Bourque M D, Di Lorenzo M, Collin P P, Russo P, Laberge J M, Moir C
Department of Surgery, Hôpital Ste-Justine, Montreal, Quebec, Canada.
J Pediatr Surg. 1989 Oct;24(10):1079-83. doi: 10.1016/s0022-3468(89)80221-0.
Malignant small-cell tumor of the thoracopulmonary region (MSCT) is an uncommon neoplasm in children. We describe five cases diagnosed since 1981 that fulfill the criteria put forth by Askin et al. Surgery was performed for diagnosis or therapy in all patients. Two patients underwent open lung biopsy, only because of tumor extent. The other three had chest wall resections. All patients received radiotherapy and chemotherapy. Three patients presenting initially with extensive disease died at intervals of 2.5 to 7 months after diagnosis. Two patients are alive and disease-free at 16 and 24 months postdiagnosis. All five cases were reviewed for standard histology and differential immunohistochemistry. Electronmicroscopy and tissue cultures were done in 3 of the 5 patients. All five patients were neuron-specific enolase-positive. MSCT is an exclusion diagnosis not always readily made. Clinical course and pathologic expertise may point to the correct diagnosis.
胸肺区域恶性小细胞肿瘤（MSCT）在儿童中是一种罕见的肿瘤。我们描述了自1981年以来诊断的5例符合Askin等人提出标准的病例。所有患者均接受了手术以进行诊断或治疗。两名患者仅因肿瘤范围而行开胸肺活检。另外三名患者接受了胸壁切除术。所有患者均接受了放疗和化疗。最初表现为广泛疾病的三名患者在诊断后2.5至7个月内死亡。两名患者在诊断后16个月和24个月时存活且无疾病。对所有5例病例进行了标准组织学和鉴别免疫组化检查。5例患者中有3例进行了电子显微镜检查和组织培养。所有5例患者神经元特异性烯醇化酶均呈阳性。MSCT是一种并非总能轻易做出的排除性诊断。临床病程和病理专业知识可能有助于做出正确诊断。
