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

Plasma concentrations of neuropeptide Y, analysed in 112 healthy children, decreased significantly with age, while sex, discomfort induced by the sampling procedure or induction of general anaesthesia did not have a significant influence. An age-adjusted upper reference limit was established and proved to be useful when tested prospectively in 56 children with tumours. Two of 18 children with preliminary diagnosis of rhabdomyosarcoma had elevated plasma neuropeptide Y; both showed malignant ectomesenchymoma (p < 0.01), a mixed tumour with neural crest features. Among 38 children with neural crest derived tumours, all 7 with benign ganglioneuromas had plasma neuropeptide Y concentrations below the reference limit, while 13 of 31 with neuroblastoma had elevated concentrations (p < 0.05). Only neuroblastoma patients with elevated plasma neuropeptide Y had a poor outcome; 10 of 13 died, whereas all with normal concentrations are alive after 3-74 months of follow-up (p < 0.001).
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PMID:Plasma neuropeptide Y in healthy children: influence of age, anaesthesia and the establishment of an age-adjusted reference interval. 802 3

Intra-oral soft tissue sarcoma is a rare disease. We reviewed a tumour registry over a 45 year period, between January 1951 and January 1997, and identified a total of 11,250 head and neck cancers in British Columbia. Of the head and neck cancers, there were 139 cases (1.24%) of sarcoma; and of these, there were only 16 cases (0.14%) of intra-oral soft tissue sarcoma. The initial presentation of intra-oral soft tissue sarcoma is most often as an asymptomatic mass and discomfort is reported by less than one half of the patients. The commonest lesion identified was rhabdomyosarcoma (RMS). In this review, we identified 2 unique cases, 1 case of intra-oral hemangiopericytoma and 1 case of oral carcinosarcoma. The prognosis remains poor. Treatment includes wide surgical excision which may be supplemented with radiotherapy and chemotherapy.
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PMID:Head and neck and intra-oral soft tissue sarcomas. 981 25

Early methods of fabricating customized radiation carriers for inaccessible areas, such as the nasopharyngeal space, normally required the patient to be under conscious sedation or general anesthesia to allow impressions for indirect processing techniques. This article describes the use of computed tomography to design and fabricate a carrier for the intracavity application of iodine 125 seeds for a patient with rhabdomyosarcoma of the nasopharynx. With tomography printed copies, a wax pattern was formed, flasked, boiled out, and eventually replaced with silicone material. The patient had to be available only for a conventional thin-cut computed tomography scan without contrast; no fitting appointments or anesthesia were necessary. The indirect procedure eliminated discomfort from impression making and allowed for precise fabrication of the nasopharyngeal carrier. The described technique can be used for other intracavitary radiation applications.
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PMID:Use of computed tomography for the fabrication of a custom brachytherapy carrier: a clinical report. 1258 80

The authors present the case of a 20-month-old boy who underwent fractionated radiation therapy to the paranasal sinuses and anterior skull base during treatment for nasopharyngeal parameningeal rhabdomyosarcoma. Subsequent magnetic resonance imaging demonstrated progressive development of a Chiari malformation Type I (CM-I) and partial hypoplasia of the posterior fossa. Since the tonsillar herniation was discovered, the child, now 3 years old, remains asymptomatic except for mild, intermittent neck discomfort. For the time being, his family has elected for him to undergo clinical and neuroimaging follow up. The authors believe this is the first report of a progressive acquired CM-I after cranial irradiation in the pediatric population.
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PMID:Acquired Chiari malformation type I following fractionated radiation therapy to the anterior skull base in a 20-month-old boy. Case report. 1650 1

We herein present the first-reported case in the English-language literature of a spindle cell rhabdomyosarcoma in the hypopharynx of an adult. A 58-year-old male presented with a progressive, 7-month history of discomfort during swallowing, accompanied by hoarseness for 1 month. Computed tomography and magnetic resonance imaging revealed a tumor in the left pyriform sinus; frozen section evaluation suggested that it was rhabdomyosarcoma. The tumor was removed completely via left lateral cervical incision. Section margins were negative, and laryngeal function was preserved. The postoperative pathological results indicated spindle cell rhabdomyosarcoma. The patient received postoperative radiotherapy (6,000 cGy in 200 cGy fractions, delivered over 30 days). However, he was also required to undergo urgent tracheostomy due to severe laryngeal obstruction, precipitated by laryngeal edema, 3 months subsequent to radiotherapy. The patient was free of disease 25 months postoperatively but is still unable to close his tracheostomy.
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PMID:Spindle cell rhabdomyosarcoma in the hypopharynx of an adult. 2519 6

Lacrimal sac tumours are rare, but must be considered in the diagnosis of patients presenting with masses in the medial canthal region. We report a single case of lacrimal sac rhabdomyosarcoma in a 31-year-old man. The patient self-presented to the eye department with a 4-week history of discomfort, epiphora and a medial canthal mass. After no response to 1 week of oral antibiotics for a presumed diagnosis of dacryocystitis and the presence of firm mass extending above the medial canthal tendon, surgical exploration was carried out which revealed a lacrimal sac mass. Histologically this showed an alveolar rhabdomyosarcoma, which was confirmed on immunohistochemistry. After 4 rounds of chemotherapy and 50.4Gy of radical radiotherapy, the patient is well with no signs of further local or distant disease at 11-months follow-up and 20 months following initial diagnosis. To our knowledge, there are no previously reported adult cases of lacrimal sac alveolar rhabdomyosarcoma in the peer-reviewed literature. We want to highlight the unique diagnosis in this case as well as drawing attention to the possibility of malignancy in patients responding poorly to management when an initial diagnosis of dacryocystitis is made in the presence of a medial canthal mass.
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PMID:Adult alveolar rhabdomyosarcoma of the lacrimal sac. 2520 22

Although the second peak of the age distribution of rhabdomyosarcoma (RMS) is at adolescence, renal RMS is extremely rare at this age group. This tumor is indistinguishable from other renal tumors based on clinical and imaging findings, and the diagnosis relies on histology and immunohistochemical staining. We report a unique case of adolescent renal RMS associated with tumor thrombus extending into the inferior vena cava (IVC) and right atrium.An 18-year-old female adolescent presented with shortness of breath and palpitations, associated with right flank discomfort, and hematuria. A pleomorphic-type renal RMS with Budd-Chiari syndrome and arrhythmia induced by IVC and RA thrombosis was diagnosed. Despite complete tumor resection, the patient developed multiple lung metastases a month after surgery. Chemotherapy was recommended, but the patient declined. She died within a year of the initial operation.Adolescent renal RMS is rare and associated with poor outcome. Early aggressive multimodal therapy seems to be appropriate, in particular, in the presence of tumor thrombosis.
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PMID:Primary Renal Rhabdomyosarcoma in an Adolescent With Tumor Thrombosis in the Inferior Vena Cava and Right Atrium: A Case Report and Review of the Literature. 2722 46