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

Over the past 9 months, three cases of primary pulmonary rhabdomyosarcoma have been treated at British Columbia Children's Hospital. Two patients (aged 24 and 37 months) presented with spontaneous pneumothoraces and had cystic changes in the affected lung on chest radiograph. The third patient (aged 42 months) was evaluated for chronic cough, fever, and failure to thrive. Chest x-ray showed a large mass in the left lower lobe as well as mediastinal adenopathy. All three of these lesions originated within congenital lung cysts, one a peripheral bronchogenic cyst and the others cystic adenomatoid malformations. This report suggests that there is a significant risk for the development of rhabdomyosarcoma within malformed pulmonary tissue.
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PMID:Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases. 140 24

Magnetic resonance (MR) imaging, ultrasound, and computed tomography (CT) were performed for diagnosis and follow-up of rhabdomyosarcoma (RMS) arising from the genitourinary tract in five children, pelvic musculature in one, and the perineum in three others. MR imaging performed at 1.0 T included the following: spin-density-, T1-, and T2-weighted images in all nine patients; gadopentatate dimeglumine (Gd-DTPA)-enhanced T1-weighted images in five; and short TI inversion recovery (STIR) images in two children. Longitudinal T1-weighted images were of additional help in localizing the primary tumor at diagnosis and detecting local spread. Residual or recurrent disease within the bladder was best detected by spin-density images. Gd-DTPA enhancement improved contrast in two studies, but paramagnetic artifacts obscured intravesical lesions in three other studies. STIR images disclosed pelvic and retroperitoneal lymphadenopathy. MR imaging is recommended as the key method of diagnosis and follow-up of pelvic RMS.
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PMID:Magnetic resonance imaging for diagnosis and follow-up of genitourinary, pelvic, and perineal rhabdomyosarcoma. 147 20

The majority of lesions in the neck of children will prove to be benign and of congenital origin arising from such structures as the thyroglossal duct and the branchial clefts in addition to hemangiomas and lymphangiomas. However, it is essential that lymphadenopathy be proven infectious and any asymptomatic lymph node enlargement must be considered Hodgkin's disease until proven otherwise. The occasional neuroblastoma or rhabdomyosarcoma presenting in the neck can usually be identified as solid by sonography, leading to early investigation and biopsy. Because of the abundance of important structures that course through the neck, surgery should be conducted in an operating suite with sophisticated, modern anesthetic techniques and with a surgeon experienced in dealing with the full array of lesions that occur.
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PMID:Common lumps and bumps of the head and neck in infants and children. 266 78

Forty-two difficult-to-cultivate group A coxsackieviruses (i.e., group A types other than A7, A9, and A16), collected primarily from throat swab specimens of patients suffering from fever, pharyngitis, lymphadenopathy, and cough during the 1986 enterovirus season, were isolated in less than 24-h-old suckling mice. Thirty-six moribund mice were sacrificed and autopsied, and then their brains and back musculature were inoculated into rhabdomyosarcoma (RD), guinea pig embryo (GPE), rhesus monkey kidney (RhMk) and human carcinoma of the larynx (HEp-2) cell cultures. Twelve of the 36 suckling mice isolates were adapted to grow in RD and GPE cells after two passes and have been identified in RD cells by type-specific antisera as group A coxsackievirus types A2, A4, and A8. Three passes in RhMk or HEp-2 cell cultures were insufficient to affect a discernible cytopathic effect. Coxsackievirus types A1, A19, and A22, unable to grow in any of the four cell cultures tested, were identified by virus neutralization in suckling mice. These data denote the efficacy of suckling mice for the isolation of difficult-to-cultivate group A coxsackieviruses.
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PMID:Sensitivity of rhabdomyosarcoma and guinea pig embryo cell cultures to field isolates of difficult-to-cultivate group A coxsackieviruses. 284 70

