplastic primary vitreous. High resolution US with Doppler provided unique diagnostic information in patients with penetrating ocular trauma, orbital and periorbital masses, and intraocular structural abnormalities. High resolution US examination of the eye and periorbital tissues is readily performed using widely available equipment and often delineates subtle structural abnormalities not shown by CT or MRI.
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PMID:High resolution ultrasound with Doppler: a diagnostic adjunct in orbital and ocular lesions in children. 150 82

Orbital abnormalities encountered in the pediatric population differ substantially from those found in adult patients. Retinoblastoma, the most serious intraocular tumor, is often difficult to diagnose, but use of computed tomography (CT) (which reveals the characteristic focal calcification) and magnetic resonance (MR) imaging allows this tumor to be differentiated from pseudogliomas, such as Coats disease, and retrolental fibroplasia. CT and MR imaging help in the differentiation of orbital cellulitis from preseptal, lacrimal, and eyelid infectious processes and of orbital pseudotumor from Graves disease. In cases of orbital trauma, CT is excellent for detecting orbital fractures and metallic foreign bodies; MR imaging is better for depicting ocular and optic nerve injuries. Both modalities allow the differentiation of rhabdomyosarcoma from dermoid, cavernous hemangioma, and lymphangioma and provide helpful information for the diagnosis of many other tumors. Since CT and MR imaging have widely expanded the capabilities of orbital imaging, it is more important than ever before for radiologists to understand pediatric orbital disease.
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PMID:CT and MR imaging of the pediatric orbit. 160 40

The authors analyzed 1422 orbital tumors examined 1955-1986 in the eye pathology laboratory. The 5 leading malignant orbital tumors were adenocarcinoma (207 cases), squamous cell carcinoma (135 cases), rhabdomyosarcoma (52 cases), lymphosarcoma (39 cases), and malignant mixed tumor (29 cases). The 5 leading benign orbital tumors were cavernous hemangioma (304 cases), benign mixed tumor (109 cases), inflammatory pseudo-tumor of the orbit (101 cases), dermoid cyst (100 cases), and optic meningioma (65 cases). Rare tumors of the orbit included 1 case each of alveolar soft tissue sarcoma, chondrosarcoma, mesenchymal chondrosarcoma, synovial sarcoma, giant cell tumor, granular myoblastoma, metastatic leiomyosarcoma of the uterus and metastatic lymphoepithelioma. The criteria for pathological classification and differential diagnosis are discussed.
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PMID:[Histopathologic classification of 1422 orbital tumors]. 186 Apr 6

The most common ocular and orbital tumors presenting in infancy, childhood and adolescence are presented and discussed in this review. It has been prepared specifically for the clinical pediatrician and focuses on the clinical recognition of ophthalmic neoplasms, their diagnostic evaluation employing the use of advanced imaging techniques, biopsy when indicated and extent of disease workup. In addition, current treatment modalities are discussed. Ocular tumors addressed include: retinoblastoma, capillary hemangioma, lymphangioma, dermoid and epidermoid cysts, teratoma, glioma, astrocytic hamartoma, neurofibroma, rhabdomyosarcoma and fibrous tumors. Two aggressive and potentially fatal tumors, rhabdomyosarcoma and retinoblastoma, are presented in detail. In addition, the ocular tumors associated with the phakomatoses (von Hippel-Lindau, tuberous sclerosis and neurofibromatosis) are reviewed.
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PMID:Ophthalmic neoplasms in infancy and childhood. 219 81

