Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0079731 (B-cell lymphoma)
 16,671 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ifosfamide/mesna was given to 97 patients who had malignant solid tumors diagnosed before they were 21 years of age. Patients received 1.6 g/m2 ifosfamide daily x 5, given i.v. over 15 min, followed by 400 mg/m2 i.v. mesna at 15 min and 4 and 6 h after ifosfamide. Responses were noted in patients with osteosarcoma, Ewing's sarcoma, rhabdomyosarcoma and other soft-tissue sarcomas, rhabdoid tumor, neuroblastoma, Wilms' tumor, primitive neuroectodermal tumor, retinoblastoma, germ-cell tumors, and B-cell lymphoma. Toxicity included mild to moderate nausea and vomiting, transient, reversible myelosuppression, transient elevations of serum blood urea nitrogen (BUN) and creatinine and liver enzymes, infections, and self-limiting neurotoxicity characterized by changes in mental status, motor dysfunction, cranial nerve palsy, cerebellar dysfunction, and seizures. Neurotoxic symptoms were generally seen in patients who had previously received cisplatin. Ifosfamide is an important alkylating agent that should be combined with other agents in phase II and III trials. Alternate dose schedules should also be investigated.
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PMID:Ifosfamide in pediatric malignant solid tumors. 250 57

Ifosfamide was given to 61 patients with malignant solid tumors diagnosed before the age of 21 years. In this phase II study, all patients received 1.6 g/m2/day X 5 iv over 15 minutes followed by mesna at a dose of 400 mg/m2 iv at 15 minutes and 4 and 6 hours after ifosfamide. Responses were observed in five of 15 patients with osteosarcoma, two of ten with neuroblastoma, two of six with Wilms' tumor, two of five with rhabdomyosarcoma, four of eight with other soft tissue sarcomas, one of one with retinoblastoma, one of two with germ cell tumors, one of one with B-cell lymphoma, and one of one with a primitive neuroectodermal tumor. Fifty-nine of 61 patients had received prior alkylating agent therapy which included cyclophosphamide, cisplatin, mechlorethamine, melphalan, or dacarbazine. Fourteen of 19 responses developed in patients whose tumors were resistant to treatment with cyclophosphamide. A patient with malignant Schwannoma who had received no prior chemotherapy developed a complete response which lasted 12 months. A patient with brain metastases of osteosarcoma has had complete response for greater than 2 years. Complete response was also observed in a patient with B-cell lymphoma. Toxicity consisted of mild to moderate nausea and vomiting, transient reversible myelosuppression, occasional elevation of serum BUN or creatinine, and transient neurotoxicity characterized by somnolence, confusion, weakness, tremor, hallucinations, or seizures. We conclude that ifosfamide is an important alkylating agent without apparent complete cross-resistance with cyclophosphamide, and as such should be further investigated for determination of its activity in patients with pediatric neoplasms and considered for incorporation into phase II-III trials for certain tumors.
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PMID:Phase II trial of ifosfamide in children with malignant solid tumors. 310 34

Expression of the RCK gene, which is a target gene on 11q23 of the t(11;14) (q23;q32) translocation in the B-cell lymphoma cell line RC-K8, was studied by Northern and Western blot analyses. The RCK gene product is a member of the D-E-A-D box protein/RNA helicase family. With the use of Northern blot analysis, a 7.5-kb transcript of the RCK gene was shown to be expressed ubiquitously in human and mouse tissues. Polyclonal antibodies against the RCK gene product were raised, and the RCK gene expression pattern was examined in human and mouse tissues. Two different polyclonal anti-rck antibodies detected a specific 54-kilodalton product named rck/p54 in the majority of human and mouse tissues tested by Western blot analysis. However, rck/p54 was shown to be very low in the human brain and was not detectable in lumbar muscle and lung tissues, although RCK mRNA is abundantly present in these tissues. It is of interest that malignant transformed human cells arising from tissues with low or no expression of rck/p54, such as neuroblastoma, glioblastoma, rhabdomyosarcoma, and lung cancer cell lines, produced a moderate amount of rck/p54 protein, suggesting that rck/p54 plays a role in tumorigenesis. In addition, the rck/p54 protein was localized to cytoplasm by immunostaining with the use of laser microscopy and by subcellular fractionation.
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PMID:The rck/p54 candidate proto-oncogene product is a 54-kilodalton D-E-A-D box protein differentially expressed in human and mouse tissues. 761 84

Autologous bone marrow transplantation is used for children with cancers which are only partially responsive to bone marrow toxic doses of cancer chemotherapy and irradiation. The use of megatherapy and autologous bone marrow transplantation instead of conventional doses for children has yielded substantial benefits in B-cell lymphoma, relapse of leukaemia, disseminated neuroblastoma and germ cell tumors. In the case of Wilms' tumor, rhabdomyosarcoma, osteosarcoma and Ewing's sarcoma with a partial initial response or a relapse, autologous bone marrow transplantation has been used to a limited extent. Based on the best results from conventional therapy and autologous bone marrow transplantation, respectively, the present need for autologous bone marrow transplantation in Denmark is estimated to be 24 transplantations a year.
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PMID:[Autologous bone marrow transplantation in children]. 831 84