The configurations of immunoglobulin genes and T-cell receptor beta chain genes were analyzed by Southern blotting in DNA derived from nonlymphoid malignant tumors and lymphomas. Gene rearrangements were not detected in any of the 35 cases of nonlymphoid malignant tumors. On the contrary, they were shown in all 14 cases of non-Hodgkin's lymphomas, 2 of 3 cases of Hodgkin's disease and 2 cases diagnosed as non-Hodgkin's lymphoma or angioimmunoblastic lymphadenopathy. The differentiation by light microscopy between lymphoma and nonlymphoid malignant tumors was a diagnostic problem in five cases; the molecular genetic analysis of DNA was contributory in all five diagnostically difficult aspirates. By gene rearrangement studies, the diagnosis of lymphoma was confirmed in two cases and nonlymphoid malignant tumors were accurately indicated in aspirates diagnosed finally as rhabdomyosarcoma (one case) and carcinoma (two cases).
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PMID:Molecular genetic analysis in the diagnosis of lymphoma in fine needle aspiration biopsies. II. Lymphomas versus nonlymphoid malignant tumors. 321 73

Eighty-nine children with localized rhabdomyosarcoma of orofacial and laryngopharyngeal sites were treated in accordance with the first and second Intergroup Rhabdomyosarcoma Study (IRS) protocols (IRS-I and IRS-II) between 1972 and 1984. Treatment included surgery (or biopsy) and chemotherapy for all patients and radiotherapy in the majority. The actuarial estimate of the three-year survival rate for all patients was 83% and did not differ significantly by primary site, histologic findings, or presence of adenopathy. A trend for a worse survival rate was seen in clinical group III patients and in those less than 5 years of age at diagnosis. Factors associated with an increased risk of local/regional relapse included omission of radiotherapy and a radiation dose of less than 40 Gy (4000 rad). We conclude that treatment of these patients as recommended in the IRS-I and IRS-II protocols results in very good local and regional tumor control and survival rates. Salvage therapy for local/regional recurrence may yield long-term remission and possibly cure.
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PMID:Pediatric orofacial and laryngopharyngeal rhabdomyosarcoma. An Intergroup Rhabdomyosarcoma Study report. 366 51

Seventeen pediatric patients with a major salivary gland malignancy (16 parotid, 1 submaxillary) were reviewed. Eight patients presented with carcinoma. The usual presentation was a mass over the affected gland. Six patients had localized disease, which was treated by excision. This was accomplished by either a total or subtotal parotidectomy or resection of the submaxillary gland. Two patients received adjuvant radiation therapy. All six patients with localized carcinoma are alive, without evidence of disease. Two patients presented with metastatic disease and died of the disease despite treatment with multiagent chemotherapy, and in one case, radiation therapy. Nine patients had rhabdomyosarcoma (RMS). The usual presentation was a mass at the angle of the mandible. Five patients had involvement of one or more cranial nerves, and two had concomitant cervical adenopathy. Eight patients had a biopsy and then were treated according to an existing prospective institutional protocol. The ninth patient initially underwent a superficial parotidectomy. Seven patients received radiation therapy. In one patient, rapid progression of the disease precluded this treatment. Seven patients died of progressive local and distant disease 2 months to 2 years (median, 6 months) from the time of diagnosis. Two patients are alive, without evidence of disease, 3 and 7 years after presentation. We conclude that carcinoma should be managed with complete excision. For RMS of the salivary gland, a biopsy should be performed, and treatment should consist of chemotherapy and radiation therapy.
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PMID:Primary malignancy of the salivary gland in children. 812 Jul 60

