Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, California; Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, California.
Division of Pediatric Surgery, Children's Hospital Los Angeles, Los Angeles, California.
J Surg Res. 2020 Sep;253:115-120. doi: 10.1016/j.jss.2020.03.019. Epub 2020 Apr 27.
Pleuropulmonary blastoma (PPB) is the most common primary lung cancer in children. While rare, these tumors are highly aggressive. Tumor recurrence and overall survival are dependent on histologic grade and extent of surgical resection. We sought to examine our institutional experience with PPB to determine the effect of gross total resection (GTR) on recurrence and patient outcomes.
After IRB approval, a retrospective chart review from 1998 to 2018 was performed. Cases were confirmed by histology and Dehner Grade (I to III). Data collection included demographics, treatment, extent of surgical resection, and patient outcomes.
Eight patients with nine procedures were identified. Histologically, three cases were type 1, 2 type 2, and four poor prognosis type 3. Three patients received neoadjuvant chemotherapy to facilitate surgical resection. The operative goal was to achieve GTR (>95%), and to this end, three partial lobectomies, five lobectomies, and one pneumonectomy were performed. All nine cases achieved GTR, of which eight had negative microscopic margins. Two patients with type III disease recurred (one locally, one distant) and died. One type 3 patient had a positive microscopic hilar margin not amenable to further resection. The patient recurred (distant) but is in remission. With respect to patient outcomes, the event-free survival was 2.3 y with an overall survival of 3.3 y.
From our experience, GTR of PPB is associated with minimal surgical morbidity and good overall survival. Multi-institutional studies are needed to determine if positive surgical margins affect outcomes given the morbidity of mediastinal dissection.
胸膜肺胚细胞瘤 (PPB) 是儿童中最常见的原发性肺癌。虽然罕见，但这些肿瘤具有高度侵袭性。肿瘤复发和总生存率取决于组织学分级和手术切除范围。我们试图研究我们机构在 PPB 方面的经验，以确定大体全切除 (GTR) 对复发和患者预后的影响。
在获得机构审查委员会批准后，我们对 1998 年至 2018 年的病历进行了回顾性分析。通过组织学和 Dehner 分级（I 级至 III 级）来确认病例。数据收集包括人口统计学、治疗、手术切除范围和患者预后。
共确定了 8 例患者的 9 次手术。组织学上，3 例为 1 型，2 例为 2 型，4 例为预后不良的 3 型。3 例患者接受新辅助化疗以促进手术切除。手术目标是实现 GTR（>95%），为此，我们进行了 3 次部分肺叶切除术、5 次肺叶切除术和 1 次肺切除术。所有 9 例均达到 GTR，其中 8 例切缘阴性。2 例 3 型疾病患者复发（1 例局部，1 例远处）并死亡。1 例 3 型患者存在无法进一步切除的阳性微小肺门切缘。该患者复发（远处）但处于缓解期。关于患者预后，无事件生存率为 2.3 年，总生存率为 3.3 年。
根据我们的经验，PPB 的 GTR 与最小的手术发病率和良好的总生存率相关。鉴于纵隔解剖的发病率，需要进行多机构研究以确定阳性手术切缘是否会影响结果。