Primary heart tumors are uncommon in children. The majority of them are benign, with only 10% malignant. Among malignant cardiac tumors, sarcoma (rhabdomyosarcoma, angiosarcoma, synovial sarcoma) and lymphoma (Burkitt's lymphoma, large B-cell lymphoma, lymphoblastic lymphoma) predominate. There are few published pediatric series on malignant primary cardiac tumors. We report here 3 observations of primary malignant cardiac tumors, 2 cases of sarcoma (angiosarcoma and synovial sarcoma) and 1 case of Burkitt's lymphoma. A precise pathological diagnosis is necessary for the proper management of these patients. For sarcoma, treatment associates surgery and chemotherapy. Surgery should be as complete as possible because of the lack of chemotherapy sensitivity of some sarcomas, mainly angiosarcoma and synovial sarcoma. Therefore, the prognosis of cardiac sarcoma remains poor. For primary cardiac lymphoma, management should not be different from lymphoma in other locations. Chemotherapy is the main treatment, and surgery has to be used only when complications occur. Prognosis depends on histology and not lymphoma location, and so is better than the prognosis for sarcoma.
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PMID:[Malignant primary cardiac tumors in childhood and adolescence]. 2033 33

Childhood head and neck cancers are relatively uncommon. Of all head and neck cancers occurring in children, non-Hodgkin's lymphoma (NHL) is the most common, others being rhabdomyosarcoma and nasopharyngeal carcinoma. In the head and neck region, sinuses are the second commonest primary site of NHL after neck lymph nodes. These can be of several different types depending on the predominant cell type and histologic appearance, the most common histological variant being diffuse large B-cell lymphoma. In an attempt to simplify the classification and to develop a universally acceptable classification and staging, they have been classified and staged numerous times over the last three decades, adding more confusion to the topic. Clinical presentations vary according to the histological type. The low grade lymphomas present with a nasal cavity or para-nasal sinus mass associated with obstructive symptoms and/or lymphadenopathy, while high grade lymphomas present with aggressive signs and symptoms including non-healing ulcer, epistaxis, septal perforation and bony destruction. The primary treatment consists of chemotherapy and / or radiation therapy, which is able to achieve remission in two-third of the patients, however, prognosis remains poor with cumulative five-year survival rates at about 30% for all the types of sino-nasal NHLs. Newer targeted therapy (monoclonal antibodies) and combination therapies (including stem cells) are currently being tested in order to improve survival rates in these patients. This article aims at providing an overview of clinico-epidemiologic characteristics, staging system currently in use, management, prognosis and possibilities of future research in the field of childhood sinonasal NHLs.
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PMID:Non-Hodgkin's lymphoma of the sino-nasal tract in children. 2047 39

In recent years, the synchronous occurrence of tumors of different histotypes arising in the same organ has been reported more frequently in the literature. In the stomach, adenocarcinoma has been described with coexisting primary rhabdomyosarcoma, carcinoid, and low-grade B-cell lymphoma of mucosa-associated lymphoid tissue. The simultaneous development of adenocarcinoma and gastric mesenchymal tumor has been documented rarely. We report one such case. A 65-year-old male was diagnosed with a proximal gastric adenocarcinoma and underwent subtotal gastrectomy. Subsequent histopathological examination revealed the presence of another tumor at the gastric antrum. This was a gastrointestinal stromal tumor of low risk category (GIST). The literature has only a few previous reports of this very rare association. It is not known whether this synchronicity is incidental or there is a causative factor inducing the development of tumors of different histotypes in the same organ. Pathologists, oncologists and surgeons should be aware of this interesting condition.
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PMID:Synchronous adenocarcinoma and gastrointestinal stromal tumor in the stomach. 2061 20

Tumours of the testicular adnexa include a heterogeous group of mesothelial, mesenchymal and germ cell tumours. Adenomatoid tumour, pseudosarcomatous myofibroblastic proliferations and rhabdomyosarcoma are the more frequently encountered neoplasms. Lymphoma/leukemic infiltration secondary to testicular involvement or primary tumour elsewhere is not unusual. However, Primary Para-Testicular Lymphoma (PPTL) involving spermatic cord and/or epididymis with sparing of the testicular parenchyma is extremely rare. Accurate staging and typing is crucial for effective management. We present a rare case of Diffuse Large B Cell Lymphoma (DLBCL) involving the left paratesticular tissue with testicular sparing in a young immunocompetant male patient.
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PMID:Primary Paratesticular Lymphoma with Testicular Sparing: Account of an Unusual Scrotal Mass. 2713 82

Head and neck pathology present a unique set of challenges including the morphological diversity of the neoplasms and presentation of metastases of unknown primary origin. The detection of human papillomavirus and Epstein-Barr virus associated with squamous cell carcinoma and newer entities like HPV-related carcinoma with adenoid cystic like features have critical prognostic and management implications. In salivary gland neoplasms, differential diagnoses can be broad and include non-neoplastic conditions as well as benign and malignant neoplasms. The detection of specific gene rearrangements can be immensely helpful in reaching the diagnosis in pleomorphic adenoma, mucoepidermoid carcinoma, secretory carcinoma, hyalinizing clear cell carcinoma and adenoid cystic carcinoma. Furthermore, molecular techniques are essential in diagnosis of small round blue cell neoplasms and spindle cell neoplasms including Ewing sarcoma, rhabdomyosarcoma, synovial sarcoma, biphenotypic sinonasal sarcoma, dermatofibrosarcoma protuberans, nodular fasciitis and inflammatory myofibroblastic tumor. The detection of genetic rearrangements is also important in lymphomas particularly in identifying 'double-hit' and 'triple-hit' lymphomas in diffuse large B cell lymphoma. This article reviews the use of in situ hybridization in the diagnosis of these neoplasms.
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PMID:Clinical Utility of In Situ Hybridization Assays in Head and Neck Neoplasms. 3046 69