Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Kaposiform hemangioendothelioma (KH) is an endothelial-derived spindle cell neoplasm often associated with Kasabach-Merritt syndrome. Most cases arise in infancy and childhood and are soft-tissue tumors. The tumor displays an appearance between capillary hemangioma and Kaposi's sarcoma. We report a case of KH in a 1-year-old girl involving a mass that showed abnormal enhancement of soft tissue superficial to the right temporal bone with partial destruction of the temporal bone, the temporomandibular joint, mandibular condyle, and occipital bone. The physical finding of a discolored mass led clinicians to consider a hemangiomatous lesion, whereas the radiological picture suggested a more aggressive diagnosis of rhabdomyosarcoma and aggressive fibromatosis.
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PMID:A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging. 1172 24

Four patients with relatively large orbital tumors are described. These patients had superomedial rhabdomyosarcoma, inferomedial cavernous hemangioma, lateral primitive neuroectodermal tumor, and superior neurofibroma, respectively. Continuing enlargement and encroachment upon vital ocular structures and suspicion of malignancy warranted surgical intervention. Following exposure of the anterior portions of these tumors, a cryoprobe was used for the complete removal of the mass. Cryoextraction greatly facilitates the excision of well-defined, solid, encapsulated, benign or malignant tumors excluding the lacrimal gland and thus minimizes trauma to adjacent tissues. More importantly, the tumor can be removed intact without capsular rupture and risk of tumor spill-over or hemorrhage. At the histopathological level, the application of a cryoprobe did not alter the microscopic structures of the lesions and did not cause any difficulty for histopathologic interpretation.
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PMID:Cryoextraction in the management of orbital tumors. An old technique revisited. 1204 27

Chest wall lesions in childhood include a wide range of pathologies. Benign lesions include lipoma, neurofibroma, lymphangioma, haemangioma and mesenchymal hamartoma. Malignant lesions include neuroblastoma, rhabdomyosarcoma, Ewings sarcoma, Askin tumour and primitive neuroectodermal tumours. Manifestations of systemic diseases such as leukaemia, lymphoma, Langerhans cell histocytosis and infections such as tuberculosis and actinomycosis may also cause chest wall lesions. The imaging characteristics of the above are reviewed but only a minority of lesions show diagnostic imaging characteristics. Most lesions require biopsy and histopathological examination for definitive diagnosis. The role of different imaging modalities is discussed, with an emphasis on magnetic resonance imaging for demonstrating lesion morphology and local spread, with computed tomography and nuclear medicine being used mainly to assess remote disease.
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PMID:Chest wall lesions. 1245 4

Radiological diagnosis of deep soft tissue is often difficult. In the present study, thallium-201 ( Tl) uptake into haemangiomas and deep malignant soft tissue tumours was investigated in order to assess its clinical utility. Tl scintigraphy was reviewed in four patients presenting with soft tissue haemangiomas. Early and delayed planar images, obtained at 15 min and 3 h following the intravenous injection of Tl (111 MBq), were examined. The Tl uptake ratio was calculated by dividing the count density of the tumour region of interest (ROI) by that of the background ROI. Results were compared with those of five cases of rhabdomyosarcoma and a single instance of angiosarcoma. All haemangioma lesions demonstrated increased Tl uptake in early images. However, Tl uptake in delayed images was markedly decreased. No significant differences were observed in the early uptake ratio between haemangiomas (1.60-2.72) and reference malignant tumours (1.48-2.45); however, the difference was significant in delayed images (range, 1.01-1.26 vs. 1.43-2.03, respectively) ( P<0.02). Deep soft tissue haemangiomas revealed Tl accumulation in early images; however, a rapid washout was observed in delayed images. This distinctive feature may facilitate the use of Tl scintigraphy in the diagnosis of haemangiomas.
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PMID:Differentiation of soft tissue haemangioma by 201Tl scintigraphy. 1261 75

A 7-week-old infant with sudden onset adduction deficit and proptosis is reported. The main differential diagnoses included orbital myositis, orbital cellulitis, capillary haemangioma and rhabdomyosarcoma. A CT scan revealed a postseptal cellulitis-like picture with thickening of the medial rectus muscle. He was given a course of antibiotics, withholding steroids and biopsy. His condition resolved completely on high-dose antibiotics alone. To our knowledge this is the youngest patient with infectious orbital myositis and postseptal cellulitis described in the literature. The clinical course emphasizes the importance of administering sufficiently high doses of antibiotics.
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PMID:Acute adduction deficit in a 7-week-old infant. 1266 Aug 49

