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)

Uterine rhabdomyosarcoma is rare, with only 60 reported cases. We describe an asymptomatic patient with metastatic uterine rhabdomyosarcoma manifested as extensive mediastinal lymphadenopathy. This 73-year-old woman had been previously treated for endometrial rhabdomyosarcoma and was referred to us when a right hilar density was seen on a chest radiograph; computed tomography showed a nodule in the right upper lobe and extensive right hilar and subcarinal lymphadenopathy. The diagnosis of metastatic rhabdomyosarcoma was made by fiberoptic bronchoscopy using transbronchial needle aspiration (TBNA). The patient is well 11 months after completing five cycles of chemotherapy. This is the first reported case of uterine rhabdomyosarcoma with metastasis to the mediastinum and the first case diagnosed with TBNA, which avoided the need for an invasive diagnostic procedure.
 ...
PMID:Uterine rhabdomyosarcoma metastatic to mediastinal lymph nodes: diagnosis by transbronchial needle aspiration. 993 36

Cervicothoracic lesions are not uncommon in children. All cervicothoracic lesions except superficial lesions extend from the neck to the thorax through the thoracic inlet. Evaluation of this area involves multiple imaging modalities: plain radiography, ultrasonography, nuclear medicine, computed tomography, and magnetic resonance (MR) imaging. However, MR imaging is the method of choice for assessing the full extents of cervicothoracic lesions and their relationships to neurovascular structures. Cervicothoracic lesions can be classified as congenital lesions, inflammatory lesions, benign tumors, malignant tumors, and traumatic lesions. Lymphangioma is the most common cervicothoracic mass in children; other congenital lesions include hemangioma, thymic cyst, and vascular anomalies. Inflammatory adenopathy reactive to tuberculosis, mononucleosis, tularemia, cat-scratch fever, infection with human immunodeficiency virus, or other upper respiratory tract infections can manifest as cervicothoracic lesions; tuberculous abscesses and abscesses of other origins can also be seen. Lipoma, lipoblastoma, aggressive fibromatosis, and nerve sheath tumors (either isolated lesions or those associated with neurofibromatosis) can also occur as cervicothoracic masses. Malignant cervicothoracic tumors include lymphoma, thyroid carcinoma, neuroblastoma, and chest wall tumors (rhabdomyosarcoma, Ewing sarcoma, and neuroectodermal tumor). Traumatic cervicothoracic lesions include pneumomediastinum of traumatic origin, traumatic pharyngeal pseudodiverticulum, esophageal foreign-body granuloma, and cervicothoracic hematoma.
 ...
PMID:Cervicothoracic lesions in infants and children. 1033 90

Five cases of alveolar rhabdomyosarcoma with atypical clinical features are reported. Three patients showed lymphadenopathy as the first clinical manifestation, mimicking a lymphoma or a non identified primary tumor with lymph node metastases. One patient presented systemic neoplastic disease and two had the primary tumor in atypical locations, such as the mediastinum and retroperitoneum. All patients died and in four of them an autopsy was performed. The histological diagnosis was confirmed by immunohistochemical studies on four cases. The alveolar rhabdomyosarcoma has a poor prognosis and can have a variable clinical presentation and morphology, simulating lymphomas, leukemias and systemic metastatic disease with an unknown primary neoplasm, such as in the cases here in reported.
 ...
PMID:[Atypical biological behavior of alveolar rhabdomyosarcoma in five patients]. 1034 63

