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

We describe a relatively rare case of botryoid rhabdomyosarcoma (RMS) in a 15-month-old female presenting with fever, abdominal pain, urinary retention, haematuria and a tumour protruding through the urethra. The diagnosis was verified by cystoscopy with biopsy and MRI. She was treated with combined chemotherapy and surgery. Early diagnosis and treatment are crucial to the prognosis. RMS should be considered as a differential diagnosis in cases whose symptoms include urinary retention, haematuria, urinary incontinence and atypical abdominal pain, and a primary ultrasound scan should be done. Cystoscopy and MRI should be considered, especially in cases involving atypical urological symptoms.
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PMID:[Rhabdomyosarcoma in the urogenital region of a child]. 1731 57

Malignant tumours of the locomotor apparatus in children mainly comprise bone tumours such as osteosarcoma or Ewing's sarcoma and soft tissue sarcomas such as rhabdomyosarcoma. The diagnosis must be considered in any case of progressively worsening limb pain at a fixed site, possibly associated with a soft tissue mass. Standard radiological examinations, Doppler ultrasound, then MRI confirm the diagnosis of a solid tumour, allow staging and may sometimes suggest the invasive nature of the tumour. The biopsy provides a precise histological diagnosis. A frozen section is usually necessary to confirm this diagnosis using the currently available genetic biology tools. Treatment must be adapted to the known prognostic factors, mainly tumour operability, initial size, histological type, response to neoadjuvant chemotherapy and initial staging. Treatment must comprise neoadjuvant chemotherapy followed by local treatment combining radical surgery with reconstruction if necessary and sometimes external beam radiotherapy. This treatment must be completed by postoperative adjuvant chemotherapy resulting in an average total duration of treatment between 6 and 12 months. Long-term follow-up is designed to ensure absence of disease recurrence, attentive orthopaedic follow-up and absence of late sequelae related to anticancer therapy.
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PMID:[Malignant tumours of the locomotor apparatus in children: an "intermediate" prognosis]. 1784 1

A wide variety of pathologies arise from the petrous apex. Such lesions may present with symptoms caused by mass effect or cranial nerve palsies, or may be detected during an investigation for an unrelated disease. CT and MRI are complementary in providing an appropriate differential diagnosis and in aiding surgical planning. This pictorial review appraises the anatomy and contents of the petrous apex. Benign (e.g. cholesterol granuloma, cholesteatoma), inflammatory (e.g.apical petrositis) and dysplastic (e.g. Pagets disease) lesions of the petrous apex are discussed and illustrated. Whilst it is more frequent for neoplastic lesions to extend from adjacent structures, we demonstrate a variety of aggressive tumours and tumour-like conditions (e.g. metastasis, rhabdomyosarcoma, Langerhan's cell histiocytosis, endolymphatic sac tumour) that directly involve the petrous apex. A range of normal radiological appearances are seen, some of which may be mistaken for significant pathology (e.g. asymmetric marrow space development, simple effusions, cephaloceles). An imaging algorithm to aid the formulation of a differential diagnosis is also presented.
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PMID:Imaging of the petrous apex: a pictorial review. 1820 55

Infants and very young children with malignant brain tumors usually have unfavourable locations and are not candidates for craniospinal irradiation. New therapeutic approaches must be attempted to improve poor survival rates. The primary goal of the present study was to report on the safety profile and toxicity of intrathecal administration of liposomal cytarabine in children <4 years with malignant brain tumors. This is the first study including this group of patients receiving intrathecal liposomal cytarabine. Nine patients with a median age of 26 months were eligible for the study. The diagnoses were ependymoma (3), peripheral neuroectodermic tumor (PNET) (2), meduloblastoma, atypical teratoid rhabdoid tumor (ATRT), cerebral lymphoma, and rhabdomyosarcoma with CNS invasion. Liposomal cytarabine at doses between 20 and 35 mg were administered by lumbar puncture. Dexamethasone was given for arachnoiditis prophylaxis. A total of 44 doses (median = 6) of liposomal cytarabine were administered. Neurological side effects possibly related to liposomal cytarabine were observed in five patients (55.6%). This incidence was higher than previously reported in children older than 3 years. Eight of the patients (89%) experienced an initial improvement of clinical symptoms after initiation treatment, confirmed by MRI. This study demonstrates the feasibility of using intrathecal liposomal cytarabine in children under 4 years of age with malignant brain tumors.
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PMID:Intrathecal liposomal cytarabine in children under 4 years with malignant brain tumors. 1938 44

We aimed to examine different intratumoral changes after single-dose and fractionated radiotherapy, using diffusion-weighted (DW) and dynamic contrast-enhanced (DCE) MRI in a rat rhabdomyosarcoma model. Four WAG/Rij rats with rhabdomyosarcomas in the flanks received single-dose radiotherapy of 8 Gy, and four others underwent fractionated radiotherapy (five times 3 Gy). In rats receiving single-dose radiotherapy, a significant perfusion decrease was found in the first 2 days post-treatment, with slow recuperation afterwards. No substantial diffusion changes could be seen; tumor growth delay was 12 days. The rats undergoing fractionated radiotherapy showed a similar perfusion decrease early after the treatment. However, a very strong increase in apparent diffusion coefficient occurred in the first 10 days; growth delay was 18 days. DW-MRI and DCE-MRI can be used to show early tumoral changes induced by radiotherapy. Single-dose and fractionated radiotherapy induce an immediate perfusion effect, while the latter induces more intratumoral necrosis.
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PMID:Dynamic contrast-enhanced and diffusion-weighted MRI for early detection of tumoral changes in single-dose and fractionated radiotherapy: evaluation in a rat rhabdomyosarcoma model. 1950 9