Most ocular and orbital tumors of childhood are distinct from tumors that occur in adults. Many are congenital with early presentations. Most pediatric orbital tumors are benign; developmental cysts comprise half of orbital cases, with capillary hemangioma being the second most common orbital tumor. The most common orbital malignancy is rhabdomyosarcoma. The most common intraocular malignant lesion is retinoblastoma. Choroidal melanoma, which is common in adults, is extremely rare in children. The orbit is the most common location for metastases in children, whereas the choroid is the predominant site in adults. Pediatricians play a vital role in diagnosis of pediatric ocular tumors. They are the first to recognize ocular problems that may not be apparent to parents. It is therefore important to recognize the signs and symptoms of ocular tumors of childhood so that prompt ophthalmologic evaluation and treatment may be undertaken. Whereas the malignant tumors may be life-threatening, both malignant and benign tumors may be vision-threatening.
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PMID:Pediatric tumors of the eye and orbit. 1271 10

Facial swelling is a common clinical problem in pediatric patients. The causes of swelling are diverse, and knowledge of the typical clinical and imaging manifestations and the most common sites of occurrence of these conditions is needed to formulate a differential diagnosis. The general clinical manifestations may be classified into the following four groups: (a) acute swelling with inflammation, (b) nonprogressive swelling, (c) slowly progressive swelling, and (d) rapidly progressive swelling. Conditions that may account for acute swelling accompanied by inflammation include lymphadenitis, sinusitis, odontogenic infection, and abscess. Contrast-enhanced computed tomography is the modality of choice for detection of abscesses requiring surgical drainage. Nonprogressive midfacial swelling is suggestive of a congenital anomaly (eg, a cephalocele, nasal glioma, or nasal dermoid or epidermoid cyst). Slowly progressive swelling may indicate the presence of a neurofibroma, hemangioma, lymphangioma, vascular malformation, or pseudocyst, or of fibrous dysplasia. The differential diagnosis for rapidly progressive facial swelling in association with cranial nerve deficits should include rhabdomyosarcoma, Langerhans cell histiocytosis, Ewing sarcoma, osteogenic sarcoma, and metastatic neuroblastoma.
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PMID:Causes of facial swelling in pediatric patients: correlation of clinical and radiologic findings. 1641 50

In this review we examine the diagnosis and treatment of pediatric liver tumors- both malignant and benign. The two most common malignant tumors are hepatoblastoma and hepatocellular carcinoma. Hepatoblastoma is seen in younger children, hepatocellular carcinoma in older children. Other malignant liver tumors are quite rare and include biliary rhabdomyosarcoma, angiosarcoma, rhabdoid tumor, and undifferentiated sarcoma. The commonly seen benign liver tumors in children are infantile hemangioma, mesenchymal hamartoma, and focal nodular hyperplasia. Rare benign tumors are hepatic adenoma, which is occasionally seen in teenage girls, and teratoma which is a very rare liver tumor in infants.
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PMID:Tumors of the liver in children. 1771 39

Congenital epulis, also known as congenital gingival granular cell tumor, is a rare benign intraoral tumor found only in the new born. It can be solitary or multiple and may occur in the mandible, maxilla or tongue and may or may not be associated with other congenital anomalies. The size of the mass varies and if very large may interfere with respiration and feeding at birth, thus necessitating the immediate surgical resection at birth to maintain patency of the airways. Antepartum detection by careful imaging and coordination of multidisciplinary team of maternal-fetal medicine, neonatal-perinatal medicine, anesthesiology and otolaryngology and finally the histopathologists who confirm the diagnosis is essential as the histology differentiates it from other congenital intraoral masses like haemangioma, fibroma, rhabdomyoma, rhabdomyosarcoma, lymphangioma, osteogenic and chondrogenic sarcomas.
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PMID:Congenital epulis of the newborn: a case report with review of literature. 1788 49

The histologic spectrum of nonosseous tumors and tumorlike lesions of the extraocular orbit in children differs from that in adults, and the appearance of these lesions at imaging reflects their pathologic features. Rhabdomyosarcoma is the most common extraocular orbital tumor in children. This neoplasm usually manifests in young children, grows quite rapidly, and is fairly vascular. Vasculogenic lesions are common orbital lesions in newborns and young infants. The most prevalent of these are infantile hemangioma, a true neoplasm, and venous-lymphatic malformation, a developmental anomaly. Hemangioma is quite vascular, has a predictable course of proliferation followed by slow involution, and is distinguished on magnetic resonance images by the finding of flow voids within the mass and at its periphery. Venous-lymphatic malformation in the orbit is an anomaly of venous and lymphatic development that is characterized by unenhancing, cystic lymphatic and enhancing, solid venous components. Intralesional hemorrhage is common and frequently produces distinctive fluid-fluid levels within the cystic portions. Unlike hemangiomas, venous-lymphatic malformations grow with the patient and never involute spontaneously. Infantile fibromatosis is one of the fibromatoses and affects newborns and young infants. The tumor is nodular and composed of a zonal architecture, with frequent hemorrhage or necrosis in the central portion, characteristics that confer a target appearance at imaging. These lesions usually stop growing or spontaneously regress. All of these extraocular masses typically manifest with proptosis, and imaging differentiation is desirable because the treatments and prognoses vary greatly.
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PMID:From the archives of the AFIP: Pediatric orbit tumors and tumorlike lesions: nonosseous lesions of the extraocular orbit. 1802 17


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