Lee Chang Hun, Kim Keun Il, Kim Young Dae, Lee Min Ki, Kim Jee Yeon, Park Do Youn, Sol Mee Young, Suh Kang Suek
Department of Pathology, Pusan National University College of Medicine, Busan, Korea.
J Korean Med Sci. 2003 Aug;18(4):595-8. doi: 10.3346/jkms.2003.18.4.595.
Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting children and is characterized histologically by a variably mixed blastematous and sarcomatous patterns. We herein report a very exceptional adult case of PPB. A 21-yr-old male patient presented with a left chest pain of two weeks' duration. A computed tomography scan revealed a large, multicystic tumor occupying the left lower hemithorax, leading to the impression of a ruptured mediastinal cystic teratoma. A thoracotomy for resection of the tumor was performed. On histologic examination, the tumor consisted of cystic walls and associated solid lesions which showed undifferentiated blastemal tissues with focal fibrosarcomatous and rhabdoid features. Immunohistochemically the tumor cells only showed diffuse strong positivity for vimentin. The histologic findings corresponded to a type II PPB. The authors suggest that PPB, especially of type I or II, should be included in the radiologic differential diagnosis of mediastinal cystic neoplasms in a young adult.
胸膜肺母细胞瘤(PPB)是一种罕见的恶性发育异常肿瘤,主要影响儿童,其组织学特征为不同程度混合的胚基样和肉瘤样结构。我们在此报告一例非常罕见的成人PPB病例。一名21岁男性患者出现持续两周的左胸痛。计算机断层扫描显示一个巨大的多囊性肿瘤占据左下胸腔,给人一种纵隔囊性畸胎瘤破裂的印象。进行了开胸手术切除肿瘤。组织学检查显示,肿瘤由囊壁和相关实性病变组成,表现为未分化的胚基组织,具有局灶性纤维肉瘤样和横纹肌样特征。免疫组化显示肿瘤细胞仅对波形蛋白呈弥漫性强阳性。组织学表现符合II型PPB。作者建议,PPB,尤其是I型或II型,应纳入年轻成人纵隔囊性肿瘤的放射学鉴别诊断。