Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Seven dumbbell tumours were histologically diagnosed as neurilemmoma (4), echinococcus cyst (1), chondrosarcoma (1) or neuroblastoma (1). Radical removal was achieved by thoracotomy and enlargement of the intervertebral foramen in two cases. Thoracotomy and laminectomy were performed on separate occasions in three cases. One patient, in whom the extent of the pathologic process was known preoperatively, underwent concomitant 'minithoracotomy' and laminectomy. Postoperative bleeding required reoperation in one case. The patient with chrondrosarcoma died of local recurrence 16 months postoperatively, and the patient with neuroblastoma died of metastatic spread after 6 years. The five survivors are well. Preoperative recognition of intraspinal extension should be obtainable if a high index of suspicion is maintained in cases of paravertebral tumour. Computed tomography is the diagnostic method of choice. Single-stage removal of the intrathoracic and intraspinal portions of the tumour is desirable. The recommended procedure is posterior minithoracotomy with rib resections for exposure and possibly also laminectomy, with the patient in prone position.
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PMID:Management of dumbbell tumours. Reports of seven cases. 235 82

Sinonasal neoplasms and neoplasm-like proliferations composed of light microscopically poorly differentiated or undifferentiated, small- to medium-sized cells cause considerable diagnostic confusion. Lesions in this category include lymphoepithelioma (undifferentiated carcinoma), olfactory neuroblastoma, small-cell undifferentiated (oat cell) carcinoma, sinonasal undifferentiated carcinoma, malignant melanoma, pituitary adenoma, lymphoid hyperplasia, malignant lymphoma, plasmacytoma, lymphomatoid granulomatosis, rhabdomyosarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, Ewing's sarcoma, and synovial sarcoma. Many of these lesions can be definitively diagnosed based on light microscopic features alone, but, in some instances, additional techniques such as immunohistochemistry are of value. The authors review the pertinent clinicopathologic features of the above lesions, with emphasis on light microscopic, immunohistochemical, and ultrastructural features of particular utility in differential diagnosis.
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PMID:"Undifferentiated" neoplasms of the sinonasal region: differential diagnosis based on clinical, light microscopic, immunohistochemical, and ultrastructural features. 269 5

Small, round, blue-cell tumors (SRCT), including rhabdomyosarcoma, Ewing's sarcoma of bone and soft tissue, mesenchymal chondrosarcoma, small cell osteosarcoma, hemangiopericytoma, neuroblastoma, peripheral neurectodermal tumor (peripheral neuroepithelioma of bone and soft tissue), and the malignant small cell tumor of the thoracopulmonary region described by Askin (Askin's tumor), are often difficult to distinguish by light microscopy. We have evaluated the cytogenetics of these tumors by studying 24 tumor explants in short-term culture and 22 tumor cell lines. In Ewing's sarcoma (a tumor of unknown histogenesis), and in peripheral neuroepithelioma and Askin's tumor (tumors with evidence of neural origin), we have observed an indistinguishable t(11;22) translocation.
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PMID:Cytogenetic characterization of selected small round cell tumors of childhood. 300 99

This report evaluates the efficacy of extensive chest wall resection and prosthetic reconstruction in 15 children with chest wall malignancies. There were nine boys and six girls, with a mean age of 9.6 years. Eleven patients had primary chest wall tumors including Ewing's sarcoma (ES), six; rhabdomyosarcoma (RH), two; chondrosarcoma (CS), one; Askin's malignant neuroectodermal tumor, one; and mesenchymal sarcoma, one. Four children had metastases to chest wall and lung from Wilms' tumor (WT), two; osteogenic sarcoma (OS), one; and neuroblastoma (NB), one. Chest wall resection of two to six ribs and reconstruction with Marlex mesh (seven), lattisimus flap (two), prolene mesh (one), and more recently, a Gortex patch (five), was performed. Eight of the patients required concomitant en-bloc pulmonary resection (wedge, five; lobectomy, two; pneumonectomy, one) and two required resection of diaphragm. Fourteen received adjunctive therapy (chemotherapy, 14; irradiation, eight [preoperative, five; postoperative, three]. Six patients had second-look resections after chemotherapy. There was no operative mortality. Early pulmonary function was normal; however, pulmonary restrictive disease and scoliosis occurred with growth. One ES patient developed a radiation-induced second malignant tumor at age 10 and one ES child died at age 6 (no evidence of disease) of meningitis. Average survival length for ES patients was 77 months (range, 18 to 132 months.) Currently, eight patients are alive and five are free of disease. Extensive chest wall resection and reconstruction is useful in the treatment of primary chest wall tumors, but is palliative in metastatic cases. The Gortex patch is the current prosthetic of choice.
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PMID:Chest wall resection and reconstruction for malignant conditions in childhood. 320 68

A multicentre registry of children who had been successfully removed from therapy for some common childhood cancers (Hodgkin's disease, non-Hodgkin's lymphoma, neuroblastoma, nephroblastoma, acute lymphatic leukaemia and other leukaemias) was established in Italy in 1981. The present study describes mortality and occurrence of second primary malignancies (SPMs) among 1467 children who were alive when the registry was established. Follow-up ended on December 31, 1983 for mortality and 1 year later for the occurrence of SPMs. Sixty-seven deaths were recorded, 11 of which were due to causes other than progression of the original disease. Eleven incident SPMs were identified (i.e. 3 acute myeloid leukaemias, 3 thyroid carcinomas, 1 bilateral breast carcinoma, 1 liver malignant mesenchymoma, 1 astrocytoma, 1 chondrosarcoma and 1 osteosarcoma) corresponding to an incidence rate of 2.1/1000 patient-years at risk. Anecdotal reports were collected regarding 2 further SPMs (a thyroid carcinoma and a myeloid leukaemia) as well as several benign tumours, including 2 mammary fibroadenomas.
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PMID:Late deaths and second primary malignancies among long-term survivors of childhood cancer: an Italian multicentre study. 365 74

Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.
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PMID:Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. 631 Feb 27

A 49-year-old man had a malignant soft tissue tumor of the right thigh with metastasis to the femoral region and lower quadrant of the anterior abdominal wall on the right side and the left supraclavicular lymph nodes. The neoplasm showed features of chondrosarcoma and primitive neuroectodermal tumor (combined neuroblastoma, ependymoma, astrocytoma, and oligodendroglioma). The gliomatous part of the mixed tumor was confirmed by identification of the glial fibrillary acidic protein (GFAP). The diverse cellular population suggests a tumor origin from the ectomesenchymal remnant of the neural crest. The mesenchymal component of the neural crest would differentiate into the chondrosarcoma and the neuroectodermal component into the primitive neuroectodermal neoplasm. These various neoplastic elements, then, would form a neoplasm of mixed mesenchymal and neuroepithelial origin or an ectomesenchymoma.
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PMID:Malignant neoplasm of mixed mesenchymal and neuroepithelial origin (ectomesenchymoma) of thigh. 649 17

The value of new morphologic methods in the diagnosis of bone tumors is demonstrated in a number of cases. In round cell malignancies (Ewing's sarcoma, malignant lymphoma, neuroblastoma, and anaplastic plasmacytoma) diagnostic accuracy can be improved by electron microscopic and immunohistochemical techniques. New methods are also of value in differentiating the metastatic carcinoma from malignant bone primaries. Electron microscopy may show epithelial cell features (ie, gland structures, desmosomes, and tonofilaments), while immunohistologic investigation of the cytoskeleton may facilitate differentiation of epithelial cells (positive for prekeratin) from mesenchymal cells (positive for vimentin). In the differential diagnosis of typical bone tumors, however, such as osteosarcoma, chondrosarcoma, and malignant fibrous histiocytoma, the value of enzyme histochemical, electron microscopic, and immunohistochemical methods appears somewhat restricted: alkaline phosphatase activity may be increased in both chondrosarcoma and osteosarcoma; collagen type II, the cartilage-specific collagen, is found not only in chondrosarcoma but in osteosarcoma as well. Moreover, osteosarcomas may contain a considerable number of macrophages and histiocytes, and so this feature is worthless in distinguishing osteosarcoma from malignant fibrous histiocytoma. A new approach for appraising the malignancy of bone tumors may be through flow cytometric investigation of nuclear DNA content. Osteosarcomas reveal DNA aneuploidies in more than 80% of cases, with a large proportion of cells in the S phase. These features may prove valuable for discerning osteosarcoma from myositis ossificans. In contrast to typical giant cell tumor of bone, a rare case of malignant giant cell tumor showed aneuploid cell lines indicating the malignant nature of the tumor.
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PMID:New cytomorphologic methods in the diagnosis of bone tumors: possibilities and limitations. 660 Jan 11

Chondrosarcoma is rare in children. Only 12 patients with a diagnosis of chondrosarcoma were treated at Children's Hospital Medical Center in Boston during the period from 1957 to 1980. Pediatric chondrosarcoma has a relatively rapid onset, manifested by pain, a palpable mass and neurological symptoms with the lesion localized in the spinal column. The pelvis was the most frequent location. Ten patients had primary and two patients had secondary chondrosarcoma. Of the latter two, one developed chondrosarcoma in a preexisting osteochondroma and the other had irradiation for neuroblastoma. The poorest outcomes were in patients who had involvement of the pelvic girdle and spinal column. Grade 2 and 3 lesions had the worst prognoses with metastases occurring within 12 months. Eight patients died at the time of study, and the longest survival time was 80 months. If surgically feasible, the treatment is radical excision. Radiation therapy and chemotherapy should be reserved for recurrences or distant metastasis.
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PMID:Chondrosarcoma in children and adolescents. 708 77

We describe the outcomes and complications of 14 patients with paranasal sinus and anterior cranial fossa lesions surgically treated by an extended transbasal approach, originally described by Kawakami, in our department. They were 10 patients with malignant tumors, 2 with benign tumors, and 2 with inflammatory diseases. A bifrontal craniotomy was performed using a high coronal skin incision, and the orbital rim and roof were removed after the dissection of the dura mater from the anterior skull base. Transcranial resection of the tumor was performed, and assisted by transnasal and transmaxillar resection using a nasal endoscope. Reconstruction of the anterior skull base was performed with the fasica lata and galeopericranial flap in all cases, temporal musculo-pericranial flap in 3 and free bone flap from the cranial convex in 3. Among 10 patients with malignant tumors (malignant melanoma; 4, squamous cell carcinoma; 2 adenocarcinoma; 1, malignant plemorphic adenoma; 1, chondrosarcoma; 1, and neuroblastoma; 1), total removal was performed in 5 patients and subtotal removal in 5 patients. Though local recurrence of the tumor was recognized in 6 patients, only one underwent additional surgery. Eight patients survived, and 2 patients died of systemic metastasis of the tumor and complications due to liquorrhea. Seven patients obtained a good quality of life, and the mean survival period in 8 patients still living was 27 months after the first surgery. In 2 patients with benign tumors (chordoma and osteoma), partial and total removal was performed. The patient with chordoma was operated on several times by this approach and the transoral approach, respectively. Each had a good postoperative course.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Operative results in fourteen cases of paranasal sinus and anterior cranial fossa lesions surgically treated by an extended transbasal approach]. 747 98


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