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

The study was concerned with development of parameters to be used in making prognosis of survival in squamous-cell cancer of the skin, fibrosarcoma, malignant fibrous histiocytoma of the bone, leiomyosarcoma, rhabdomyosarcoma, secondary chondrosarcoma, chondroma and osteochondroma. One of them--tumor growth rate--is a maximum tumor size/case history duration ratio (Formula: see text). Other coefficients represent ratios between some characteristics (maximum size or Tt.s) and parameters of a patient's status (height, weight/height ratio, body surface area). A correlation between the suggested coefficients and survival was established.
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PMID:[Clinico-morphological indices for the differential diagnosis and prognosis of the course of a series of malignant tumors]. 282 Jan 46

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

Chest wall tumors (CWT) are rarely seen in childhood and surgery constitutes a complementary part of the therapy. The early and late results of CWT resection and chest wall reconstruction were evaluated retrospectively. The children who underwent chest wall resection for CWT between January 1990 and November 2003 were evaluated retrospectively. Seventeen children (male/female = 12/5, mean age: 7.58 years) underwent chest wall resection for CWT. Fifteen patients underwent initial biopsy (tru-cut, n = 8 or open biopsy, n = 7) and two underwent initial resection. The diagnosis was malignant tumor in 12 (70%) and benign in 5 (30%). They were Ewing's sarcoma (ES) (n = 4), primitive neuroectodermal tumor (PNET) (n = 3), Askin's tumor (n = 1), rhabdomyosarcoma (RMS) (n = 2), neuroblastoma (n = 2), osteochondroma (n = 1), aneurysmal bone cyst (n = 2) and hamartoma (n = 2). Preoperative chemotherapy was given to most patients with malignant tumor. All patients had only local tumor at the time of resection. Thoracotomy was performed in all patients. All tumor tissues with the affected rib/ribs were resected en bloc with the adjacent tissues. The number of resected ribs was 1 (n = 6), 2 (n = 7) and 3 (n = 4). Chest wall defects were repaired primarily (n = 8) or with grafts (n = 9). Dura (n = 4), Neuro-patch (n = 3) and Goretex (n = 2) were used for closure. Wound infection and pleural fistula occurred in one patient. Patients with benign tumor were free of complaints or complications during follow up. All patients with malignant tumor received postoperative chemotherapy. Local recurrence did not occur in all patients. Five patients developed distant metastasis and two died. Scoliosis was encountered in one patient during follow-up. Since most of the CWT are malignant and not initially suitable for surgical excision, the management includes tissue diagnosis either by tru-cut or open biopsy. Determination of malignant condition should be followed by an intensive chemotherapy. Chest wall resection is planned to control local disease. Chest wall reconstruction may be needed for large defects following resection of CWT. Prosthetic materials can be used safely. Early complications of the surgery are limited. The patients should be closely followed up for late complications such as scoliosis, restrictive pulmonary disease and for the development of metastasis, which is a part of natural course of malignant CWT in children.
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PMID:The results of surgical treatment of chest wall tumors in childhood. 1632 38

We conducted a retrospective case-series review to identify the various diagnoses of neoplasms of the nasal cavity and paranasal sinuses in a pediatric population. Our study group was made up of 54 children-23 boys and 31 girls, aged 8 months to 16 years (mean: 9 yr). All patients had been diagnosed with a tumor of the nasal cavity or paranasal sinuses between Jan. 1, 1955, and Dec. 31, 1999, at one of four university-based, tertiary care referral centers. We compiled data on tumoral characteristics (location, size, and histopathology), morbidity and mortality, and rates of recurrence. Lesions included adnexal neoplasm, ameloblastic fibro-odontoma, basal cell carcinoma, benign fibrous histiocytoma, blue nevus, chondrosarcoma, compound nevus, epithelioma adenoides cysticum, esthesioneuroblastoma, Ewing sarcoma, fibrosarcoma, giant cell granuloma, granulocytic sarcoma, hemangioma, hemangiopericytoma, Langerhans cell histiocytosis, lymphangioma, lymphoma, melanoma, neuroblastoma, neurofibroma, ossifying osteofibroma, osteochondroma, osteosarcoma, port wine stain, rhabdomyosarcoma, Spitz nevus, and xanthogranuloma. To the best of our knowledge, this is the largest such study of its kind to date. We believe that the large size of this study and the data on disease incidence will allow clinicians to be better informed of the differential diagnosis of neoplasms of the nasal cavity and paranasal sinuses in the pediatric population.
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PMID:Differential diagnosis of pediatric tumors of the nasal cavity and paranasal sinuses: a 45-year multi-institutional review. 2108 77

This study aimed to evaluate pediatric cases treated surgically for an initial diagnosis of bone and soft tissue tumor between January 1987 and January 2012. This retrospective study evaluated 328 patients with pathologically confirmed tumor from a total of 374 patients hospitalized with an initial diagnosis of tumor. The cases were analyzed with respect to frequency, age, gender, and localization. One hundred and eighty (54.8%) males and 148 (45.2%) females, with a mean age of 13 years (range: 1-18 years), were included in the study. The tumors were determined as 258 (78.6%) bone tumors and 70 (21.4%) soft tissue tumors. The most common benign bone tumor was observed to be osteochondroma (n=61, 30.7%), and the most common malignant bone tumor was osteosarcoma (n=31, 52.6%). The most common benign soft tissue tumor was hemangioma, which is a vascular tumor (n=28, 43.8%), and the most common malignant soft tissue tumor was rhabdomyosarcoma (n=5, 83.3%). It is thought that similar studies will serve to form larger series and facilitate inter-regional comparisons by collecting data from centers that surgically treat bone and soft tissue tumors, thereby benefitting both pediatric and public health.
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PMID:Distribution of pediatric bone and soft tissue tumors in the central Black Sea region. 2534 92

