Pleuropulmonary blastoma - A clinicopathologic study of 50 cases
Pleuropulmonary blastoma - A clinicopathologic study of 50 cases
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DOI：
10.1002/(sici)1097-0142(19970701)80:1
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发表时间：
1997-07-01
期刊：
影响因子：
6.2
通讯作者：
Dehner, LP
中科院分区：
文献类型：
--
作者：
Priest, JR;McDermott, MB;Dehner, LP
背景。胸膜肺母细胞瘤（PPB）是一种儿童期独特的发育异常性肿瘤，表现为肺部和/或胸膜来源的肿块，组织学上以原始的、不同程度混合的胚基性和肉瘤性外观为特征。
方法。对所有病例的组织学材料进行了回顾，肿瘤被分为I型（单纯囊性）、II型（囊性和实性）或III型（单纯实性）。通过标准统计学方法将有关症状表现、家族史、手术所见、病理亚型、治疗干预措施和预后等数据与生存率进行相关性分析。
结果。该系列包括24名男性和26名女性。伴有或不伴有发热的呼吸困难是最常见的临床症状。在明确病理诊断时或之前，19名儿童（38%）通过影像学检查发现患侧肺部有囊肿形成。7例I型、24例II型和19例III型PPB的发病年龄有显著差异，分别为10个月、34个月和44个月（P<0.001）。7例I型PPB中有1例（14%）出现局部复发，43例II型和III型PPB中有18例（46%）出现局部复发；13名患者发生远处转移，主要转移至脑/脊髓或骨骼，且仅见于II型或III型PPB患者。胸膜或纵隔受累的患者预后明显比无此受累的患者差。I型患者的5年生存率为83%，II型和III型患者为42%。基于病理亚型的生存率差异未达到统计学意义。
结论。PPB是一种儿童早期具有侵袭性的胸腔内肿瘤，预后不良。尽管不同组织学亚型疾病患者的生存率差异未达到统计学意义，但单纯囊性的I型肿瘤患者明显较好的预后可能在更大系列的研究中得到证实。这些观察结果支持以下观点：II型PPB具有囊性和实性混合的特征，在形态学上连接了I型和III型PPB。PPB应被视为肺部相当于肾脏的肾母细胞瘤、肾上腺的神经母细胞瘤和肝脏的肝母细胞瘤的发育异常性类似物。分子遗传学研究，特别是在体质性PPB中，应该会有启示作用。鉴于II型和III型患者预后不良，必须开发新的积极的治疗方法。（C）1997年美国癌症协会
BACKGROUND. Pleuropulmonary blastoma (PPB) is a unique dysontogenetic neoplasm of childhood that appears as a pulmonary and/or pleural-based mass and is characterized histologically by a primitive, variably mixed blastematous and sarcomatous appearance.METHODS. Histologic material from all cases was reviewed and the tumors subclassified as type I (purely cystic), type II (cystic and solid), or type III (purely solid). Data regarding presenting symptoms, family history, operative findings, pathologic subtypes, therapeutic interventions, and outcome were correlated with survival by standard statistical methods.RESULTS. The series was comprised of 24 males and 26 females. Respiratory difficulty with or without fever was the most common clinical symptom reported. Cyst formation in the affected lung was identified radiographically in 19 children (38%) at or before the definitive pathologic diagnosis. The ages at presentation of the 7 type I, 24 type II, and 19 type III PPBs were significantly different: 10, 34, and 44 months, respectively (P < 0.001). Local recurrence developed in 1 of 7 type I PPBs (14%) and in 18 of 43 type II and III PPBs (46%); distant metastasis occurred in 13 patients, chiefly to the brain/spinal cord or bone, and was observed only in those with type II or type III PPB. Patients with pleural or mediastinal involvement fared significantly worse than those without such involvement. Five-year survival was 83% for type I and 42% for types II and III. Survival differences on the basis of pathologic subtype did not reach statistical significance.CONCLUSIONS. PPB is an aggressive, intrathoracic neoplasm of early childhood with an unfavorable outcome. Although survival differences among patients with different histologic subtypes of disease did not reach statistical significance, the apparently better outcome for patients with purely cystic type I tumors may be borne out in a large series. These observations support the premise that type I and III PPB are bridged morphologically by type II PPB with its combined cystic and solid features. The PPB should be regarded as the pulmonary dysontogenetic analogue to Wilms' tumor in the kidney, neuroblastoma in the adrenal gland, and hepatoblastoma in the liver. Molecular genetic investigations, especially in constitutional PPB, should be revealing. In view of the poor outcomes for patients with types II and III, new and aggressive therapies must be developed. (C) 1997 American Cancer Society.