Fresco Rodrigo, Saldombide Lyber, Suárez Liliana
Servicio de Oncología Clínica, Hospital de Clínicas, Facultad de Medicina, Montevideo, Uruguay.
Tumori. 2003 May-Jun;89(3):324-7. doi: 10.1177/030089160308900318.
Askin tumor, or malignant small round cell tumor of the thoracopulmonary region, is an extremely infrequent entity occurring primarily in children and adolescents. Its histopathologic and cytogenetic features suggest that it belongs to the family of Ewing's sarcoma and primitive neuroectodermal tumors. We report the case of a 43-year-old woman affected by an Askin tumor with bone metastases at diagnosis, presenting synchronously with a plasmacytoma. This is the first reported case of the simultaneous occurrence of an Askin tumor and a malignant hemopathy. The progression of the former and the remission of the plasmacytoma during chemotherapy were remarkable, since Askin tumor treatment shares drugs used for the treatment of plasma cell tumors. Given the infrequent presentation of these diseases in a young adult and the coexistence of two neoplasias characterized by typical chromosomal abnormalities, we consider the possibility of a genetic cancer susceptibility in our patient.
Askin瘤，即胸肺区域恶性小圆细胞瘤，是一种极为罕见的肿瘤，主要发生于儿童和青少年。其组织病理学和细胞遗传学特征表明它属于尤因肉瘤和原始神经外胚层肿瘤家族。我们报告了一例43岁女性患者，诊断时患有Askin瘤并伴有骨转移，同时还患有浆细胞瘤。这是首例报道的Askin瘤与恶性血液病同时发生的病例。由于Askin瘤的治疗与用于治疗浆细胞肿瘤的药物相同，化疗期间前者的进展和浆细胞瘤的缓解非常显著。鉴于这些疾病在年轻成年人中罕见的表现以及两种具有典型染色体异常的肿瘤并存，我们考虑患者存在遗传性癌症易感性的可能性。