We conducted a retrospective study to analyze the role of the cervical and lateral skull base approaches in the surgical excision of parapharyngeal space (PPS) tumors. Our study population was made up of 34 patients-15 males and 19 females, aged 13 to 73 years (mean: 50.6)-who had presented to us with a PPS tumor during a 9-year period. The 34 cases included 23 benign tumors and 11 malignancies. The 23 benign tumors consisted of 11 pleomorphic adenomas, 5 schwannomas, 2 paragangliomas, 2 Warthin tumors, 1 oncocytoma, 1 hamartoma, and 1 osteochondroma. The 11 malignancies included 3 cases of recurrent nasopharyngeal carcinoma, 2 cases of mucoepidermoid carcinomas, and 1 case each of carcinoma ex pleomorphic adenoma, liposarcoma, lymph node metastasis from nasopharyngeal carcinoma, lymph node metastasis from follicular thyroid carcinoma, rhabdomyosarcoma, and cranial nerve (CN) X neurofibrosarcoma. Among the benign tumors, 14 were removed via the transparotid approach, 3 via the transcervical approach, 3 via the transcervical-transparotid approach, 2 via the petro-occipital-trans-sigmoid approach, and 1 via the Fisch type A infratemporal fossa approach. During postoperative follow-up, 5 of the 23 patients with a benign tumor exhibited facial nerve paresis, 2 developed CN IX to XII palsy, and 1 each developed Frey syndrome and CN X palsy. In the malignant tumor group, 4 of the 11 patients were treated via the transcervical-transparotid approach, 3 via the Fisch type C infratemporal fossa approach, 3 via the transcervical-transmandibular approach, and 1 via the transcervical-lateral petrosectomy approach. The neural deficits observed during the postoperative period were more extensive among the patients with a malignant tumor. We conclude that the transparotid and transcervical approaches were adequate for excising most benign tumors. For malignant tumors, large tumors, and tumors with skull base involvement and transcranial extension, the transparotid-transcervical, transcervical-transmandibular, infratemporal fossa, and petro-occipital-trans-sigmoid approaches were necessary.
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PMID:Surgical management of parapharyngeal space tumors: The role of cervical and lateral skull base approaches. 2792 99

PD-1 and its ligands have been shown to play a significant role in evasion of malignant tumour cells from the immune system. Last year, the Unites States Food and Drug Administration (FDA) approved anti-PD-1 inhibitors for treatment of non-small cell lung carcinoma and recently expanded the use of immunotherapy for metastatic urothelial cell carcinoma and Hodgkin lymphoma. However, studies on expression of PD-1 and its ligand in malignant bone and soft tissue sarcoma are sparse. In this study, we evaluated PD-1 and PD-L1 expression on variants of liposarcomas and rhabdomyosarcomas, osteosarcomas and chondrosarcomas. Tissue microarrays (TMAs) for liposarcomas (well differentiated, myxoid/round cell, and pleomorphic), rhabdomyosarcomas (alveolar, embryonal, pleomorphic, and spindle cell), conventional osteosarcomas and chondrosarcomas were stained for PD-1 and PD-L1 antibodies. Adipose tissue, skeletal muscle, bone, osteochondroma and lipoma were used as control and benign counterparts. Western blot was performed to evaluate expression of PD-1 and PD-L1 in four sarcoma cell lines. Osteosarcomas, chondrosarcomas, and all variants of liposarcomas and rhabdomyosarcomas over-expressed PD-1 relative to normal tissue. Expression of PD-1 in rhabdomyosarcomas was associated with higher tumour stage. Only one case of pleomorphic liposarcoma, one case of pleomorphic rhabdomyosarcoma and two cases of alveolar rhabdomyosarcomas were positive for PD-L1. Normal adipose tissue, skeletal muscle, and bone were negative for both PD-1 and PD-L1 and lipomas and osteochondroma weakly expressed PD-1 but not PD-L1. Western blot confirmed the presence of PD-1 protein in all four sarcoma cell lines. Overall, our results showed cytoplasmic expression of PD-1 in the bone and soft tissue sarcomas, while PD-L1 was negative. Whether these data are an indication for effectiveness of immunotherapy in the management of malignant bone and soft tissue sarcomas remains to be elucidated.
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PMID:PD-1 and PD-L1 expression in bone and soft tissue sarcomas. 2868 24

Osteochondroma is the most common benign bone tumor. Approximately 15% of osteochondromas occur as multiple lesions. Multiple osteochondromatosis has a higher risk of developing chondrosarcomas, which are of low grade with good prognosis. About 10% of all chondrosarcomas may undergo dedifferentiated change, which has a poorer prognosis. Dedifferentiated peripheral chondrosarcoma developing within an osteochondroma is extremely rare. Dedifferentiation usually occurs in the form of osteosarcoma, malignant fibrous histiocytoma, fibrosarcoma, or rhabdomyosarcoma. We report a case of proximal epithelioid sarcomatous dedifferentiation in secondary chondrosarcoma in a 39-year-old male with multiple osteochondromatosis in bilateral arm. To the best of our knowledge, epithelioid sarcomatous dedifferentiation has not been described in the literature.
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PMID:Proximal epithelioid sarcomatous dedifferentiation in secondary chondrosarcoma in a known case of multiple osteochondromatosis. 2870 94