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

Seven drugs were administered to nude mice with transplanted TNB9 neuroblastoma. DTIC, cyclophosphamide and ifosfamide were the first-line drugs against TNB9. Chemotherapy including ifosfamide was effective to three of six children with malignant solid tumor resistant to cisplatin and/or cyclophosphamide. Two of the patients who were pretreated with radiotherapy were found to have hemorrhagic cystitis after ifosfamide administration. It therefore seems that chemotherapy with ifosfamide should be withheld after radiotherapy.
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PMID:[Ifosfamide treatment for children with malignant tumor]. 368 93

Within 1 year 74 children with neuroblastoma were registered, 30 patients with stage I-III (= 41%) and 44 with stage IV-metastatic disease (= 59%). An aggressive chemotherapy regimen employing Adriamycine, Cyclophosphamide, Vincristine, and Dacarbazine yielded 10/24 partial and 9/24 complete remissions after 9 weeks. 5/24 children were treated less than 9 weeks so far. At the end of the chemotherapy protocol (week 33) 6 recurrences were observed; 3 of these children died. 5 patients remained in complete remission, 1 in partial remission. 12/24 of patients were not evaluable because of treatment less than 33 weeks so far. The one year run of the study is too short to evaluate the benefit of Interferon (randomized trial). The toxicity of the regimen is tolerable, including bone marrow depression, vomiting and hyperpyrexia. Breaking off therapy was only necessary in one patient.
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PMID:[Neuroblastoma study NBL79-society of pediatric oncology -- report after 1 year (author's transl)]. 616 57

A patient with olfactory neuroblastoma who had bone marrow metastasis at the time of diagnosis is presented. Previous therapy for this disease consisted of surgery and radiation. There is limited information relating to the efficacy of chemotherapy. Our patient was treated with combination chemotherapy (dacarbazine [DTIC-Dome], cyclophosphamide [Cytoxan], doxorubicin hydrochloride [Adriamycin], and vincristine sulfate [Oncovin]) and radiation to the primary site. Objective findings, more than two years after diagnosis, support a good partial response. Although a 50%, five-year survival time has been reported, the five-year cure rate is 18%. This report suggests that the role of combination chemotherapy should be further evaluated in certain patients with olfactory neuroblastoma.
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PMID:Olfactory neuroblastoma. Response to combination chemotherapy. 736 26

One hundred and ten patients with advanced neuroblastoma were treated with the protocol of the Study Group of Japan between January 1985 and March 1991. Patients received six cyclic courses of regimen A1, consisting of cyclophosphamide (1,200 mg/m2), vincristine (1.5 mg/m2), tetrahydropyranyl adriamycin (40 mg/m2), and cisplatin (90 mg/m2). Primary tumors and regional lymph node metastases were removed some time during the first six cycles of regimen A1. After six cycles of A1, the patients were divided into three groups. Patients in group 1 received alternating treatment with regimen B (cyclophosphamide and ACNU) and intensified A1, and those in group 2 were treated with alternating administration of regimen C (cyclophosphamide and DTIC) and intensified A1. Patients in group 3 were treated with supralethal therapy and bone marrow transplantation (BMT). Event-free survival rates at five years were 38.8% in the chemotherapy group (groups 1 and 2) and 50.0% in the transplant group (group 3). Because of the study design that was not in truly randomized fashion and because of the small number of patients in each risk group, it is indicated, though not concluded, that the transplant group had a better prognosis than the chemotherapy group in the cases with stage III disease or with amplified N-myc oncogene, based on the statistical calculations. Differences in survival rates for patients who underwent BMT when complete remission (CR) was achieved and for those who achieved CR but who did not undergo marrow transplant were statistically insignificant. BMT-related death occurred in 3 of 31 cases (9.7%) undergoing marrow transplant, and the causes of the death included hemorrhagic pneumonia, myocardial disturbance and hemorrhagic uremia.
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PMID:Treatment combined with bone marrow transplantation for advanced neuroblastoma: an analysis of patients who were pretreated intensively with the protocol of the Study Group of Japan. 783 40

