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)

A case of ganglioneuroblastoma associated with malignant mesenchymoma is reported. The tumor originated from the retroperitoneum and was comprised not only of neuroblastoma and ganglioneuroma, but also of typical rhabdomyosarcoma, liposarcoma, undifferentiated mesenchymoma, as well as cartilaginous tissue. No distinct border existed between thses different components. The histogenesis of the tumor is discussed. We suggest that the tumor derived from a remnant of neural crest (ectomesenchyme).
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PMID:Ganglioneuroblastoma associated with malignant mesenchymoma. 118 58

The immunohistochemical distribution of alpha and beta subunits of S-100 protein (S-100 alpha, S-100 beta, respectively) in 138 cases of human brain tumors was investigated by the avidin-biotin immunoperoxidase method. Brain tumors can be divided into four groups: group 1 [S-100 alpha (+) and/or S-100 beta (+)]; astrocytoma, glioblastoma, ependymoma, subependymoma, oligodendroglioma, choroid plexus papilloma, gangliocytoma, meningioma, chordoma, malignant melanoma. Group 2 [S-100 alpha (+) and S-100 beta (-)]; pineoblastoma, pituitary adenoma, craniopharyngioma, rhabdomyosarcoma. Group 3 [S-100 alpha (-) and S-100 beta (+)]; acoustic Schwannoma. Group 4 [S-100 alpha (-) and S-100 beta (-)]; medulloblastoma malignant lymphoma, germinoma. The S-100 beta immunoreactivity pattern in brain tumors was similar to those obtained using conventional anti-S-100 protein sera. In the first group of brain tumors both the number of positively stained tumor cells and the staining intensity were generally greater for S-100 beta than for S-100 alpha with a few exceptions including one gemistocytic astrocytoma, one subependymoma, one malignant melanoma, and some cases of glioblastomas. As to the relationship between malignancy and S-100 protein in glioma, S-100 beta immunoreactivity decreased according to degree of malignancy, while that of S-100 alpha varied, suggesting a heterogeneity of tumor cells in glioblastomas. Immunostaining for S-100 alpha and S-100 beta might become a useful diagnostic procedure in brain tumors and may give us more detailed and precise data of S-100 protein in brain tumors.
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PMID:Immunohistochemical study on the distribution of alpha and beta subunits of S-100 protein in brain tumors. 188 40

Long-term results of complete removal of vertebrae with a minimum follow-up period of seven years are reported in 23 consecutive patients from March 1968 to January 1981. Seven patients were treated with vertebrectomy above the sacrum: three for a giant-cell tumor (T11, T12, and L1; T11; and L4), one for chondrosarcoma (one-half T6, T7, and one-half T8), one for chordoma (L3), one for plasmocytoma (L1), and one for a metastasis of renal carcinoma (L1). The latter two patients eventually died of generalized disease, whereas the other five patients have no evidence of tumor after seven to 20 years. In the six patients in whom the spine was reconstructed using corticocancellous iliac bone, a block-vertebra was created by the grafts and the adjacent vertebrae, allowing two women to complete one or more pregnancies successfully. Sixteen patients were treated with removal of sacral vertebrae (from one-half S3, S4, and S5 to all). None of the seven patients treated with adequate surgery for a sacral chordoma has had a local recurrence. Two women have given birth to children after sacral amputations, one performed for rhabdomyosarcoma and the other for a huge ganglioneuroma. A patient treated with a hemicorporectomy for chondrosarcoma 18 years ago has no evidence of tumor.
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PMID:Complete removal of vertebrae for extirpation of tumors. A 20-year experience. 275 35

Peripheral primitive neuroectodermal tumor (PPNET) is rare, occurring most often in young adults. Approximately 50 cases have been reported, with only four cases involving the female genital tract. We report the fifth patient. The term "PPNET" should be used only for tumors with neuroectodermal elements exclusively that occur at sites outside the central and sympathetic nervous system. The pathologic differential diagnoses include rhabdomyosarcoma, immature malignant teratoma, small cell carcinoma of the cervix, and ganglioneuroma. Therapy for this tumor has varied, and no effective regimen has been established.
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PMID:Malignant peripheral primitive neuroectodermal tumor of the uterus. 303 96