We report on a case of embryonal rhabdomyosarcoma in an 11-yr-old boy investigated for mild chest pain after trauma. Chest radiography showed a massive right pleural effusion. Cytological analysis of the pleural fluid demonstrated the presence of malignant small undifferentiated cells. The rhabdomyoblastic nature of the cells was confirmed by positive immunostains of HHf35 actin, desmin, and skeletal muscle myosin; histological examination of a core biopsy confirmed the diagnosis of rhabdomyosarcoma. Computed tomography and gallium scan revealed the presence of an extensive anterior and lower chest wall mass involving the mediastinum, as well as retroperitoneal lymphadenopathy. Massive pleural effusion is a frequent presentation in malignant disease, but is rare in rhabdomyosarcoma. The immunochemical stain studies performed on cytological smears in this case proved to be very useful for making the definitive and accurate diagnosis. Diagn. Cytopathol. 1999;21:125-128.
 ...
PMID:Rhabdomyosarcoma in a child with massive pleural effusion: cytological diagnosis from pleural fluid. 1042 51

This retrospective study was undertaken to determine the prevalence, clinical characteristics, management methods and prognosis of testicular cancer at Kenyatta National Hospital, Nairobi. All histologically confirmed testicular cancer patients recorded at the Histopathology Department between 1993 and 1997 were analyzed. The mean age was 34.8 years with a peak incidence in the 30-44 year age group. About 10.26% of patients had history of cryptochirdism. The clinical symptoms presented were painless testicular swelling (n = 31, 79.49%), testicular pain (n = 11, 28.08%), scrotal heaviness (n = 9, 23.08%), abdominal swelling (n = 6, 15.38%), gynecomastia (n = 1, 2.56%), and eye swelling (n = 1, 2.56%). On examination, 32 patients (82.05%) had testicular masses, 10 (25.64%) had abdominal masses, 7 (17.91%) had supraclavicular and cervical lymphadenopathy, 1 had gynecomastia, and 1 had an orbital mass. More than 89% of patients had germ cell cancers with seminoma accounting for 67.35%, teratoma for 12.24%, embryonal carcinoma for 8.16%, rhabdomyosarcoma for 6.12%, and malignant germ cell tumor, orchioblastoma, and dysgerminoma each accounting for 2.04%. The various methods of treatment include orchidectomy and radiotherapy and chemotherapy in 3 patients (7.7%), orchidectomy and radiotherapy in 16 patients (41.03%), orchidectomy and chemotherapy in 6 patients (15.38%), and radiotherapy and chemotherapy in 10 patients (25.64%). No cisplatin-based chemotherapy was used. 18 patients were followed up, of whom 7 were alive after 5 years. Prognosis with current regimens was poor, with a 38.89% survival ratio in 5 years. Hence, cisplatin-based chemotherapy with up to 90% cure rates should be included in the testicular cancer management in this hospital.
 ...
PMID:Testicular cancer at Kenyatta National Hospital, Nairobi. 1077 80

A 17-year-old boy diagnosed with an alveolar rhabdomyosarcoma involving the perineum and with extensive lymphadenopathy was treated with chemotherapy yet developed metastases to the head and neck 6 months into therapy. Ten months after initial diagnosis, while receiving salvage chemotherapy and radiotherapy, he returned with pain on movement of his left eye, proptosis, and ptosis of the left upper eyelid. Computed tomography (CT) revealed a mass within the left lateral rectus muscle that biopsy confirmed to be metastatic alveolar rhabdomyosarcoma. Despite continued chemotherapy and radiotherapy, he ultimately died of the disease. Alveolar rhabdomyosarcoma from distant sites rarely metastasizes to the extraocular muscles. However, our case shows that alveolar rhabdomyosarcoma may metastasize to the orbit and involve a single muscle.
 ...
PMID:Primary perineal alveolar rhabdomyosarcoma metastatic to an extraocular muscle. 1287 88

