title: [Mediastinal germ-cell tumor associated with AML (M7)--the syndrome of mediastinal germ-cell tumors associated with hematologic neoplasia?].
abstract: In October 1992, a 36-year-old man was diagnosed as having mediastinum mixed germ cell tumor (stage II), and was treated with surgical operation and combination chemotherapy including VP16 (total VP16 dose; 1,500 mg/m2). After that, remission had been sustained, but leukocytosis (15,700/microliter) with 37% of peroxidase-negative blasts and thrombocytopenia developed in September, 1995. Bone marrow showed remarkable reticulin fibrosis and increase of atypical immature cells that were immunophenotypically factor VIII+/CD42+/CD61+. Thus, we diagnosed acute megakaryoblastic leukemia (M7). Based on no abnormality of chromosome 11q23 and no rearrangements of MLL gene, we diagnosed the syndrome of mediastinal germ-cell tumors associated with hematologic neoplasia. Furthermore, the neuron-specific enolase level was elevated (95.9 ng/ml). Soon after complete remission was reached by combination chemotherapy, the leukemia was relapsed, and the he died 3 months after the onset of leukemia. To our knowledge, this is the third case report of this syndrome in Japan and the first one of leukemia with high level of serum neuron-specific enolase.
authors.affiliation: Division of Hematology, Kanto Teishin Hospital.
authors.forename: K
authors.initials: K
authors.lastname: Kitazume
authors.forename: K
authors.initials: K
authors.lastname: Usuki
authors.forename: M
authors.initials: M
authors.lastname: Endo
authors.forename: M
authors.initials: M
authors.lastname: Osawa
authors.forename: S
authors.initials: S
authors.lastname: Iki
authors.forename: S
authors.initials: S
authors.lastname: Chiba
authors.forename: H
authors.initials: H
authors.lastname: Hirai
authors.forename: S
authors.initials: S
authors.lastname: Matsuya
authors.forename: A
authors.initials: A
authors.lastname: Urabe
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000097:blood; D003131:Combined Modality Therapy; D018237:Germinoma / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D007947:Leukemia, Megakaryoblastic, Acute / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D008479:Mediastinal Neoplasms / Q000175:diagnosis* / Q000628:therapy; D009364:Neoplasm Recurrence, Local; D010751:Phosphopyruvate Hydratase / Q000097:blood; D012074:Remission Induction; D013577:Syndrome
chemical_list: D014408:Biomarkers, Tumor; D010751:Phosphopyruvate Hydratase