The authors describe a case of undifferentiated rhabdomyosarcoma from unknown primary site, presenting as an acute hematologic malignancy with generalized lymphadenopathy, extensive bone marrow involvement and clinical and laboratory features of disseminated intravascular coagulation. Such a peculiar behaviour is known for rhabdomyosarcoma but is rare and can be a serious diagnostic problem for the clinician and the pathologist. The importance of a large spectrum immunohistochemistry as first diagnostic approach to any undifferentiated small-cell malignant tumor is stressed, together with the knowledge of the different immunoreactivity patterns. Desmin, MS-actin and myoglobin are the most reliable markers of this type of myogenic sarcoma.
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PMID:Rhabdomyosarcoma presenting as acute hematologic malignancy: case report and review of the literature. 889 Sep 82

Fine-needle aspiration cytology is a valuable technique in the work-up of nodules and masses arising within the head and neck. Squamous cell carcinoma is present most often, and because of this relative frequency, the primary utility of needle-aspiration cytology is in the confirmation or exclusion of this diagnosis. FNA is particularly helpful in the work-up of cervical masses and nodules because biopsy of cervical adenopathy should be avoided unless all other diagnostic modalities have failed to establish a diagnosis. As such, needle-aspiration cytology represents an accurate, inexpensive, and rapid technique for elucidation of the etiology of cervical adenopathy. The majority of aspirates from cervical lymph nodes will disclose either reactive lymphadenopathy or metastatic squamous cell carcinoma. Occasional nodules will be due to lymphoma. While primary diagnosis of lymphoma by needle-aspiration cytology is generally not considered definitive, it is helpful in clarifying the nature of the process and the direction additional diagnostic tests should take. Similarly, establishing the presence of carotid body tumors, brachial cleft cysts or epidermal inclusion cysts excludes metastatic carcinoma and negates the need for open biopsy as well as allaying concerns on the part of both clinician and patient. Fine-needle aspiration of lesions within the mouth, oral pharynx, nasopharynx, and nasal sinuses has similar diagnostic goals, in that eliminating squamous cell carcinoma is its paramount objective. Fine-needle aspiration cytology can also establish a specific diagnosis for many lesions within this area. This technique can make specific diagnoses of angiofibroma, primary adenocarcinoma of the nasal sinuses, rhabdomyoma, granular cell tumor, and rhabdomyosarcoma. Each of these represents an important clinical entity with a specific therapy. Utilizing electron microscopy and immunohistochemical techniques, along with flow cytometry, can greatly broaden the diagnostic range and specificity of needle-aspiration cytology. Flow cytometry and immunohistochemistry are particularly useful in the establishment of monoclonality in lymphoproliferative processes and, hence, aid in the separation of reactive from lymphomatous lymphadenopathy. Immunohistochemistry can establish the precise nature of lesions as variable as rhabdomyosarcoma, olfactory neuroblastoma, and granular cell tumor. The prudent use of these techniques can be cost-effective and negate the need for more invasive diagnostic procedures. Needle-aspiration cytology represents a cost-effective and rapid technique for the assessment of nodules and masses within the head and neck area. Limitations in accuracy exist. In particular, the separation of reactive atypia in benign squamous epithelium from well-differentiated squamous cell carcinoma may be exceedingly difficult, if not impossible. Nonetheless, the technique has a high degree of accuracy for the diagnosis of both primary and metastatic disease.
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PMID:Fine-needle aspiration of the head and neck. 923 65

A 15-year-old patient is described who presented with cervical lymphadenopathy. A lymph node biopsy confirmed a metastatic neoplasm composed of islands and aggregates of round to polygonal-shaped cells with surrounding hyalinised fibrous stroma. The tumour cells contained a clear glycogen-rich cytoplasm and stained for desmin, muscle-specific actin and sarcomeric actin. Electron microscopy revealed paranuclear thick and thin filaments. These findings confirm striated muscle differentiation and a diagnosis of glycogen-rich clear cell rhabdomyosarcoma. The tumour recurred in a cervical lymph node after 2 years. This is yet another tumour that must be considered in the differential diagnosis of clear cell tumours of the head and neck region.
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PMID:Clear cell (glycogen-rich) rhabdomyosarcoma presenting as cervical lymphadenopathy. 989 72


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