A 20-year-old man had a left parapharyngeal neoplasm that displayed features of sarcoma of the nerve sheath, meningioma with an osseous component, rhabdomyosarcoma, and ganglioneuroma. The diverse cellular population suggests a tumor origin from the ectomesenchymal remnants of the neural crest. The mesenchymal part of the crest would differentiate into a variety of mesenchymal neoplastic elements, and the neuroectodermal (neuroepithelial) portion into the ganglioneuroma. These various neoplastic constituents then would form a neoplasm of mixed mesenchymal and neuroepithelial origin, or an ectomesenchymoma.
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PMID:Parapharyngeal neoplasm of mixed mesenchymal and neuroepithelial origin. 624 67

Three enolase isozymes (alpha alpha, alpha gamma, and gamma gamma) and S-100 protein in the extract of neuroendocrine tumors (neuroblastoma, ganglioneuroblastoma, ganglioneuroma, and pheochromocytoma) and nonneuroendocrine tumors (Wilms' tumor, rhabdomyosarcoma, and hepatoblastoma) were determined by means of enzyme immunoassay systems. All of the tumors examined showed a high level of alpha alpha-enolase (1.71 to 19.0 micrograms/mg protein). Levels of nervous system-specific enolases (NSE; alpha gamma and gamma gamma) in the neuroendocrine tumors were also rather high (alpha gamma, 1.64 to 7.45 micrograms/mg protein; gamma gamma, 0.052 to 5.56 micrograms/mg protein). However, the NSE concentration in the extract of nonneuroendocrine tumors was low (alpha gamma, less than 0.88 micrograms/mg protein; gamma gamma, 0 microgram/mg protein). The level of S-100 protein was relatively high in ganglioneuroma (greater than 500 ng/mg protein) and ganglioneuroblastoma (greater than 100 ng/mg protein), but low in neuroblastoma (less differentiated neuroendocrine tumor) and nonneuroendocrine tumors. Serum levels of enolase isozymes were also determined in neuroblastoma patients before and after resection of primary tumor or effective chemotherapy. The elevated level of serum NSE (alpha gamma and gamma gamma) was markedly decreased with little change in the alpha alpha level by the treatment.
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PMID:Determination of three enolase isozymes and S-100 protein in various tumors in children. 631 26

Twenty-one children with predominantly solid tumours detected by US were also examined by MRI (7 neuroblastomas, 2 Wilms tumours, 3 hepatoblastomas, 2 germinal cell tumours, 1 ganglioneuroblastoma, 1 gangliocytoma, 1 Cushing's adenoma, 1 phaeochromocytoma, 1 retroperitoneal rhabdomyosarcoma, 1 diffuse lymphangiectasia of a kidney, 1 splenunculus). The findings from both methods were compared with respect to the identification of the organ involved, extent of the tumour, effect on neighbouring structures and tissue characteristics. US and MRI were of equal value in defining the origin of the lesion and in demonstrating pathological enlargement of lymph nodes. Exact tumour extent could be better demonstrated with MRI because of the ability to perform multiplanar sections and to demonstrate intrathoracic and intraspinal spread. MRI was superior in 9 cases in demonstrating tumour structure and in 6 cases in the evaluation of vascular involvement and vascular anatomy. MRI is therefore recommended as an additional method to US for diagnosis and for treatment planning.
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PMID:[Abdominal tumors in children. A comparison between magnetic resonance tomography (MRT) and ultrasonography (US)]. 842 85

The authors report a series of 10 children under 15 years of age with primary intraspinal neoplasm who underwent surgical resection from 1981 to 1994. The tumors consisted of 4 intramedullary tumors (myxopapillary ependymoma, pilocytic astrocytoma, subpial liporna, cavernous angioma), 2 intradural extramedullary tumors (2 neurinomas), and 4 extradural tumors (ganglioneuroma, chordoma, osteochondroma, rhabdomyosarcoma). The initial symptoms in these patients were gait disturbance (50%), weakness of lower extremities (50%), pain (40%), weakness of upper extremities (10%), and tetraparesis (10%). The neurological findings on admission in these patients revealed motor paresis (90%), sensory disturbance (60%), abnormality of deep tendon reflex (60%), pathological reflex (30%), neurogenic bladder (30%), and Lasegue's sign (10%). All patients were treated surgically; laminectoy was performed in 8 patients, laminoplasty in one patient, and transoral approach in one patient. Five patients (50%) underwent a grossly total resection, 3 (30%) had a subtotal resection, and 2 (20%) had a partial resection. Mean follow-up period was 5.6 years. One (10%) patient died due to recurrence and dissemination of the tumor at eight months after surgery in a case of rhabdomyosarcoma. 9 (90%) have had no recurrent tumor and have improved neurologically. In 4 patients (40%) spinal deformity developed, and 2 patients required operative treatment for the spinal deformity. We emphasized the advantage of laminoplasty to prevent postoperative spinal deformity in children.
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PMID:[Clinical study of intraspinal neoplasms in children]. 875 72

