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)

From 1982 to 1987, 40 children with non-metastatic thoracic neuroblastoma were treated with the same therapeutic regimen. According to TNM staging, there were 11 CS I, 19 CS II, and 10 CS III. All patients underwent surgery; 30 had primary surgical excision; in 10 whose tumors were deemed unresectable, surgery was delayed until after a trial of chemotherapy. Operation was completed by several courses of chemotherapy in case of microscopic residual disease or regional lymph node involvement; radiotherapy was delivered in case of gross residual disease. Using this therapeutic approach, EFS is 92% with a median follow-up of 40 months. Severe complications were rare and sequellae appear to be related to the disease, i.e., neurologic consequences of cord compression.
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PMID:Nonmetastatic thoracic neuroblastomas: a review of 40 cases. 205 69

From 1982 to 1987, forty children with non-metastatic thoracic neuroblastoma were treated with a same therapeutic regimen. According to TNM staging, there were II CS I, 19 CS II and 10 CS III. All patients underwent surgery; thirty had primary surgical excision; in ten whose tumor were deemed uresectable, surgery was delayed until after a trial of chemotherapy. Operation was completed by several courses of chemotherapy in case of microscomic residual disease or lymph node involvement; radiotherapy was delivered in case of gross residual disease. Using this therapeutic approach. Event Free Survival is 92% with a median follow up of 40 months. Severe complications were rare and sequellae appear to be related to the disease i.e. neurologic consequence of cord compression.
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PMID:[Localized thoracic neuroblastoma: the role of surgery and therapeutic results. Apropos of 40 cases]. 208 61

In the three most widely used neuroblastoma staging systems--Evans, Pediatric Oncology Group (POG), and TNM-Union Internationale Contre le Cancer (UICC)--few staging problems are presented by the completely resected tumor or with the patient who has metastatic disease. Difficulties arise with the localized tumor when there is extension across the midline and with the demonstration of lymph node involvement. A new International Staging System for Neuroblastoma (INSS) is proposed that incorporates both lymph node invasion and extension across the midline into surgical staging. Clear criteria for diagnosis are established and clinical responses are defined. New prognostic factors may be added. The surgical role remains significant in staging, diagnosis, and therapy. Use of a common system throughout the world would facilitate the evaluation of clinical trials and help achieve the goal of developing optimum therapy for the biologically different types of neuroblastoma.
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PMID:A surgical perspective on the current staging in neuroblastoma--the International Neuroblastoma Staging System proposal. 273 83

The role of surgery for children with neuroblastoma was evaluated by using a recently proposed TNM staging system. One-hundred thirty patients were retrospectively assigned a TNM clinical stage (CS) preoperatively and a pathologic stage (PS) postoperatively. Patients with CS 4 were separated into CS 4A and CS 4B according to their age and pattern of metastases. Patient survival was analyzed according to CS, age, location of primary, and PS. Actuarial survival of patients was as follows: CS 1, 100%; CS 2, 82%; CS 3, 63%; CS 4A, 50%; and CS 4B, 5%. For all stages, patients younger than 1 year old survived longer than those older than 1 year (72% v 32%). Prognosis for CS 1 was the same regardless of age. For CS 2 and CS 3, patients younger than 1 year old lived longer. CS 4A had better survival than CS 4B. Survival by site was 100% for cervical, 62% for mediastinal, 45% for pelvic, and 36% for retroperitoneal primaries. The role of surgery was evaluated by analyzing survival according to the postoperative PS. PS 1-2-3 A were regarded as satisfactory resections since all macroscopic tumor was removed. PS 3B as a debulking procedure, and PS 3C as an unresectable lesion which was biopsied. Patients with nonmetastatic disease (CS 1-3) with PS 1 and PS 2 disease had a 100% survival rate; PS 3A, 93%; PS 3B, 58%; and PS 3C, 21%. This proves the value of total resection in nonmetastatic disease. The role of surgery could also be proven in metastatic disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Evaluation of the role of surgery in 130 patients with neuroblastoma. 400 75

From 1975 to 1979, 173 children with neuroblastoma were treated according to the same protocol at the Institut Gustave-Roussy. They were classified according to the site of the primary tumor (abdominal: 122; thoracic: 29; others: 22) and according to TNM staging (stage I: 8; stage II: 24; stage III: 35; stage IV: 99; stage V: 2). Depending on stage and age, treatment consisted of surgery and radiotherapy associated with cyclic multiagent chemotherapy (vincristine, Adriamycin, cyclophosphamide). It resulted in a significant improvement of prognosis in stage III patients, especially those with abdominal tumors. In the latter group, prognosis depended mainly on the possibilities of resection of the tumors. Therefore, making these tumors operable remains the major goal of therapy in such patients. Radiotherapy is quite efficient in sterilizing the small post-surgical residual tumors. Prognosis in children over 1 year of age with metastases still remains very poor, even though the quality of the survival is improved.
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PMID:[Neuroblastomas treated at the Gustave-Roussy Institute from 1975 to 1979. 173 cases]. 686 66

The TNM classifications of neuroblastoma, nephroblastoma and soft tissue sarcoma were adopted at the International Conference for TNM Classification (UICC) held in May 1980. There is no TNM system under contemplation, however, for primary liver carcinoma in childhood. Accordingly, we have formulated the proposed Japanese TNM system for this carcinoma in children and examined its validity in 136 cases of hepatoblastoma seen in the listed 14 institutions. The basic policy of the Committee on the Japanese TNM Classification is not to include the resectability of the tumor and regional lymph nodes or any other status of the disease resulting from therapeutic intervention as a component of the pTNM system. This is a feature which makes our proposed system widely divergent from the accepted classification scheme for the three types of tumor cited above.
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PMID:The proposed Japanese TNM classification of primary liver carcinoma in infants and children. 688 56