The authors present a rare form of orbital cavitary rhabdomyosarcoma in which lymphangioma was mistakenly diagnosed on the basis of echography and MRI. Rhabdomyosarcoma can usually be differentiated from lymphangioma by echographic and MR imaging, because cavitation is very rare in orbital rhabdomyosarcoma, but rhabdomyosarcoma should be suspected whenever the clinical presentation of a rapidly progressive unilateral exophthalmos is observed in a child.
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PMID:Orbital cavitary rhabdomyosarcoma: a diagnostic dilemma. 2030 11

Vascular tumours and malformations, fibrous and fibrohistiocytic tumours and pseudotumours are the most common benign soft-tissue masses observed in children, and can be treated conservatively. Rhabdomyosarcomas are the most frequent malignant tumours, accounting for about half of soft tissue sarcomas. A child referred for a soft-tissue mass should ideally be managed by a multidisciplinary team and primary excision should be proscribed until a definite diagnosis has been established. Clinical examination, conventional radiography and US with Doppler represent the first-line examinations and are sometimes sufficient to make a diagnosis. In all other situations, MRI is mandatory to establish the aggressiveness and extension of the tumour. This technique provides the relevant data to guide the decision regarding tissue sampling.
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PMID:Soft tissue tumours: imaging strategy. 2043 21

The common childhood cancers are leukemia, CNS tumors, lymphomas, soft-tissue tumors (such as rhabdomyosarcoma and fibrosarcoma), neuroblastoma, malignant bone tumors, germ cell tumors with neoplasms of gonads and hepatic tumors. Usually the conventional imaging modalities, such as x-ray, ultrasound, computed tomography (CT) and MRI, are being routinely used for the management of these pediatric malignancies. However, most of these modalities provide structural information and are lacking in functional/metabolic status of these malignancies. Recently, PET and PET/CT have emerged as a functional diagnostic imaging modality for the management of various cancers in adult population. Up to now most of the data published in the literature are on PET alone. PET used in conjunction with CT is useful as it provides an enhanced view of the anatomical details and the malignant focus then can be located with highest accuracy. PET and PET/CT has been found to be useful in, for example, CNS tumors, lymphomas, soft-tissue tumors, neuroblastoma, malignant bone tumors and germ cell tumors. PET/CT has a limited role in early diagnosis, however, it plays an important role in initial staging, treatment response evaluation and detection of metastatic disease in these cancers. Despite the fact that PET/CT has better diagnostic value when compared with conventional imaging, such as CT and MRI, in the management of many pediatric cancers, there are certain limitations. PET/CT has a limited role in detection of lesions smaller than 5 mm, well-differentiated tumors and tumors with low metabolic rate. Many infections and inflammation can lead to false-positive PET/CT results. In the present review we will discuss the various clinical indications of PET and PET/CT in pediatric cancers.
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PMID:Clinical applications of PET and PET/CT in pediatric malignancies. 2047 7

We report a case of alveolar rhabdomyosarcoma arising between the fourth and fifth metatarsal. A 13-year-old boy presented to outpatients with a history of pain and swelling in the lateral aspect of his left forefoot. Plain radiographs and MRI showed a soft tissue mass displacing the fourth metatarsal. Percutaneous biopsy revealed an alveolar rhabdomyosarcoma. Staging scans showed advanced metastatic disease. The patient was treated with chemotherapy. This highly malignant lesion remains challenging to diagnose, and difficult to treat successfully.
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PMID:Alveolar rhabdomyosarcoma originating between the fourth and fifth metatarsal--case report and literature review. 2065 99

We report a case of extensive paranasal sinus and orbit rhabdomyosarcoma (RMS) with intra-cranial invasion treated successfully with chemotherapy and radiotherapy. A 13-years-old male patient complained of painless and progressive proptosis of his left eye for two weeks. Ocular examination showed elevated intraocular pressure, limited extraocular movement, proptosis, and conjunctival ciliary injection in the left eye. Brain CT and MRI demonstrated a large enhancing soft tissue mass lesion with bone destruction involving left ethmoid sinus, nasal cavity, maxillary sinus, and orbital cavity with crossing of the midline to the right ethmoid sinus, nasal cavity, and intra-cranial invasion across the frontal base. The pathology of tumor biopsy revealed rhabdomyosarcoma, alveolar type. Systemic survey showed no evidence of distant metastasis. Then, the patient received combined radiochemotherapy with Taiwan Pediatric Oncology Group Rhabdomyosarcoma 2007 High-risk Treatment Protocol. No light perception in his left eye with optic disc atrophy was noted at the beginning of radiotherapy. After 44 weeks of combined radiochemotherapy, the tumor regressed, and no recurrence has been noted until now. In young patients with sudden-onset proptosis, RMS should be considered, and early diagnosis is crucial due to more effective prognosis with current radiochemotherapy protocol.
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PMID:Extensive alveolar-type paranasal sinus and orbit rhabdomyosarcoma with intracranial invasion treated successfully. 2142 Dec 11


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