The objective of the present study was to review the presentation, management, outcome and morbidity of paediatric patients presenting to a single centre with rhabdomyosarcoma of the ear and temporal region. All patients diagnosed with rhabdomyosarcoma of the ear and temporal region between 1980 and 2000 were entered into this retrospective study. Fourteen patients were identified. The median age at presentation was 4.5 years with a mean time of onset of symptoms to diagnosis of 21 weeks. In many patients, the presentation mimicked that of chronic otitis media, delaying diagnosis. Histological subtype was embryonal in 13 patients and alveolar in 1. All patients underwent multimodality treatment. The 5-year disease-free survival rate was 81%. Regional post-treatment morbidity included chronic aural discharge (6/14), facial palsy (8/14), growth disturbance (4/14) and maxillo-facial deformity occurring in four children. From the results, we conclude that these patients should usually present to an ENT surgeon who should keep the diagnosis in mind when dealing with children with chronic otitis media as early diagnosis with referral to a specialist multidisciplinary team will optimize the chance of survival. Discharge, hearing loss and aural polyp, although commonly because of chronic otitis media, should prompt urgent investigation and biopsy, particularly if associated with facial palsy, lymphadenopathy or an obvious mass.
 ...
PMID:Paediatric rhabdomyosarcoma of the ear and temporal bone. 1496 49

We report a nephroblastoma arising in a germ cell tumor of testicular origin occurring in a 22-year-old man. Orchiectomy demonstrated a malignant mixed germ cell tumor composed of mature and immature teratoma with nephroblastoma and rhabdomyosarcoma. Following chemotherapy, the patient developed supraclavicular and retroperitoneal lymphadenopathy. Excision demonstrated metastatic teratoma at both sites. No recurrence was noted with 21 months of additional follow-up. Using tissue microdissection and loss of heterozygosity analysis, we investigated the clonality of the mature teratoma, immature teratoma, nephroblastoma, and rhabdomyosarcoma components of the primary tumor and of the metastatic mature teratoma at the two separate distant sites. Nine microsatellite polymorphic makers were used to examine the pattern of allelic loss in both primary and metastatic tumors. Loss of heterozygosity was found in 4 DNA loci, and the same pattern of allelic loss was demonstrated at all 4 loci in all of the different components of the primary tumor and the metastatic mature teratomas, supporting the germ cell tumor origin of the nephroblastoma component. Loss of heterozygosity on chromosome 17p13 (TP53) was detected in metastatic mature teratoma, but not in the primary tumor. Loss of heterozygosity was observed at 11p13, the locus of WT1 inactivation in patients genetically predisposed to nephroblastoma, and this loss may be an important genetic mechanism in nephroblastomatous differentiation of germ cell tumors. These data support a common clonal origin for nephroblastoma and the other germ cell tumor components.
 ...
PMID:Nephroblastoma arising in a germ cell tumor of testicular origin. 1510 60

A 22-year-old para 2, female presented with a 2-month history of a progressively vulvar mass. Clinically, her general condition was poor. She had bilateral inguinal lymphadenopathy. Local examination revealed a large deeply infiltrating vulvar mass. Pathological evaluation revealed pleomorphic rhabdomyosarcoma.
 ...
PMID:Aggressive rhabdomyosarcoma of the vulva in a young Sudanese woman. 1733 81

Alveolar rhabdomyosarcoma is a high-grade neoplasm, which forms about 30% of rhabdomyosarcomas. A rare solid variant has been described. A 14-year-old girl presented with inguinal lymph nodal mass and was treated with 6 cycles of CHOP chemotherapy and local radiation. After 3 months, she presented with generalized lymphadenopathy, pleural and pericardial effusions. A histopathologic diagnosis of solid variant of alveolar rhabdomyosarcoma was made. The skeletal muscle origin was confirmed by positive immunostaining for desmin. A primary site was identified in the lower limb muscles. The patient was treated with salvage chemotherapy but had progressive disease. The pediatric and adolescent cases of this rare tumor reported in English language literature are reviewed. In addition, the importance of biopsy in the diagnosis of suspected lymphomas and the pitfalls of needle aspirations are briefly discussed.
 ...
PMID:Solid variant of alveolar rhabdomyosarcoma mimicking non-Hodgkin lymphoma: case report and review of literature. 1901 78


<< Previous 1 2 3 4 Next >>