Hayashi Y, Kikuchi F, Oka T, Itoyama S, Mohri N, Usuki K, Takaku F, Murakami T, Saitoh Y, Urano Y
Department of Pathology, Faculty of Medicine, Tokyo University, Japan.
Acta Pathol Jpn. 1988 Jun;38(6):789-98. doi: 10.1111/j.1440-1827.1988.tb02350.x.
Rhabdomyosarcoma manifested as a systemic disease is very rare and cases showing diffuse metastasis in the bone marrow are most unusual. Recently we encountered two cases of rhabdomyosarcoma with diffuse bone marrow metastasis which were clinically manifested as acute leukemia. The first patient was a 15-year-old female, who was admitted in 1982 with pancytopenia and many large primitive cells in bone marrow aspirates, hematological malignancy being diagnosed. Thereafter the bilateral breasts showed rapid swelling and a biopsy specimen revealed the histological features of typical alveolar rhabdomyosarcoma. The primary site of the neoplasm remained undetermined during the course. At autopsy, it was disclosed that the neoplasm originated from the left thigh and showed generalized metastasis. The second patient was a 38-year-old man, who was admitted in 1986 because of a nasal polyp obstructing the nasal cavity, and persistent nasal bleeding. Peripheral blood samples showed leukoerythroblastosis and thrombocytopenia, and large primitive cells were found in bone marrow aspirates, so that hematological malignancy was initially diagnosed. A biopsy specimen of the nasal polyp showed proliferation of large round cells and electron microscopy demonstrated the ultrastructural features of rhabdomyosarcoma.
表现为全身性疾病的横纹肌肉瘤非常罕见，而骨髓出现弥漫性转移的病例则极为不寻常。最近我们遇到了两例横纹肌肉瘤伴骨髓弥漫性转移的病例，临床表现为急性白血病。首例患者为一名15岁女性，1982年因全血细胞减少及骨髓穿刺液中出现许多大的原始细胞入院，诊断为血液系统恶性肿瘤。此后双侧乳房迅速肿胀，活检标本显示为典型的肺泡状横纹肌肉瘤的组织学特征。肿瘤的原发部位在病程中一直未明确。尸检发现肿瘤起源于左大腿并已发生全身转移。第二例患者为一名38岁男性，1986年因鼻腔息肉阻塞鼻腔及持续性鼻出血入院。外周血样本显示有幼粒-幼红细胞血象和血小板减少，骨髓穿刺液中发现大的原始细胞，因此最初诊断为血液系统恶性肿瘤。鼻腔息肉的活检标本显示大圆形细胞增生，电子显微镜检查显示为横纹肌肉瘤的超微结构特征。
