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2. Böhm P, Kunz W, Horny HP, Einsele H: Adult Gaucher disease in association with primary malignant bone tumors. Cancer; 2001 Feb 1;91(3):457-62
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adult Gaucher disease in association with primary malignant bone tumors.
  • BACKGROUND: Malignant neoplastic disorders are more common in patients with Gaucher disease (GD) than in the general population.
  • Very few cases of primary malignant bone tumors in association with GD have been reported to date.
  • Thus, the recommendations for an adequate therapy are often based on limited professional experience.
  • METHODS: The clinical, radiologic, and histologic data of the authors' two patients who had GD and primary malignant bone tumor are presented.
  • Epidemiologic data, clinical data, and treatment results from published reports of 18 patients who had GD and various malignancies and the authors' 2 patients were compared and evaluated.
  • In both cases, the bone marrow architecture was partially effaced by sheets of large histiocytic cells with striated or fibrillary cytoplasm.
  • In both patients, chemotherapy was performed.
  • Whereas the patient who had the leiomyosarcoma showed poor recovery of the bone marrow that necessitated withdrawal of aggressive chemotherapy, the patient who had non-Hodgkin lymphoma and enzyme therapy tolerated the chemotherapy well.
  • In spite of local control after preoperative radiotherapy and hemipelvectomy, the first patient developed lung metastases and finally died.
  • The second patient was continuously free of disease at a follow-up examination 32 months after chemotherapy and radiotherapy.
  • In a total of 20 patients who had malignant disorders and GD, the numbers of males and females were equivalent, and the mean age was 55 years.
  • Approximately 33% presented with a cancer originating in the bone.
  • Of these, after a mean follow-up of 36 months (range, few days-108 mos), only 2 patients were continuously free of disease, and one patient was alive with disease.
  • The other patients had died of disease or hemorrhagic complications of GD.
  • CONCLUSIONS: Because there is a relatively high incidence of malignant disorders of the bone, the study suggested that malignant disorders have to be included in the differential diagnosis of painful lytic lesions in patients who have GD.
  • Evaluation of the expense and value of enzyme therapy to patients who have GD should be undertaken with regard to the incidence of malignant disorders and patient survival.
  • [MeSH-major] Bone Neoplasms / complications. Gaucher Disease / complications
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bleomycin / therapeutic use. Cyclophosphamide / therapeutic use. Doxorubicin / therapeutic use. Etoposide / therapeutic use. Female. Humans. Ifosfamide / therapeutic use. Male. Prednisone / therapeutic use. Vincristine / therapeutic use

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  • Hazardous Substances Data Bank. BLEOMYCIN .
  • Hazardous Substances Data Bank. DOXORUBICIN .
  • Hazardous Substances Data Bank. ETOPOSIDE .
  • Hazardous Substances Data Bank. IFOSFAMIDE .
  • Hazardous Substances Data Bank. CYCLOPHOSPHAMIDE .
  • Hazardous Substances Data Bank. PREDNISONE .
  • Hazardous Substances Data Bank. VINCRISTINE .
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  • [Copyright] Copyright 2001 American Cancer Society.
  • (PMID = 11169926.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 11056-06-7 / Bleomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; UM20QQM95Y / Ifosfamide; VB0R961HZT / Prednisone; VACOP-B protocol
  • [Number-of-references] 22
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3. Fluri S, Gebbers JO: [Langerhans cell histiocytoses: 50 years to histiocytosis X]. Praxis (Bern 1994); 2004 Mar 31;93(14):559-65
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Histiocytoses encompass a heterogeneous group of histiocytic disorders that may be separated in a group of variable biological behaviour and in a clearly malignant group.
  • The histopathologic diagnosis of all types of histiocytoses is always mandatory.
  • It has a wide range of manifestations: self-healing cutaneous, benign solitary and malignant systemic manifestations require the staging in order to evaluate a possibly necessary therapy.
  • Chemotherapy should be performed within international protocols.
  • [MeSH-minor] Anti-Bacterial Agents / therapeutic use. Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Histocytochemistry. Humans. Immunosuppressive Agents / therapeutic use. Microscopy, Electron. Prognosis. Radiotherapy Dosage

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  • (PMID = 15125070.001).
  • [ISSN] 1661-8157
  • [Journal-full-title] Praxis
  • [ISO-abbreviation] Praxis (Bern 1994)
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Anti-Bacterial Agents; 0 / Antineoplastic Agents; 0 / Immunosuppressive Agents
  • [Number-of-references] 0
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