Rhabdomyosarcoma is a common childhood malignancy that may occasionally occur as a component of a mixed mesenchymal tumor, e.g., a triton tumor or malignant ectomesenchymoma. A case is reported of a 13-year-old boy who had resection of a paratesticular embryonal rhabdomyosarcoma with subsequent radiation and chemotherapy. Two years later, a retroperitoneal metastasis was resected. Histology showed a mixture of rhabdomyoblasts, ganglion cells, and a third population of cells with combined features of these two distinct cell types. Electron microscopy confirmed the presence of rhabdomyoblasts with characteristic bundles of myofilaments and Z-band material, and ganglion cells with prominent nuclei and nucleoli, rough endoplasmic reticulum, dense core granules, filaments, and tubules. Notably, the third cell population showed features of both rhabdomyoblasts and ganglion cells. Immunohistochemistry confirmed the mixed population of rhabdomyoblasts (positive for vimentin, desmin, negative for S-100, NSE), ganglion cells (positive for S-100 and NSE, negative for vimentin and desmin), and the third population expressing all test antigens. The features of this metastatic lesion are those of a malignant ectomesenchymoma with combined rhabdomyosarcoma and ganglioneuroma components. It is postulated that this lesion results from biphenotypic expression of tumor cells that previously expressed only rhabdomyoblastic differentiation. The role of prior chemotherapy and radiotherapy in this particular case is unclear.
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PMID:Rhabdomyosarcoma metastasizing as a malignant ectomesenchymoma. 1050 46

Low-affinity nerve growth factor receptor (p75) is a member of the tumor necrosis factor receptor family. It may modulate the binding of nerve growth factor (NGF) to the functional high-affinity receptor tyrosine kinase (trk) A. NGF is thought to be responsible for growth, apoptosis, and function of the nervous system. The presence of this receptor (p75) was determined in a large group of neural and nonneural tumors and fetal and adult tissues. One thousand one hundred fifty tumors were analyzed with monoclonal antibody for p75, along with selected normal fetal and adult tissues. Immunoreactivity for p75 was present in adult pericytes, perivascular fibroblasts, basal cells of several types of epithelia, perineurial cells, and dendritic reticulum cells. Additionally, a wide zone of subepithelial mesenchyme and skeletal muscle were positive in the first-trimester fetus, but were diminished or negative in the adult. Consistently positive nonneural mesenchymal tumors included dermatofibrosarcoma protuberans (DFSP), embryonal and alveolar rhabdomyosarcoma, synovial sarcoma, and spindle cell hemangio(endotheli)oma. Schwann cell tumors, ganglioneuroma, granular cell tumor, and malignant peripheral nerve sheath tumor (MPNST) were also p75 positive. Mesenchymal nonneural tumors that were variably positive (32% to 69%) for p75 included fibrosarcoma variants, solitary fibrous tumor, hemangiopericytoma, spindle cell lipoma, Ewing's sarcoma, mesenchymal chondrosarcoma, and malignant melanoma. Nervous system tumors such as paragangliomas, neuroblastoma, meningioma, and perineurioma and nonneural mesenchymal tumors, including extraskeletal osteosarcoma, benign fibrous histiocytomas, fibromas, alveolar soft part sarcoma, epithelioid sarcoma, smooth muscle and gastrointestinal stromal tumors, and angiosarcomas, were almost always negative for p75. Epithelial tumors that were consistently positive included mixed tumor and adenoid cystic carcinoma, whereas mesothelioma, adenocarcinomas, and most squamous cell carcinomas were negative. p75 is not a specific marker for nerve sheath tumors. It is present in a variety of other mesenchymal tumors including synovial sarcoma and in CD34-positive tumors such as DFSP, spindle cell lipoma, and hemangiopericytoma. The presence of p75 in nonneural tumors such as DFSP and rhabdomyosarcoma mimic its presence in early fetal mesenchyme and skeletal muscle, suggesting oncofetal expression in these tumors. p75 may be useful to distinguish DFSP from benign fibrous histiocytoma.
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PMID:Low-affinity nerve growth factor receptor (p75) in dermatofibrosarcoma protuberans and other nonneural tumors: a study of 1,150 tumors and fetal and adult normal tissues. 1156 28


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