Thirty-four cases of soft tissue tumors (STT) other than rhabdomyosarcoma in infants under 1 year of age were seen in our institution between 1955 and 1985. All were diagnosed initially as malignant tumors except for three cases of fibromatosis, and, thus, they received therapy appropriate at that time. During a recent pathologic review, four were seen to have had hemangioma, six hemangiopericytoma, one hamartoma, seven fibromatosis, eight fibrosarcoma, and eight unclassified sarcomas. Of these 34 cases, the initial histological diagnosis was confirmed in only 17. Sixteen patients were believed to have received inappropriately aggressive therapy; indeed, four of these cases diagnosed prior to 1975 had been benign. Major long-term side effects were seen in 7/16 treated patients (six radiotherapy, one surgery), who, in retrospect, should have received less aggressive treatment. The reasons for these initial errors in diagnosis included doubtful histologic features associated in some cases with alarming clinical presentation. We believe that difficulties still remain in distinguishing benign from malignant STT in this age group by histological examination. Therefore, we urge extreme caution in using aggressive therapy before the diagnosis is certain.
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PMID:Diagnostic and therapeutic problems of soft tissue tumors other than rhabdomyosarcoma in infants under 1 year of age: a clinicopathological study of 34 cases treated at the Institut Gustave-Roussy. 229 90

65 cases of cardiac tumors were diagnosed and studied by echocardiography. Among them 49 were primary tumors including 43 cases of myxoma, one case each of hamartoma, lipomatous infiltration, fibroma, hemangioma, rhabdomyosarcoma, pericardial mesothelioma, and 16 cases of secondary cardiac tumors. It was found that the nature of primary cardiac tumors could be speculated by two-dimensional echocardiography based on their pathological features. Most patients with large left atrial myxoma had obstructive symptoms of mitral valves, abnormal ECG and enlarged left atrium, whereas patients with small atrial myxoma, embolic phenomenon was liable to occur. Echocardiography of secondary cardiac tumors showed that the tumors usually invaded most frequently both the myocardium and pericardium as single or multiple nodular echoes in the myocardium under pericardium or within the pericardial cavity, with profuse pericardial effusion. Occasionally, the secondary tumor appeared as a large mobile intracavitary mass or an extracardiac one compressing the heart or large vessels. It was noticed that cardiac symptoms might be the clinical clue in certain patients with extracardiac primary malignancy.
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PMID:[Cardiac tumors: clinical and echocardiographic diagnosis of 65 cases]. 239 90

Heterotopic brain is a rare entity. Histologically, this lesion resembles mature brain and often contains specialized tissues similar to choroid plexus or glia. Specialized neural stains are necessary to differentiate this rare anomaly from other tumors or conditions found in the head and neck. The differential diagnosis includes squamous cell carcinoma, granular cell tumor, hemangioma, lymphangioma, thyroglossal duct cyst, dermoid cyst, hamartoma, rhabdomyosarcoma, and teratoma. We describe a newborn with heterotopic brain tissue occurring on the dorsum of the tongue. We found only one other description of this developmental aberration in the English literature. Our patient was successfully treated with carbon dioxide laser excision of the mass. There has been no evidence of complication or recurrence after one year of follow-up.
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PMID:Heterotopic lingual brain in the newborn. 270 10

This large series of cases demonstrates that the breast of the child or adolescent is the potential site for a number of interesting lesions exclusive of fibroadenoma and gynecomastia. Adenoma of the nipple, juvenile papillomatosis, and juvenile or cellular fibroadenoma should be correctly diagnosed to insure conservative surgical management and appropriate clinical follow-up. The results of the Juvenile Papillomatosis Registry suggest that this lesion is a morphological marker of potentially more serious breast disease in the future. Recognition of a cellular fibroepithelial neoplasm, especially in young black females, as a cellular fibroadenoma will hopefully eliminate the need for the diagnosis of "benign" cytosarcoma phyllodes. On occasion, the equivocal case may be labeled a "phyllodes tumor fo indeterminate biologic behavior." Those neoplasms with stromal atypia and mitotic activity, overgrowth of stroma with epithelial dissociation, and sarcomatous elements are appropriately designated as cytosarcoma phyllodes. In contrast to vascular tumors of the breast in adults, the majority of vascular lesions in the child's breast are bening and represent either a capillary or a histiocytoid hemangioma. Our experience and reports in the literature support the conclusion that most malignancies of the breast in children are metastatic. Rhabdomyosarcoma was the most common example in our study, but secondary involvement by acute leukemia or malignant lymphoma also occurs.
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PMID:Lesions of the breast in children exclusive of typical fibroadenoma and gynecomastia. A clinicopathologic study of 113 cases. 277 54


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