Hematological and clinical data of 14 children with neuroblastoma treated according to the German neuroblastoma therapy study NB 90 were analyzed. Therapy included 4 or 8 intensive therapy elements N1 (Etoposide 125 mg/m2 day 1-4, Vindesine 3 mg/m2 day 1, Cisplatin 40 mg/m2 day 1-4) and N2 (Vincristine 1.5 mg/m2 day 1 + 8, Dacarbazine 200 mg/m2 day 1-5, Ifosfamide 1500 mg/ m2 day 1-5, Doxorubicin 30 mg/m2 day 6 + 7) in alternating order. The hematological recovery was studied after 86 therapy elements N1/N2. G-CSF had been given in 23 therapy courses, while no cytokine was administered in 63 therapy courses. Mobilization of CD34+ cells was studied in 13 therapy courses with G-CSF. Severe myelosuppression with an absolute neutrophil count < 500/microL was noted 2-4 weeks after each therapy element. The use of G-CSF did not prevent, but shortened neutropenia. There was no difference in the number of infections nor time delay of therapy between the courses with or without G-CSF. In 11 therapy courses G-CSF was started on the day following the last chemotherapy dose (N1: day 5; N2: day 9). In 12 therapy courses G-CSF was given delayed, starting day 12 after the initiation of therapy. Kinetics of granulocyte recovery was similar in the early or delayed application of G-CSF. Neutrophil recovery after the therapy element N1 was earlier and faster compared to that of therapy element N2. The more rapid rise of the neutrophils after the N1 element was accompanied by an effective mobilization of CD34+ cells. Taking into account the limitations of this retrospective study, the data may help to optimize the application of G-CSF in a very intensive therapy study like NB90.
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PMID:[Kinetics of myelopoietic regeneration and mobilization of CD34-positive cells within the scope of the NB90 Neuroblastoma Therapy Study]. 934 Apr 28

Neuroblastoma, common in children, rarely develops in adults. We recently treated a patient with adult neuroblastoma. A 34-year-old man complained of a swelling in right inguinal region. CT scan showed swelling of retroperitoneal and inguinal lymph nodes, and bone scintigram by 99mTc-HMDP showed an abnormal uptake in the swollen lymph nodes. Chemotherapy with CDDP (cisplatinum), VP-16 (etoposide), BLM (bleomycin), ADM (adriamycin) was not effective. Histopathological examination of a biopsy specimen revealed neuroblastoma. Another chemotherapy with CPM (cyclophosphamide), VCR (vincristine), ADM, DTIC (dacarbazine), CDDP, VP-16 was effective in decreasing the tumor size. Further high dose chemotherapy with CPM, ADM, CDDP, VP-16 combined with peripheral blood stem cell transplantation led to almost complete disappearance of the tumor and normalization of blood tumor markers (neuron specific enolase and immunosuppressive acidic protein). Retroperitoneal lymph node dissection demonstrated well-differentiated neuroblastoma in the excised tissue. Six months postoperatively, the tumor recurred in the pelvic cavity. Although chemotherapy and radiotherapy were given, he died of the disease 12 months postoperatively.
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PMID:[A case of adult neuroblastoma arising in the retroperitoneal space]. 1159 7