The clinical and postsurgical TNM classifications (cTNM and pTNM) for neuroblastoma (NB), nephroblastoma (WT) and soft tissue sarcomas were presented in 1982 by the TNM Committee in UICC in collaboration with SIOP. The Japanese TNM Committee proposed new pTNM systems (J-pTNM) for NB and WT, and new cTNM and pTNM system for primary liver carcinoma in infants and children (HT). These pTNMs were based on the staging systems developed by the Malignant Tumor Committee of the Japanese Society of Pediatric Surgeons. The proposal of subdivision of M category in NB was presented for testing the new telescopic ramifications of TNM. The TNM for HT was added as a new classification recommended for testing. The effectiveness of these TNM systems was assessed using NB, WT and hepatoblastoma (HB) cases which were registered in collaborating institutes. The analyses suggested that pTNM, especially the J-pTNM system in NB, WT and HT were effective for the assessment of prognoses, although cTNM systems were not enough to assess the extent of the disease.
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PMID:[TNM classification--pediatric tumors]. 949 41

Primary sinonasal tract mucosal malignant melanomas are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. A total of 115 cases of sinonasal tract mucosal malignant melanoma included 59 females and 56 males, 13-93 years of age (mean 64.3 years). Patients presented most frequently with epistaxis (n = 52), mass (n = 42), and/or nasal obstruction (n = 34) present for a mean of 8.2 months. The majority of tumors involved the nasal cavity (n = 34), septum alone, or a combination of the nasal cavity and sinuses (n = 39) with a mean size of 2.4 cm. Histologically, the tumors were composed of a variety of cell types (epithelioid, spindled, undifferentiated), frequently arranged in a peritheliomatous distribution (n = 39). Immunohistochemical studies confirmed the diagnosis of sinonasal tract mucosal malignant melanomas with positive reactions for S-100 protein, tyrosinase, HMB-45, melan A, and microphthalmia transcription factor. Sinonasal tract mucosal malignant melanomas need to be considered in the differential diagnosis of most sinonasal malignancies, particularly carcinoma, lymphoma, sarcoma, and olfactory neuroblastoma. Surgery accompanied by radiation and/or chemotherapy was generally used. The majority of patients developed a recurrence (n = 79), with 75 patients dying with disseminated disease (mean 2.3 years), whereas 40 patients are either alive or had died of unrelated causes (mean 13.9 years). A TNM-type classification separated by anatomic site of involvement and metastatic disease is proposed to predict biologic behavior.
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PMID:Sinonasal tract and nasopharyngeal melanomas: a clinicopathologic study of 115 cases with a proposed staging system. 1271 45

Survivin is a member of the family of proteins, which inhibit apoptosis (inhibitor of apoptosis proteins - IAP). Expression of survivin was found in colorectal cancer, neuroblastoma, bladder cancer, non-small cell lung cancer, and breast cancer. There is some recent data indicating the correlation of poor prognosis and worse response to chemotherapy in patients with oesophageal squamous cell carcinoma (OSCC) expressing survivin. The aim of the present study was to assess survivin expression in cancerous tissue of patients with advanced OSCC and to test the potential correlation between survivin expression and clinicopathological data. Forty two patients (mean age 58.36+/-8.97 yrs), who were oesophagectomised due to squamous cell carcinoma of the thoracic oesophagus between 1998 and 2000, were retrospectively analysed. Cytoplasmic survivin expression, examined immunohistochemically, was found in 35 (83.33%) cases. No statistically significant correlation between survivin expression in the tumour and patients' gender, TNM stage, or vascular involvement was noted. The mean survival of patients with cytoplasmic survivin expression (17.81+/-5.51 months) was not statistically different to those with negative survivin staining (16+/-6.28 months) as assessed by Mantel-Cox test (p=0.49). Univariate regression analysis revealed UICC staging as the only predictor of survival in the analysed group (p<0.05). These results indicate that the cytoplasmic survivin expression does not seem to be the prognostic factor in advanced cases of OSCC.
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PMID:Assessment of prognostic significance of cytoplasmic survivin expression in advanced oesophageal cancer. 1549 78

Clinical outcomes for high-risk neuroblastoma patients remains poor, with only 40-50% 5-Year overall survival (OS) and <10% long-term survival. The ongoing acquisition of genetic/molecular rearrangements in undifferentiated neural crest cells may endorse neuroblastoma progression. This study recognized the loss of Retinal Degeneration protein 3, RD3 in aggressive neuroblastoma, and identified its influence in better clinical outcomes and defined its novel metastasis suppressor function. The results showed ubiquitous expression of RD3 in healthy tissues, complete-loss and significant TNM-stage association of RD3 in clinical samples. RD3-loss was intrinsically associated with reduced OS, abridged relapse-free survival, aggressive stage etc., in neuroblastoma patient cohorts. RD3 was transcriptionally and translationally regulated in metastatic site-derived aggressive (MSDAC) cells (regardless of CSC status) ex vivo and in tumor manifolds from metastatic sites in reproducible aggressive disease models in vivo. Re-expressing RD3 in MSDACs reverted their metastatic potential both in vitro and in vivo. Conversely muting RD3 in neuroblastoma cells not only heightened invasion/migration but also dictated aggressive disease with metastasis. These results demonstrate the loss of RD3 in high-risk neuroblastoma, its novel, thus-far unrecognized metastasis suppressor function and further imply that RD3-loss may directly relate to tumor aggressiveness and poor clinical outcomes.
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PMID:RD3 loss dictates high-risk aggressive neuroblastoma and poor clinical outcomes. 2637 49


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