We analyzed 143 cases of skull base surgery collected from the eight institutions of the Study Group supported by the Grant-in-Aid for Cancer Research from the Ministry of Health and Welfare of Japan. Histologically, the most common type was squamous cell carcinoma (n = 78), which was followed by olfactory neuroblastoma (n = 16) and adenoid cystic carcinoma (n = 16). The most frequent surgical approach was frontal craniotomy (n = 66), followed by front-temporal craniotomy (n = 54) and infratemporal fossa approach (n = 8). For repair of dura matter, fascia lata was used in 37 cases. galeopericranial flap in 35 and temporal muscle fascia in 16. The 5-year survival rate by Kaplan-Meier method of nose and paranasal sinus carcinoma (n = 119) was 48%. As for histological classification, the survival rates were both 65%) in adenoid cystic carcinoma (n = 12) and bone soft tissue malignancy (n = 10), 62% in olfactory neuroblastoma (n = 16), 46% in squamous cell carcinoma (n = 62) and 33% in adenocarcinoma (n = 11). All the three cases of malignant melanoma died within 1 year, so we considered skull base surgery to be contraindicated for this disease. Complications were observed in 62 out of the 143 cases (43%); local infection was most frequent in 29 cases. liquorrhea in 18, abscess in 16, necrosis of the flap and meningitis in ten cases, DIC in four, rupture of the internal carotid artery in two and cerebral thrombosis in one. Death caused directly by surgery was in ten cases (7%). It is important that a multi-center registry be maintained to have a large enough database for comparison of results, and prognosis for each histological entity and further define the role of multidisciplinary treatment.
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PMID:Surgical results of skull base surgery for the treatment of head and neck malignancies involving skull base: multi-institutional studies on 143 cases in Japan. 1168 48

The disease course of BRAF (v-raf murine sarcoma viral oncogene homolog B1)-mutant melanoma has been drastically improved by the arrival of targeted therapies. NRAS (neuroblastoma RAS viral oncogene homolog)-mutated melanoma represents 15-25% of all metastatic melanoma patients. It currently does not have an approved targeted therapy. Metastatic patients receive immune-based therapies as first-line treatments, then cytotoxic chemotherapy like carboplatin/paclitaxel (C/P), dacarbazine (DTIC) or temozolomide (TMZ) as a second-line treatment. We will review current preclinical and clinical developments in NRAS-mutated melanoma, and analyze ongoing clinical trials that are evaluating the benefit of different targeted and immune-based therapies, either tested as single agents or in combination, in NRAS-mutant melanoma.
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PMID:Treatment of NRAS-mutated advanced or metastatic melanoma: rationale, current trials and evidence to date. 2871

Abnormal modifications in N-glycosylation processing are commonly associated with neurological disorders, although the impact of specific N-glycans on neuronal excitability is unknown. By replacement of complex types of N-glycans with hybrid types in neuroblastoma cells, we provide the first study that addresses how distinct N-glycan types impact neuronal excitability. Using CRISPR/Cas9 technology, NB_1, a clonal cell line derived from rat neuroblastoma cells (NB), was modified to create an N-glycosylation mutant cell line, NB_1 (-Mgat2), which expresses predominantly hybrid type N-glycans. Western and lectin blotting, flow cytometry, TIRF and DIC microscopy, and patch clamp studies were conducted. Lectin binding revealed the predominant type of N-glycans expressed in NB_1 (-Mgat2) is hybrid while those of NB and NB_1 are complex. Kv3.1 b-expressing cells with complex N-glycans localized more glycosylated Kv3.1b to the neurites than cells with hybrid N-glycans. Further the absence of N-glycan attachment to Kv3.1b was critical for sub-plasma distribution of Kv3.1b to neurites in primary adult mammalian neurons, along with NB cells. Replacement of complex type N-glycans with hybrid type hindered the opening and closing rates of outward ionic currents of Kv3.1 b-expressing NB cells. The lacks of N-glycan attachment hindered the rates even more but were not significantly different between the NB cell lines. Taken together, our evidence supports N-glycosylation impacts the sub-plasma membrane localization and activity of Kv3.1 b-containing channels. We propose that N-glycosylation processing of Kv3.1 b-containing channels contributes to neuronal excitability, and abnormal modifications in N-glycosylation processing of Kv3.1b could contribute to neurological diseases.
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PMID:Membrane Distribution and Activity of a Neuronal Voltage-Gated K+ Channel is Modified by Replacement of Complex Type N-Glycans with Hybrid Type. 3027 98


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